Malignant airway obstruction is a life‐threatening condition that can cause suffocation and recurrent infections due to lung atelectasis. Adenoid cystic carcinoma is a rare and slow‐growing tumour of low‐grade malignancy. We report the case of a 69‐year‐old female who presented with severe chest pain, orthopnea, and a 1‐month history of progressively worsening difficulty in breathing. Emergent rigid bronchoscopy revealed a polypoid tumour originating in the proximal end of the left main bronchus that was obstructing the left main bronchus. Debulking of the tumour using rigid bronchoscopy was performed to restore ventilation to collapsed lung and obtain histopathological examination. Histological analysis revealed a diagnosis of adenoid cystic carcinoma. The patient underwent radical sleeve resection of the left main bronchus without sacrificing lung parenchyma via left posterolateral thoracotomy. No postoperative complications or disease recurrence was found at the 5‐year follow‐up. This case emphasizes the pivotal role of rigid bronchoscopic intervention in malignant central airway obstruction. This paper should be of interest to a broad readership, particularly those in the field of thoracic surgery, because we describe the case of a 69‐year‐old female who was referred to our hospital after presenting with orthopnea, severe chest pain, and progressively worsening difficulty in breathing for 1 month. Radical sleeve resection of the left main bronchus was performed without sacrificing lung parenchyma via left posterolateral thoracotomy. At 5‐year follow‐up, no operative complications or disease recurrence were noted. Adenoid cystic carcinoma (ACC) of the lung is a very rare malignant tumour and is reported to account for less than 0.04%–0.2% of lung tumours.A 69‐year‐old female non‐smoker was referred to our institute with severe chest pain, orthopnea, and a 1‐month history of progressively worsening difficulty in breathing. Chest x‐ray showed complete atelectasis of the left lung and severe deviation of the mediastinum to the left Figure . FlexiblLeft posterolateral thoracotomy was performed through the fourth intercostal space, followed by sleeve resection of the LMB with primary end‐to‐end anastomosis. The length of the resected bronchus was 3 cm. After division of Bottalo's ligament, the aortic arch was retracted upward and the left pulmonary artery was retracted downward with traction tape to obtain better exposure Figure . IntraopACCs are slow‐growing and known to run a progressive clinical course. They are prone to both late local recurrence and distant metastases.Atelectasis prevented definitive evaluation of the extent of the tumour at the distal side prior to debulking of the tumour. However, following observation of the distal extent of the tumour after the initial treatment, we considered that radical resection could be performed without sacrificing lung parenchyma.The cornerstone of treatment of ACCs is complete surgical resection because unsatisfactory results have been reported for alternative treatments such as chemotherapy and radiotherapy.Debulking of tumour under rigid bronchoscopy is a rapid, effective, and safe method of relieving and recanalizing central airway obstruction.In conclusion, emergent rigid bronchoscopic tumour debulking is a key step in the treatment of patients with malignant airway obstruction as it enables diagnostic confirmation and staging when considering surgery, and also provides significant palliative relief even in the case of unresectable lesions.Makoto Takahama drafted manuscript and have read and approved the final version of the manuscript.None declared.This case report was approved by the Osaka City General Hospital Institutional Review Board and the patient provided written informed consent for the use of these data.Written informed consent obtained from the patient for the publication of this manuscript and associated images.


The most common presenting symptoms of Rapunzel syndrome include abdominal pain (37%), nausea and vomiting (33.3%), obstruction (25.9%), and peritonitis (18.3%). Less commonly, patients may present with weight loss (7.4%) or intussusception (7.4%). Exceedingly rare complications of Rapunzel syndrome include gastric ulceration, obstructive jaundice, and acute pancreatitis as well as other malabsorptive-related complications including protein-losing enteropathy, iron deficiency, and megaloblastic anemia. This report details the case of an 11-year-old female with Rapunzel syndrome complicated by sepsis, a rare complication reported in only 2% of patients. A right upper quadrant ultrasound was obtained revealing a normal gallbladder with no gallstones and a common bile duct measuring 11 mm. Pancreas was not visualized on ultrasound.Our patient is an 11-year-old female with a 5-year history of poor weight gain, presenting with a 3-week history of constant nonradiating epigastric abdominal pain, nausea, vomiting, and decreased appetite. She was initially evaluated by her primary care provider at symptom onset and was prescribed a proton pump inhibitor and dicyclomine for pain, which did not provide relief. Upon arrival to the emergency department, physical exam was notable for malnutrition, with a weight z-score for age –2.15 and BMI < first percentile ; dehydration, with heart rate of 109 beats per minutes; and mild epigastric abdominal pain. Initial labs were notable for an albumin of 2.9 g/dL, ALT 151 µ/L, AST 100 µ/L, total bilirubin 0.4 mg/dL, direct bilirubin 0.1 mg/dL, lipase 1030 µ/L, triglycerides 65 mg/dL, WBC 11.7 K/mmE. coli within 6 hours of collection. A nasogastric tube was placed for gastric decompression due to nausea and vomiting. An abdominal x-ray was obtained to confirm NGT position and revealed the NGT was appropriately placed; however, the tube was noted to take an unusual course along the periphery of the stomach (Fig. She was admitted with intravenous fluids as well as ketorolac and morphine for pain control. On hospital day 2, she became febrile to 102.1°F, measured orally. Blood and urine cultures were obtained before initiating piperacillin-tazobactam. Blood cultures returned positive for The patient underwent emergent laparotomy, which revealed a large gastric bezoar containing hair, lice, nail clippings, and plant-like material extending from the stomach to the terminal ileum with several rope-like turns resulting in kinking of the small bowel Fig. . SeveralPsychiatry evaluated the patient before discharge, and she endorsed trichotillomania and trichophagia. Her parents and siblings denied ever witnessing her pulling or eating her hair or consuming any nonedible material. She had no known history of compulsive behaviors such as nail biting. On physical exam, she had no visible hair thinning or areas of alopecia. Further dietary history revealed the patient had a fair appetite at home eating 3 meals a day and snacks; however, she was a very picky eater as long as parents could recall and ate the same meals every day, typically pasta and chips, and refused to try new foods. Parents reported being concerned about her protein intake. When protein was encouraged and provided at meals, she would start gagging. Parents denied ever being told by her pediatrician that she was underweight. Cognitive behavioral therapy was recommended, and she was discharged to an inpatient nutritional and physical rehabilitation program.6 In the pediatric population, lifetime prevalence is around 1%–3%, and only a subset of patients will have trichophagia leading to trichobezoar.6 Rapunzel syndrome is rare form of trichobezoar with extension into the small bowel. It most commonly presents with abdominal pain and nausea/vomiting.2 Symptoms of severe malnutrition, acute pancreatitis, and bowel perforation are extremely rare. A literature search in 2016 by Ullah et al revealed only 2% of patients with Rapunzel syndrome had the complication of sepsis.4 The reports of sepsis describe a 5-year-old neglected female with a fatal outcome and another 5-year-old female with few details provided about the case.4 Our literature review revealed only 1 additional case of Rapunzel syndrome complicated by sepsis reported since then.5 This case occurred in a 17-year-old female with a 5-month history of abdominal pain acutely worsening 2 weeks before presentation.5 Since bowel perforation, although still a rare complication, is reported more frequently than sepsis, it maybe that subacute symptoms of abdominal pain and delayed presentation contribute to the development of sepsis. Our patient reported a 3-week history of abdominal pain, and the aforementioned cases described ongoing pain for 5 months and an unspecified, but presumably prolonged, amount of time in the case of neglect. To our knowledge, this is the first case reported in the literature of a patient with Rapunzel syndrome having concurrent E. coli sepsis, bowel perforation, and acute pancreatitis.3 Acute pancreatitis is thought to occur due to irritation and obstruction of the ampulla of Vater by mass effect of the bezoar tail as evidenced by a prominent and erythematous ampulla visualized on endoscopy. Trichobezoars may go unrecognized until they are large enough to cause gastrointestinal symptoms, as there may not be obvious signs of trichotillomania on exam, and the behavior often occurs in private. Cognitive behavioral therapy is the cornerstone of treatment, and a thorough psychiatric evaluation is warranted given the high incidence of other comorbid conditions such as mood, anxiety, eating, and substance use disorders.6Trichotillomania is classified as an obsessive-compulsive disorder with onset usually between the ages of 10–13 years old with a female predominance in late adolescence.


Parosteal osteosarcomas are low-grade bony malignancies that are treated primarily with surgical resection and reconstruction. This report discusses a unique case of a pediatric patient who presented with a parosteal osteosarcoma of the distal radius causing extensive erosive mass effect and growth disturbance of the adjacent ulna. Likely due to their slow-growing nonaggressive nature, parosteal osteosarcomas have not been previously described to abut adjacent bony structures through direct contact. The patient presented in a significantly delayed manner due to social circumstances, inadvertently revealing this novel behavior. This report reviews this rare case and describes the current understanding of this tumor.The patient is a 13-year-old male who presented with a parosteal osteosarcoma of his distal radius. He presented with a palpable wrist mass and wrist stiffness. He presented in a delayed manner with advanced local disease due to social factors. Imaging revealed an osseous radial mass that abutted the ulna and likely stunted its growth. The patient ultimately underwent complex resection and allograft reconstruction of both his distal radius and ulna. Intraoperative pathology was confirmed to have negative tumor margins. Allograft reconstruction of the radius and ulna was performed utilizing patient-specific custom cutting guides. At the 6-month postoperative visit, the patient had no recurrence of the mass, minimal pain, and had almost regained range of motion of the extremities. Clinical radiographs at the 6-month postoperative visit demonstrated allograft incorporation.A previously unreported case of pediatric parosteal osteosarcoma of the distal radius with erosion of the adjacent ulna through direct contact is presented. The challenges in and the importance of arriving at a definitive diagnosis in a timely manner for the proper treatment of this malignancy are emphasized. Parosteal osteosarcomas are low-grade bony malignancies that typically occur on the surface of metaphyseal long bones. They occur more commonly in women in the third decade of life. They carry a reported 16% risk of dedifferentiation and metastatic spread and are managed primarily with wide surgical resection and reconstruction [There are several diagnostic challenges present in identifying parosteal osteosarcomas. The histopathology of a parosteal osteosarcoma can appear benign. Both imaging and pathology can mimic other benign processes such as an osteochondroma . MoleculThis report presents an unusual case of a 13-year-old skeletally immature male with a parosteal osteosarcoma involving the distal radius with erosive mass effect of the adjacent ulna. Due to social factors, the patient presented in a delayed manner. Treatment was further delayed due to difficulties diagnosing the lesion. The patient ultimately underwent successful wide resection with negative margins and reconstruction with a strut allograft, plates and screws.The patient was a 13-year-old male, right hand dominant football player who was referred for a left wrist mass. He was brought in by his step-mother, who had noticed the mass and was concerned about the patient’s progressive decrease in wrist range of motion. Upon chart review, the patient had radiographs of the forearm documenting a left distal radius mass one year prior to his initial visit with an orthopedic oncologist. Further history revealed that after presenting the previous year, the patient was lost to follow-up and did not obtain further imaging or treatment during the interim. Physical exam revealed a large solid volar mass at the wrist without any sensorimotor deficits. The radial and ulnar pulses were both 2+. He had limited range of motion at the wrist with approximately 15 degrees of supination and 10 degrees of pronation. He lacked approximately 15 degrees of flexion and extension compared to the contralateral wrist. New radiographs demonstrated that the mass had grown approximately 3 mm in width, 5 mm in length, and increased in radiopacity compared to imaging one year prior  and the difference between the most distal point of the radial and ulnar metaphysis (DIDI) were measured to be approximately 1.35 and 1.6 cm on initial presentation one year prior. Similarly, more recent radiographs demonstrated approximately 1.15 to 1.68 cm of ulnar shortening.Differential diagnosis at this time included osteochondroma, melorheostosis, high grade surface osteosarcoma, bizarre parosteal osteochondromatous proliferation, and low grade parosteal osteosarcoma. An open surgical biopsy targeting the ulnar aspect of the mass under fluoroscopic guidance was performed. Intraoperative frozen sections revealed lesional tissue with benign fibrous tissue and bone. Biopsied tissue was decalcified for permanent histology preparation. Therefore, molecular studies on the samples obtained were not able to be done. Permanent tissue histology demonstrated hyaline cartilage merging with bony trabeculae and associated bland fibrovascular and fatty tissues. The biopsy was ultimately deemed nondiagnostic.Due to the unique location, an inconclusive biopsy, and the tumor’s growth over the past year, there remained an ongoing suspicion of malignancy. Multiple discussions were had with the patient and family to perform a repeat biopsy of the mass and 3 months later a repeat open biopsy was performed following the initial procedure. Tissue was sent to an outside institution for consultation with molecular studies. The outside facility found the biopsy to be MDM2 positive on stain, and the patient was diagnosed with a low-grade parosteal osteosarcoma  concluded that there were negative margins for malignancy and confirmed the diagnosis of low-grade parosteal osteosarcoma of the distal radius. The Ulna did have reactive changes however it did not have MDM2 amplification which favored the radiographic changes being due to “extensive erosive mass effect” rather than tumor involvement. At his six-month postoperative appointment, the patient had almost regained full range of motion Fig. . RadiogrParosteal osteosarcomas are rare low-grade bony malignancies with a slight female preference found in the second to fourth decades of life . Okuda eAlthough they were once thought to be a subtype of intramedullary osteosarcomas, the World Health Organization (WHO) now recognizes parosteal osteosarcomas as a distinct entity with its own unique clinical course and pathologic characteristics . On presHistological and immunochemical analysis showed a fibro-osseous lesion with hypocellular spindle cell stroma containing trabeculae of bone. Spindle cells usually have minimal cytologic atypia and limited mitotic activity . Okuda eParosteal osteosarcomas are well-differentiated malignant tumors with low local recurrence rate and metastatic potential. Treatment consists of wide resection without chemotherapeutic intervention. Ruengwanichayakun et al. demonstrated that the degree of dedifferentiation of these tumors had the largest inverse effect on long-term survival. They reported 96% 5-year survival in parosteal osteosarcomas compared to 65% in cases with dedifferentiation. Similarly, they reported the 10-year survival rates of the two groups to be 96% and 60%, respectively . Their fIn this case, there was a significant delay in treatment due to various social factors. The patient initially presented with advanced local disease and was lost to follow-up for an additional year. Surgical resection was further delayed following diagnosis due to the patient’s desire to participate in his high school sports season. Fortunately, despite the delayed presentation, the patient’s lesion did not worsen significantly on repeat plain radiographs. This reflects the slowly evolving natural history of this tumor.Direct local abuttment of adjacent bone has been reported in aggressive bony malignancies and has been associated with a higher likelihood of local recurrence and poorer outcomes . In thisUtilizing the mean distal ulnar growth rate and negative ulnar variance measurements, an estimation of when the tumor began impeding the distal ulnar growth and approximate how long the tumor had been growing. The Hafner Method was utilized to calculate the ulnar variance for the patient. In short, this is a validated method of measuring negative ulnar variance in pediatric patients by measuring the difference between the most proximal point to the radial and ulnar metaphysis (PRPR) and the difference between the most distal point of the radial and ulnar metaphysis (DIDI). Even at initial presentation, the patient had approximately 1.35 to 1.6 cm of ulnar shortening. Assuming the mean growth rate of the distal ulna to be approximately 0.9 cm per year; the mean ulnar growth rate in skeletally immature males is reportedly 1.1 cm per year, with the distal ulna accounting for 85% of the growth . Using tFinally, the case also demonstrated the common diagnostic difficulties associated with this malignancy. Pathology can often have a benign appearance, making molecular studies crucial in making the correct diagnosis. Aberrant gene signaling can only be detected at the active margins of the tumor, and as in this case, these can be prone to sampling errors . AdherinPresented is a case of low-grade parosteal osteosarcoma in the distal radius in a skeletally immature individual. Described is a unique phenomenon direct contact between adjacent bones. To date, this has not been previously described in the literature for this malignancy. In addition, highlighted are the challenges encountered during the management of this case. Tissue diagnosis was obtained and limb salvage was possible in part due to the advances made in molecular testing for this malignancy. Although the diagnosis and treatment were delayed in this case for various reasons, the patient thus far has had a positive outcome, which provides insight into the unique natural history of this tumor.


Cosmetic and functional outcomes are of great concern for nasal planum reconstruction, and the wishes of the owner in this regard may limit the extent of the excision and risk non-curative intent surgery. In veterinary medicine, several surgical techniques have been described, depending on the type, location, and extent of the tumor. In this study, a rotation alar fold flap technique is described and used in a short clinical case series following a subtotal or partial planectomy for oncological surgical purposes. No intraoperative and minor self-limiting post-operative complications were recorded, and the cosmetic and functional outcomes were considered very satisfactory in all cases. The rotation alar fold flap offers a valuable, feasible, functional, and aesthetically satisfactory alternative surgical option for selected cases of localized tumors involving the central and ventral planum. This technique, among others, can be used safely to improve the quality of life and the long survival time of our pets.Nasal planum reconstruction is a surgical challenge, and several surgical techniques have been described. The objective of this study was to describe the rotation alar fold flap technique and to report clinical outcomes in a short case series. The feasibility of the technique was first assessed in a canine cadaveric model. The rotation alar fold flap was obtained by a single sharp horizontal incision of the dorsolateral nasal cartilage, preserving the caudal mucosal attachment to the ventral nasal concha. The flap was then rotated ventro-medially for the reconstruction of the ventral aspect of the nasal planum unilaterally or bilaterally. The rotation alar fold flap technique was used following a subtotal or partial planectomy for excision of a squamous cell carcinoma or mast cell tumors in three dogs. No intraoperative complications were recorded. Superficial surgical site infection was reported in two cases and minor dehiscence was reported in one case. However, survival of the flap was not affected. The cosmetic and functional outcomes were considered very satisfactory in all cases. The rotation alar fold flap technique offers a safe, valuable, feasible, functional and aesthetically satisfactory alternative surgical option for selected cases of localized tumor involving the central and ventral planum. Neoplasms of the nasal planum are relatively rare in dogs . SquamouSubtotal planectomy for a tumor located in the central part of the nose without the involvement of the alar folds can be considered . The alaThe objective of this study is two-fold: (a) to describe a rotation alar fold flap technique following subtotal or partial planectomy, by assessing its feasibility in a cadaveric model, and (b) to report the outcomes in three dogs.The feasibility of the technique was assessed on a canine cadaver (Labrador Retriever), euthanized for reasons unrelated to this study, and for which the owners donated the body post-mortem for teaching and research. The cadaver was stored at −18 °C and then thawed to room temperature for the feasibility study. The dog was positioned in sternal recumbency and its head was secured to the table with waterproof tape over a vacuum positioning cushion to maintain the most appropriate position for the surgical procedure. A subtotal planectomy was performed by removing, with a sharp incision, the central planum including the septum and the ventral planum including the philtrum and both nasal sulci, spearing the dorsal planum and the alar cartilage.The rotation alar fold flaps were obtained on both sides by a single sharp horizontal incision of the dorsolateral nasal cartilage, preserving the alar fold and its caudo-lateral mucosal attachment to the ventral nasal concha. The flaps were then pivoted ventro-medially for the reconstruction of the nasal vestibulum and ventral aspect of the nasal planum. The mucosal edges of the flap were sutured together in the middle and to the dorsal aspect of the rostral lips in a simple interrupted suture pattern with a 4.0 absorbable monofilament suture. The nasal septum and the central planum were not reconstructed .The feasibility of the same technique following unilateral partial planectomy was assessed in another canine cadaver (Labrador Retriever). Unilateral partial planectomy was performed, with the sulcus and ventral planum being unilaterally removed with a sharp incision, sparing the central planum, the philtrum, and the alar cartilage. The rotation alar fold flap was created in the same way described above and it was used to unilaterally reconstruct the nasal defect.Case 1. A 10-year-old, 36.5 kg, intact male Golden Retriever was referred for further investigations of a 4-month history of unilateral right serous nasal discharge associated with an ulcerative lesion involving the right rostral nostril. On admission, an ill-defined area of depigmented tissue within the medial margin of the right nare was visible. The submandibular and superficial cervical lymph nodes were palpably normal in size. Pre-operative complete blood count and serum biochemistry profiles were within normal range, and the serology for canine sino-nasal aspergillosis was negative. Rhinoscopy and computed tomography (CT) examination of the head, neck, and thorax were performed. Computed tomography of the nasal cavity revealed a poorly defined, contrast-enhancing soft-tissue attenuating mass involving the philtrum, the rostral, and the right lateral part of the septum. There was no evidence of bony destruction or thoracic abnormalities. Histopathology examination of the nasal biopsies was consistent with SCC. Cefalexin (20 mg/kg IV) was administered for antimicrobial prophylaxis at induction and every 90 min throughout the procedure. The patient was placed in sternal recumbency, and the nose and maxillary region were clipped and aseptically prepared. Two minutes before surgery, xylometazoline hydrochloride (0.1%) was sprayed into the nasal cavities with a nasal spray diffusor device. A subtotal planectomy was performed to achieve at least 5 mm gross surgical margins: the central planum including the septum and the ventral planum including the philtrum and both nasal sulci were excised with a sharp incision, sparing the dorsal planum and the alar cartilage. Nasal reconstruction was performed as described above for the ex vivo study , meloxicam , and paracetamol . The dog was discharged 2 days postoperatively with a 7-day oral course of meloxicam (0.1 mg/kg), paracetamol , and clavulanate amoxicillin (12.5 mg/kg BID). Upon discharge, the dog appeared comfortable and was eating and drinking normally. Clinical follow-up at 1 and 2 weeks after surgery showed progressive healing of the surgical wound without dehiscence. A moist superficial surgical site infection was present at 1 week post-op. The antibiotics course was continued for 1 more week. By the third week, the wound was completely healed. Owner satisfaction with the procedure and the appearance of the dog was very high. Clinical follow-up at 3 and 5 months was unremarkable: there were no signs of tumor recurrence or signs of any complications. Seven months after surgery, the owner reported that the dog was lethargic and inappetent. No visible signs of tumor recurrence were apparent or enlargement of the regional lymph nodes. Blood work abnormalities included severe increased ionized calcium . The owner declined further investigation and humane euthanasia was elected.Case 2. A 12-year-old, 10 kg, intact male Shih-Tzu was referred for recurrence of an incomplete resected low-grade (Kiupel grading system) MCT affecting the left rostral lip. Physical examination revealed an approximately 1 cm round, alopecic cutaneous mass immediately ventro-lateral to the philtrum and the left ventral planum. The pre-operative complete blood count and serum biochemistry profiles were largely unremarkable. Abdominal ultrasound followed by ultrasound-guided hepatic and splenic fine-needle aspirates and cytology examination revealed no evidence of distant tumor metastasis. Sentinel lymph node mapping using a peri-tumoral intradermal injection of lipid-based contrast medium (Lipiodol Ultra-Fluid™), as previously described by Brissot et al. (2017) [. (2017) , revealeHistopathology examination of the excised tissue confirmed the complete excision of a high-grade (Patnaik and Kiupel grading system) MCT with no evidence of metastasis of the sentinel lymph node.The dog was discharged the day after surgery, with a 7-day course of meloxicam (0.1 mg/kg) and clavulanate amoxicillin (12.5 mg/kg BID). Clinical follow-up 2 weeks after surgery revealed an uneventful healing process. According to the owners, the functional and aesthetic outcomes were very satisfactory. Due to financial constraints, no adjuvant treatment was planned for this dog. At long-term telephonic follow-up (26 months after surgery), the dog was still alive without visible signs of tumor recurrence.Case 3. A 12.5-year-old, 12.6 kg, intact male Cocker Spaniel was referred for unilateral right nasal discharge and a 3 cm cutaneous mass in the region of the right upper lip and rostral region. Cytology of the FNA was consistent with an MCT. The pre-operative complete blood count and serum biochemistry profiles were largely unremarkable. A CT scan of the head, neck, and thorax revealed a focal homogenous, soft tissue attenuating, contrast-enhancing mass in the right upper lip, whereas CT of the thorax was within normal limits with no overt signs of thoracic metastasis. CT-indirect lymphography using an intra-tumor injection of water-soluble contrast medium iohexol (Omnipaque™) revealed intense uptake of contrast within the right lateral mandibular lymph node and mild uptake of the right medial retropharyngeal lymph node. Abdominal ultrasound examination followed by ultrasound-guided hepatic and splenic fine needle aspiration and cytology examination revealed no evidence of tumor metastasis. Cefalexin (20 mg/kg IV) was administered for antimicrobial prophylaxis at induction and every 90 min throughout the procedure. Chlorphenamine maleate (0.5 mg/kg IM) was also administrated at induction. The patient was placed in left lateral recumbency with the head slightly elevated and at an approximately 45° angle when viewed from the front. Two minutes before surgery, xylometazoline hydrochloride (0.1%) was sprayed into the nasal cavities with a nasal spray diffusor device. Following lymphadenectomy of the right submandibular and right retropharyngeal lymph nodes, the tumor excision was performed with the largest possible proportional margins. The oral mucosa was considered the deep surgical margin. Dorsally, the surgical excision included the right lower planum. The reconstruction of the rostral labial and nasal defects was achieved by a combined full-thickness labial advancement flap and a unilateral right side rotation alar fold flap as described above (ed above .The dog was discharged the day after surgery with a 7-day course of meloxicam (0.1 mg/kg SID) and clavulanate amoxicillin (12.5 mg/kg BID). The antibiotic course was protracted due to a local superficial surgical site infection and minor wound dehiscence of the tip of the labial flap.2, IV, every week with 4 doses and then every 2 weeks with 4 further doses) and prednisolone  administration [Second-intention healing allowed the complete closure of the wound within 3 weeks of surgery. Owner satisfaction with the procedure and the appearance of the dog was very high. Histopathology examination was consistent with incomplete excision of a low-grade (Kiupel grading system) and high-grade (Patnaik grading system) MCT. Histopathological examination of the sentinel lymph nodes did not reveal evidence of tumor metastasis. Adjuvant chemotherapy was started 4 weeks after surgery. The protocol was based on vinblastine (2 mg/mstration . At clincase 1), the philtrum and the septum were not reconstructed. However, that did not cause flattening of the dorsal planum and did not affect the final aesthetic or functional outcome. This confirms the satisfactory cosmetic outcome reported by Chiti et al. (2017) [The rotation alar fold flap technique described here was used successfully in three dogs for nasal reconstruction following subtotal and partial planectomy. No intraoperative complications directly associated with the surgical technique were observed and the post-operative complications were limited to local infection and limited wound dehiscence that did not affect the viability of the flap itself. This technique presents several advantages. It is relatively easy and safe to perform. Comparatively with complex skin flaps, the use of local planum tissue in the flap requires a limited amount of time, reducing the anesthesia time, the anesthetic risks related to a prolonged surgery as well as the cost . The ala. (2017) , where tcases 2 and 3) nor could it be used to reconstruct the large nasal vestibule defect after the subtotal planectomy (case 1). The rotation alar fold flap described here can be unilateral or bilateral based on the specific case needs, and it can also be used to reconstruct the nasal vestibule and the lower nasal planum due to its rotation in a ventro-medial direction. However, its use is limited to the reconstruction of the central and lower planum.In the initial technique described by Pavletic (2010) , the alacase 1 and at least proportionate margins [cases 2 and 3. The excision was complete for cases 1 and 2, but it was incomplete for case 3. The MCT of case 3 was graded as low grade according to the Kiupel grading system but high grade according to the Patnaik grading system, with no histological evidence of metastasis to the sentinel lymph node. Mast cell tumors occurring on the muzzle seem to behave more aggressively and are characterized by local infiltration [In our short case series, nasal reconstruction followed oncologic surgery. Obtaining surgical margins is a priority, and ideally, it should not be sacrificed at the expense of cosmetic outcomes . However margins  for the ltration . Howeverltration . In our Accurate preoperative evaluation of the extent of the tumor to define the margins of the excision is crucial ,6. Compucase 1 to evaluate the extent of the lesion and also to rule out other nasal diseases. In cases 2 and 3, staging was completed with sentinel lymph node mapping using a peri-tumoral intradermal injection of contrast medium, abdominal ultrasound, and fine needle aspiration of the liver and spleen. In those two cases, the sentinel lymph nodes were excised too. Because local or distant metastatic disease from nasal planum SCC appears to be rare [case 1, lymph node staging was limited to physical examination and diagnostic imaging. For this case, for which the excision of the tumor was complete, 7 months after surgery, hypercalcemia associated with severe clinical signs was diagnosed. The owner elected for humane euthanasia and declined further investigations and post-mortem necropsy. Hypercalcemia as a paraneoplastic syndrome of recurrent or metastatic SCC could have been possible; however, no signs of local recurrence or enlargement of regional lymph nodes were noticed at that time, and other pathologies or other types of tumor as a cause of hypercalcemia cannot be completely ruled out.A contrast-enhanced computed tomography study, including the head, neck, and thorax, was performed in two of our cases, and surgical planning was based on the tomographic findings. Rhinoscopy was also performed in Intra- and post-operative bleeding is one of the major concerns during nasal and maxilla-facial surgeries. In order to reduce the rate and extent of bleeding and to improve the visibility of the surgical field, xylometazoline hydrochloride (0.1%) was sprayed into the nasal cavities in all of our cases. Xylometazoline is an imidazole derivative that acts directly on the alpha-adrenergic receptors of the arterioles of the nasal mucosa to produce vasoconstriction resulting in decreased blood flow. Its efficacy is comparable to the diluted adrenaline in the control of anterior epistaxis in human medicine . The usePost-operative complications in maxilla-naso-facial surgery are common and frequently include incisional dehiscence or stenosis of the nasal opening, which is likely due to excessive tension ,3,13,24,Self-limiting superficial surgical infection occurred in two of our cases. This was the consequence of continuous licking of the wound. The Elizabethan collar, in fact, protected the wound from being scratched and rubbed with the legs and paws, but it was ineffective in protecting the wound from the tongue. The superficial infection did not affect the survival of the flap and no further management was required aside from a protracted course of antibiotics.case 3, where a combination of lip advancement-rotation alar flaps was performed. In this case, the dehiscence involved the tip of the labial flap. The tension and motion of this region play a major role in this complication. Also, in this case, no further treatment was required, aside from local care of the wound and a protracted antibiotic course.Minimal wound dehiscence was observed in In conclusion, we describe a rotation alar fold flap technique and its application in three clinical cases following subtotal or partial planectomy. This nasal reconstruction technique offers a safe, valuable, feasible, functional and aesthetically satisfactory surgical option. This technique could be used for selected cases of localized tumor involving the central and ventral nasal planum and for which the oncology surgical principle and the functional and cosmetic expectations of the client can be respected.


The popularity that transorbital approaches (TOAs) gained in recent years has allowed their diffusion into skull base surgery. However, for their correct application, it is essential to know the potential and the limits of this group of transorbital routes, as well as the knowledge of anatomy from this relatively new surgical perspective. In this paper, the authors illustrate step-by-step the superior eyelid transorbital approach from a macroscopic and endoscopic perspective, highlighting the main anatomical relationships to understand the application of this surgical route for the treatment of skull base pathologies. Surgical cases are provided to illustrate indications for this approach.Background: The transorbital approaches (TOAs) have acquired growing notoriety, thanks to their ability to offer alternative corridors to the skull base. However, the limited access and the unfamiliarity with this surgical perspective make recognition of key landmarks difficult, especially for less experienced surgeons. The study wants to offer a detailed description of the anatomy to comprehend the potential and limitations of TOAs. Methods: Measurements of the orbit region and the surrounding areas were performed on two hundred high-resolution CT scans and thirty-nine dry skulls. Five specimens were dissected to illustrate the TOA, and one was used to perform the extradural clinoidectomy. Three clinical cases highlighted the surgical applications. Results: A step-by-step description of the key steps of the TOA was proposed and a comparison with the transcranial anterior clinoidectomy was discussed. The mean work distance was 6.1 ± 0.4 cm, and the lateral working angle increased 20 ± 5.4° after removing the lateral orbital rim. Conclusions: TOAs are indicated in selected cases when tumor involves the lateral portion of the cavernous sinus or the middle skull base, obtaining a direct decompression of the optic nerve and avoiding excessive manipulation of the neurovascular structures. Comprehension of surgical anatomy of the orbit and its surrounding structures is essential to safely perform these approaches. The evolution of neurosurgical techniques that occurred in the last 20 years has favored the development of increasingly minimally invasive approaches for the treatment of skull base pathologies ,2,3. TheIn 2010, the term “transorbital neuroendoscopic surgery” (TONES) was created for the first time to define a group of endoscopic approaches that offered alternative surgical corridors for the treatment of skull base pathologies .The application of TOAs has been subsequently extended to the lesions of the anterolateral skull base such as meningiomas, schwannomas, dermoid cysts, CSF leak, and infections ,22,23,24However, the limited surgical view and workspace, and the unfamiliarity with the surgical perspective offered by TOAs make recognition of key surgical landmarks challenging, especially for less experienced surgeons. This study offers a detailed step-by-step description of the superior eyelid transorbital route, identifying the main anatomical landmarks to better understand the principles and applicability of this innovative surgical approach. The microscopic and endoscopic view of the main passages is offered to better understand the anatomical relationships. Moreover, the anatomy of the orbital cavity and the adjacent region have been studied, highlighting the most important applications of this approach.The research was reviewed by the Institutional Review Board and conducted according to the ethical guidelines of the Declaration of Helsinki. The patients’ consent was obtained.Thirty-nine dry skulls (78 sides) were studied by two authors (S.S. and M.N.) to analyze the main bony relationships between the orbit region and middle cranial fossa. A total of eight measurements were performed with the use of a sliding caliper : four meTwo hundred high-resolution CT scans (400 sides) were utilized to measure the ACP length, optic roof, and optic strut, and the working distance to the tip of the ACP from the orbital floor.-Type 1: the optic canal was adjacent to the sphenoid sinus (SpS) without impression;-Type 2: the optic canal caused an impression on the SpS;-Type 3: the optic canal was identified in the SpS;-Type 4: the optic canal was found lateral to the SpS and the posterior ethmoid sinus .The course of the optic canal and the ACP pneumatization was classified according to DeLano’s classification  into 4 dThe measurement of the working angle on the axial plane was evaluated on five post-op CT scans in patients who underwent a TOA. Considering the tangent to the lateral orbital rim as the lateral limit of the surgical corridor, we measured the additional working angle on the axial plane in these cases in which the frontal process of the zygomatic bone was removed.The radiological measurements for the morphological analysis were obtained through a dedicated analysis image software .Ten sides of five embalmed latex-injected human heads were dissected to illustrate the main surgical steps of the TOA. The dissection included a first phase of soft tissue exposure , followed by the endoscopic/microscopic phase. A rigid endoscope , paired with 0°, 30°, or 45° rod lenses , was used.A standard pterional approach with subsequent extradural anterior clinoidectomy was performed on an embalmed latex-injected specimen for the comparison of the two surgical corridors to the ACP.https://www.jamovi.org, accessed on 1 February 2023), and the final values were expressed as percent, mean, and standard deviation.The statistical evaluation was performed with Jamovi Version 2 statistic software (The jamovi project (2022) (Version 2.3) [Computer Software] The specimen was positioned supine with the head in a neutral position, and a 3 cm S-shaped superior eyelid skin incision was made 5 mm above the upper lid lash line following the skinfold .The palpebral portion of the orbicularis ocularis muscle was exposed A. The inIf a wider surgical corridor was required, the incision was further extended laterally to directly expose the margin of the lateral orbital rim (LOR) after its identification by palpation B,C.Once exposed, the periosteum was divided at the level of the frontal process of the zygomatic bone and subperiorbital dissection was performed D. DuringThe sphenozygomatic suture was exposed up to the meningo-orbital foramen (when it is present), where the meningo-lacrimal artery, an orbital branch of the middle meningeal artery, runs. This artery could be used as a landmark to identify the proximity to the meningo-orbital band and the superior orbital fissure (SOF). During the dissections, the foramen and the meningo-lacrimal artery were recognized in eight cases (80%).From this point, the use of the malleable retractor was required. The spheno-frontal suture was considered the projection of the Sylvian fissure ,49. OnceThe craniectomy was performed by drilling the orbital surface of the body of the zygomatic bone and the greater wing of sphenoid bone (GWS), using the SOF and IOF as the medial border, and the frontozygomatic suture as the superior limit. After this step, the temporalis muscle and the dura of the temporal pole were exposed E,F. If aThe MOB is a dural band that connects the periorbita with the fronto-temporal dura D 40]. T. T40]. TStarting from the SOF, an interdural dissection was performed to separate the temporal dura from the meningeal layer of the lateral wall of the cavernous sinus (LWCS). The direction of the dissection was parallel to the cranial nerves of the LWCS to avoid damaging them or to accidentally enter in the CS, injuring the cavernous segment of the internal carotid artery (ICA) B–D. The The interdural peeling of the LWCS allowed the visualization of the ACP and the surrounding dura was detached before proceeding with the extradural anterior clinoidectomy. In order to perform this maneuver, the medial portion of the LWS, one of the three anchor points of the ACP, was accurately removed. Then, the orbital roof was taken off, and CN II was identified in the optic canal (OC) A. The clThe analysis of the endocranial surface on the dry skulls highlighted that the average thicknesses of the optic strut and roof were 3.6 ± 0.9 mm (range 2–6 mm) and 1.8 ± 0.6 mm (range 0.9–3.2 mm), respectively. The longitudinal length and transverse diameter of the ACP were 10.2 ± 3.6 (range 5.6–17.4 mm) and 5.5 ± 2.9 mm (range 2.8–8.5 mm). A carotid clinoid foramen between the anterior and middle clinoid processes was identified on five sides (five specimens) .At the level of the orbital region, the thickness of the optic strut and the maximum transverse diameter of the OC were 3.1 ± 1.2 mm (range 1–5 mm) and 7 ± 0.9 mm (range 5–9 mm), respectively. The distances between the OC and the anterior and posterior ethmoid canal were 18.2 ± 3.6 mm (range 8–26 mm) and 6.6 ± 2.5 mm (range 3–16 mm) .The CT measurements  were perAnalyzing the clinical cases with the removal of the lateral orbital rim, the working angle on the axial plane increased by 20 ± 5.4° (range 14.7°–27.8°) laterally compared to the trajectory with preservation of the lateral orbital rim.A 55-year-old woman presented with a 1-year progressive proptosis in the right eye, associated with occasional episodes of headache and a periorbital, dull, achy sensation. A brain MRI scan documented the presence of a hyperostotic lesion involving the right sphenoid wing and the lateral orbit with adjacent dural thickening, with a mass effect on the lateral rectus muscle A–C. At tThe postoperative course was uncomplicated. Histological analysis confirmed the diagnosis of the sphenoid wing and intraosseous meningioma (WHO grade 1). Five years after surgery, no evidence of bone regrowth or tumor was documented D–F.A 64-year-old woman presented with a 2-year history of diplopia in the long-distance gaze. A brain MRI scan identified a small extra-axial temporal pole lesion, that continuously increased in subsequent radiological follow-up. A–C. A suA 51-year-old woman presented with a 5-year history of progressive pain in the left hemiface, occasionally stimulated by thermal triggers, that was poorly controlled by medical therapy. A hyperostotic lesion on the right side involving the greater and the lesser sphenoid wing with adjacent dural thickening was found in the MRI scan A–C. At tIn the immediate post-operative course, the patient reported blurry vision and palpebral motility impairment in the right eye, that progressively improved during the hospitalization. Histological analysis confirmed the diagnosis of secretory meningioma of the sphenoid wing (WHO grade 1). After one month, no signs of recurrence were reported at the radiological follow-up  D–F, witTransorbital approaches represent an innovative surgical route not only for the treatment of orbital pathologies, but also offer a direct surgical corridor to the LWCS ,40,51,52However, several potential complications must be considered. The palpebral phase must be conducted adequately in order to avoid potential complications related to levator palpebrae muscle injury, such as the risk of post-operative ptosis ,53,54,57The extradural transorbital anterior clinoidectomy has a surgical trajectory along the longitudinal axis of the ACP, allowing a direct view of the opticocarotid region with a short working distance ,36,37. Ip < 0.01) [The removal of the lateral orbital rim performed in five clinical cases demonstrated an increase in the working angle on the axial plane by 20 ± 5.4° (range 14.7°–27.8°) laterally with respect to the trajectory with preservation of the lateral orbital rim, improving the surgical maneuverability and especially the resection rate, as described by Kong et al. (54.5.% vs. 26.3%  < 0.01) . Recentl < 0.01) . TherefoBefore proceeding, a pre-operative thin-slice CT angiography study is suggested to highlight any anatomical variants of the orbital and sellar region, but mostly for eventual ICA anomalies, such as agenesia or hypoplasia. Moreover, the morphological study highlighted the presence of carotico-clinoid foramina in five sides on dry skulls, while in the CT scans, pneumatization of the ACP was found in 7.5% of cases and variants of the course of the optic nerve according to DeLano’s classification were detected . The varIt should also be kept in mind that several vascular anatomical variants exist. In our study, the meningo-lacrimal artery was recognized in eight cases (80%), while in the literature it was identified in 60% of patients . AlthougOne of the most important limitations to be noted is certainly the limited vision of the operating field and the reduced maneuverability can limit the management of complications and the degree of excision of the lesion. Among the factors that can limit the gross total resection using TOAs are the predominant extension towards the medial region of the cavernous sinus and posterior and infratemporal fossa, multicompartmental tumoral extension, vascularized diseases, and lesion pattern (en-plaque type) ,52,60,64Most of the data available in the literature are preliminary studies on case reports or small groups of patients. Considering the types of lesions commonly treated , the slow tumor growth in most cases, and the novel introduction of this surgical technique, it is difficult to establish the disease recurrence rate due to the scarcity and heterogeneity of available data. Concerning schwannomas, Kong et al., in their clinical series of 50 patients, reported no tumor regrowth or recurrences during 21.9 months of follow-up, although 5 patients had previously undergone treatment (GK or surgery) . RegardiThe reconstruction phases are essential to avoid post-operative complications such as CSF leak, infection, meningitis, pseudomeningocele, enophthalmos, chronic subdural hematoma, and subretinal hemorrhage ,54,56,57The comprehension of the surgical anatomy of the orbit and its surrounding structures is essential to understand the safe application of transorbital approaches. This route is highly suggested in selected cases with tumor extension lateral to the optic nerve, with an average maximum diameter of the lesion around 4 cm, to obtain direct decompression of the optic nerve and main vascular arteries and avoid excessive manipulation of the neurovascular structures.


Lymphangioma is a congenital anomaly in which abnormal lymphatic drainages localize to form a benign mass, but it has the tendency to grow in size and the potential to infiltrate surrounding structures, causing devastating effects and leading to severe morbidity. The most common site of lymphangioma is the neck region (cystic hygroma colli), whereas lymphangioma in the lower limbs is very rare, accounting for only 2% of cases. Accordingly, the prenatal diagnosis of lymphangioma of the lower limbs has been scarcely reported. This study describes two cases of lymphangioma of the lower limbs, focusing on unique sonographic features and the natural course of rapidly progressive changes, which is different from nuchal lymphangioma. Based on previous isolated case reports together with our two cases, lymphangioma of the lower limbs usually develops in the second trimester, tends to have rapidly progressive changes, and is unlikely to be associated with aneuploidy and structural anomalies. Diagnoses can be made by using sonographic findings pertaining to the subcutaneous complex and multi-septate anechoic cystic lesions in the lower limbs, the latter of which can infiltrate visceral structures. Prenatal detection can be helpful in laying the groundwork for providing counseling to the parents and the planning of management strategies, i.e., opting to terminate the pregnancy, revising delivery plans, and looking towards the postnatal management of the infant. Lymphangioma is a congenital anomaly in which abnormal lymphatic drainages localize to form a benign mass. The abnormality may develop as early as the 6th week of gestation. Because of a developmental defect, the lymphatic system cannot communicate properly with the venous system, leading to dilatation of the afferent lymphatic channels and culminating in an internal buildup of fluid and cyst formation. Though lymphangioma is pathologically benign, it has a tendency to rapidly grow in size and the potential to infiltrate surrounding structures, causing devastating effects and leading to severe morbidity. Lymphangioma can occur in a variety of anatomical locations. The most common area is the neck region 75%), known as cystic hygroma colli or nuchal lymphangioma, followed by the axillary region 20%), retro-peritoneum and abdominal viscera (2%), limbs and bones (2%), and cervico-mediastinum 1%) [% [1]. To0%, retro%, known Case 1:A 36-year-old primigravid woman naturally conceived and attended our antenatal care clinic as a high-risk pregnancy because of elderly gravida. Her prenatal care course was unremarkable. She had no significant familial diseases or underlying medical diseases. Nevertheless, amniocentesis for chromosome study at 16 weeks of gestation was performed because of elderly gravida. The fetus had a normal karyotype; 46, XX. An ultrasound examination before amniocentesis revealed normal structures, including both lower limbs A. An ultCase 2:A 34-year-old primigravid woman had an uneventful antenatal course of pregnancy. The pregnancy was naturally conceived and her prenatal care course was unremarkable. She had no significant familial diseases or underlying medical diseases. Non-invasive prenatal testing  revealed a low risk of aneuploidy. An ultrasound examination for fetal anomaly screening at 20 weeks of gestation showed normal fetal biometry, normal amniotic fluid volume, and normal placenta. Nevertheless, upon surveying the fetus’ anatomy, mild edema of the fetal right thigh was noted A, while Lymphangioma is a congenital anomaly in which abnormal lymphatic drainages localize to form a benign mass. Despite being pathologically benign, lymphangioma has a tendency to rapidly grow in size and the potential to infiltrate surrounding structures, causing devastating effects and leading to severe morbidity. The most common site of lymphangioma is the neck region , whereas lymphangioma of the lower limbs is very rare, accounting for only 2% of cases. The prenatal diagnosis of lymphangioma of the lower limbs has scarcely been reported. This study describes two cases of lymphangioma of the lower limbs, focusing on unique sonographic features and the natural course of rapidly progressive changes. According to this study and previous case reports, the diagnosis of lymphangioma of the lower limbs can be made on the basis of sonographic findings of the avascular subcutaneous complex and multi-septate anechoic cystic lesions in the lower limbs, flank, or pelvis, which can infiltrate visceral structures. The differential diagnosis includes teratoma, isolated hemangiomas, Klippel–Trenaunay–Weber syndrome, Wilms’ tumor, and neuroblastoma.Interestingly, aneuploidy is very common among fetuses with nuchal lymphangioma, with a rate of approximately 70% of affected fetuses, the majority being 45XO . NeverthThe overall prognosis of fetal lymphangiomas is poor, with a mortality rate of 50 to 100%. However, little is known about the prognosis of non-lymphangiomas. Most cases in several reports had poor prognosis, though some had good outcomes . TherefoThis study, together with previous cases reports, illustrates the interesting points of lymphangiomas of the fetal lower extremities as follows: (1) The diagnosis of lymphangioma should be suspected whenever an avascular, complex cystic subcutaneous mass with septations is noted in a fetus. (2) Fetal non-nuchal lymphangioma is unlikely to be associated with aneuploidy and structural anomalies as these differ from nuchal lymphangioma. (3) The extent of growth can affect adjacent structures in the fetal pelvis as well as the perineum and may be associated with poor prognosis. (4) Serial sonographic examinations are important because the early detection of rapid growth of the mass is helpful in laying the groundwork for providing counseling to the parents and the planning of management strategies, i.e., opting to terminate the pregnancy, revising delivery plans, and looking towards the postnatal management of the infant. (5) The first sonographic sign in our two cases were asymmetry of both thighs due to edema of the affected side.


Oryctolagus cuniculus) has emerged in recent years as medical treatments are required with increasing frequency. In this report, we describe a case of cutaneous malignant melanoma occurring in the scrotum in a wild rabbit, with testicular metastases, as an unusual metastatic site only described in human patients to date. Case presentation and gross, histopathological, and immunohistochemical findings are detailed. Descriptions of the biological behaviour of spontaneous tumours may serve to improve current knowledge in animal species and humans in which the same neoplasm occurs. Furthermore, the increasing popularity of rabbits as pets allows for more data on the spontaneous occurrence of cancer.Oncology in pet rabbits (Melanocytic skin tumours have been rarely described in pet rabbits, and exposure to UV light in sparsely haired areas has been hypothesised to play a cancerogenic role. Here, we describe a case of cutaneous malignant melanoma arising from the skin of the scrotum in an 8-year-old male wild rabbit, with testicular metastases as an unusual metastatic site for melanoma reported in humans to date. The tumour was nearly 5 cm in size, firm, and highly pigmented, with multifocal superficial ulcerations and large areas of intratumoural necrosis. The adjacent testis was 1.5 cm, multinodular, and black, obscuring tissue morphology. Histologically, the dermis was expanded by an infiltrative, densely cellular neoplasm composed of nests and sheets of polygonal to spindle neoplastic melanocytes, supported by scant fibrovascular stroma. Neoplastic cells showed intermediate N/C ratio, moderate basophilic cytoplasm, often obscured by abundant brownish granular pigment, and eccentric nuclei with prominent nucleoli. Cellular pleomorphism and nuclear atypia were severe, and high mitotic activity was observed. Diffuse dermal lymphovascular invasion was also observed. The testis was delimited by a thin tunica albuginea, and the parenchyma was largely obscured in its morphology by densely packed neoplastic cells. Seminiferous tubules, lined with a thin basement membrane and containing neoplastic and scattered spermatogenic cells, were occasionally observed. Neoplastic cells within the skin and the testis were positive for HMB-45, Melan-A, and S-100. The growing popularity of rabbits as pets allows for a greater ability to accumulate data on the spontaneous occurrence of tumours in these animals. Furthermore, descriptions of the biological aspects of spontaneously occurring tumours may serve to improve current knowledge in animal species and humans in which the same neoplasm may occur. Oryctolagus cuniculus) has emerged in recent years as they are allowed to age and medical care is required with increasing frequency [Neoplastic disease in pet rabbits  weighing 1.8 kg was referred to a private veterinary hospital in the province of Catania  due to a large neoplasm, highly pigmented, and affecting the skin of the scrotum. The mass had occurred several months earlier, and the owners had reported frequent ulcerations and superficial bleeding over the past two months. The animal showed a deterioration in general health over the last 24 h, showing anorexia, lethargy, and apathy.An 8-year-old, intact, captive-bred, male, crossbreed, and wild rabbit ); Metacam ; butorphanol ; and medetomidine  to provide surgical anaesthesia. An endotracheal tube was placed through an otoscope, and anaesthesia was induced and maintained with isoflurane at 2% . The rabbit was placed in dorsal recumbency, and the surgical area was regularly shaved and disinfected. Skin incisions were made parallel to the tumour, and subcutaneous tissue was bluntly dissected, and the mass was removed with the adjacent, abnormal left testicle. Furthermore, the right testicle was surgical removed, even though it did not show any morphological alterations. Monofilament suture materials (3-0) were used intradermally for closure of subcutaneous and cutaneous layers.The surgically removed scrotal neoplasm and affected left testis were fixed in 10% neutral-buffered formalin and sent to the Department of Veterinary Science of the University of Messina  for histopathological examination.After trimming, tissue specimens were routinely processed for histology, embedded in paraffin wax, and 3 μm thick tissue sections were stained with haematoxylin and eosin (HE) for histopathological evaluation.Immunohistochemistry (IHC) was performed on 3 μm thick, paraffin-embedded tissue sections, using the Ventana BenchMark ULTRA automated platform with cell conditioning 1 for 64 min and preperoxidase inhibition and primary antibody incubation for 16 min at 37 °C. The OptiView DAB IHC Detection kit  was used to detect protein expression of the following primary antibodies: MART-1/Melan A ; anti-Melanosome ; and anti-S100 . All slides were counterstained with Haematoxylin II  and Bluing Reagent  for 4 min at room temperature. Suitable positive controls were used for each IHC reaction, while negative controls were obtained by omitting the specific antisera and substituting PBS for the primary antibody.On gross examination, the tumour was 5 × 3.5 cm in size, highly pigmented, with an irregular cutaneous surface, characterised by diffuse hair loss and multifocal ulcerations, with a firm and compact texture. On the cut surface, the tumour was black in colour, with large areas of intratumoural necrosis. The testis was 1.5 cm in diameter, multinodular, with high pigmentation on the cut surface that completely obscured tissue morphology . Because2). Diffuse dermal lymphovascular invasion was observed, as well as large areas of intratumoural colliquative necrosis and haemorrhage . Nuclei were round and eccentric, with coarsely stippled chromatin and 1–2 prominent nucleoli. Anisocytosis and anisokaryosis were marked, with karyomegaly, and multinucleated cells were occasionally observed. Mitoses were 18 in 10 high-power fields (400× magnification, 2.37 mmmorrhage . NuclearThe testis was delimited by a thin tunica albuginea, and the testicular parenchyma was largely obscured in its morphology by densely packed neoplastic cells. Occasionally, markedly expanded seminiferous tubules, lined with a thin basement membrane and filled with polygonal neoplastic cells mixed with scattered degenerated spermatogenic cells were observed .Based on the histopathological findings, a diagnosis of cutaneous malignant melanoma with testicular metastases was made, and the cell immunophenotype was characterised.Neoplastic cells within the skin of the scrotum and within the testis were positive for HMB-45, Melan-A, and S-100 .In this report, we describe a case of cutaneous malignant melanoma of the scrotum with testicular metastases in a wild rabbit, as an unusual metastatic site only described in human patients to date. The case presentation was detailed, together with pathological and immunohistochemical features. Furthermore, cutaneous melanomas are uncommon neoplasms described in pet and laboratory rabbits, and of note, the New Zealand white rabbit breed appears to be overrepresented ,14,15. RIn veterinary medicine, malignant melanoma commonly occurs in dogs, and the site of occurrence, particularly for the oral cavity, is a crucial determinant of clinical outcome . AdditioIn veterinary medicine, the testicle represents an uncommon anatomical site for metastatic tumours, and metastatic testicular tumours represent an extremely rare event . AlthougIn pet rabbits, primary testicular neoplasms are rarely reported, mainly because males are usually neutered in the first few years of life to prevent reproduction and decrease social aggression . Among tConservative surgery remains the treatment of choice for rabbit cancer if the tumour has not spread to other parts of the body, although anaesthesia-related surgical complications are commonly reported in pet rabbits as well as postoperative complications related to stress and/or pain ,28. ChemHere, we describe a case of cutaneous malignant melanoma arising from the skin of the scrotum in a wild rabbit with testicular metastases, as an unusual metastatic site for cutaneous malignant melanoma, which has so far been reported in human patients. The growing popularity of rabbits as pet allows for a greater ability to accumulate data on the spontaneous incidence of tumours in these animals that are allowed to live natural life spans. In addition, descriptions of the biological aspects of naturally occurring tumours in the rabbit as an animal model may serve to improve current knowledge in other animal species and humans in which the same neoplasm is reported.


Spindle cell tumors, called solitary fibrous tumors (SFTs), are of mesenchymal origin, and can develop in the orbit. As ‘intermediate malignancy’ tumors, only a small percentage show malignant behavior, such as invasion of surrounding tissue.A 57-year-old woman presented with a 19-year history of a giant right orbital mass. Orbital computed tomography (CT) revealed an inhomogeneously-enhancing mass compressing and engulfing the eyeball and optic nerve. She underwent lid-sparing orbital exenteration. Microscopic characteristics and immunohistochemistry (IHC) tests were indicative of a benign SFT. No recurrence was observed at the 4-year follow-up.Early and complete tumor resection is recommended. Solitary fibrous tumors (SFTs), rare spindle cell tumors, were first described by Klemperer and Rabin in 1931 . Since WIn June 2018, a 57-year-old woman presented with a massive, painless mass in the right orbit which had developed over 19 years. As the tumor grew, vision in the right eye gradually deteriorated until it was completely lost approximately 15 years before presentation. Due to budgetary constraints, the patient avoided seeking appropriate medical care.Upon ophthalmological examination, she had a best-corrected visual acuity of no light perception and 20/20 of the right and left eyes, respectively. In the right orbit, a large rubbery mass stretched horizontally from the bridge of the nose to the lateral canthus, and vertically from the eyebrow to the center of the cheek. The upper eyelid covering the tumor was pushed forward due to the large size of the mass. The vessels of the eyelids were dilated and tortuous Fig. A. The paOrbital CT revealed a huge mass in the right orbit measuring 10.6 × 9.5 × 11 cm. After administration of contrast material, inhomogeneous enhancement and vascular-like enhancing structures were observed. The mass was closely related to the internal rectus muscle. The eyeball appeared totally engulfed and compressed by the large mass and was displaced anteromedially. In the bone structures, no evident erosion or extension damage was found; however, the temporal orbital wall was observed to be slightly thinner than anticipated  Fig. D-2E, B-cThe patient had good cosmetic results and exhibited no symptoms of recurrence during the 4-year follow-up.Patients with orbital SFTs are predominantly middle-aged adults , 5. TherSymptoms and signs are related to tumor size and location. Patients with orbital SFTs often present with proptosis, eyelid swelling, blepharoptosis, diplopia, ocular motility restriction, and a slow-growing painless palpable mass in the periocular area . HeadachThe reported radiological features of SFT are non-specific. Orbital SFTs are seen as well-circumscribed soft tissue tumors with moderate to intense enhancement on CT images, which are attributed to the high vascularity within the tumor . AlthougBenigh SFTs typically exhibit low mitotic activity and lack nuclear pleomorphism and/or necrosis . HistomoMicroscopic features alone are insufficient to confirm the diagnosis of SFT and further IHC analysis must be conducted. SFT cells typically stain positive for specific markers, such as CD34 and STAT6, and variably positive for vimentin, S-100 protein, progesterone receptors (PR), P53, BCL-2, and Ki-67 . CD34, aThe clinical behavior of SFTs is variable . The majComplete resection is necessary for adequate local tumor control. Other adjuvant treatments, including preoperative transarterial embolization, radiotherapy, and chemotherapy for the treatment of recurrent orbital SFTs, have been reported in individual cases , 34, 35.In conclusion, we present an extremely rare case of orbital SFT which was definitively treated with surgical excision. To the best of our knowledge, this is the largest orbital SFT reported thus far. The tumor exhibited an aggressive course of eyeball compression and had typical histomorphological and IHC features indicative of a benign SFT. Therefore, careful, long-term follow-up is necessary.


A 3-month-old male infant was bought to the emergency department with almost 3 weeks of projectile, bilious emesis after each feed. On presentation, he was cachectic and severely dehydrated and had a palpable olive in the epigastric region. Hypertrophic pyloric stenosis was excluded by abdominal ultrasound. The barium meal demonstrated a massively distended stomach, absence of distal gas, and triple bubble sign. Given the radiological findings, the patient underwent an emergency exploratory laparotomy and a congenital duodenal web (CDW) was discovered. While the clinical picture suggested the diagnosis of HPS, barium meal eventually revealed congenital duodenal web. To our knowledge, no other cases of palpable olive mass as the presenting sign of proximal bowel obstruction have been published. Congenital duodenal web is a reported cause of intestinal obstruction in neonates, and its symptomatic incidence is 1 : 10,000 to 1 : 40,000 [th percentile weight for age), a product of consanguineous healthy parents, had an uneventful prenatal history with a normal antenatal ultrasound, and is developmentally age appropriate.A 3-month-old male infant presented with 20-day history of bilious and projectile vomiting after each feed with substantial weight loss and lethargy. He is full-term with a birth weight of 3 kg . He had a scaphoid abdomen and visible peristalsis transversely across the epigastrium from the infant's left to right   Videos  and 3. T) Videos  and 3.+ 4.3 mmol) and chloride (Cl− 96 mmol/L) VBG were remarkable for uncompensated metabolic alkalosis ph: 7.54 pCO2: 35.7 mmHg cHCO3: 31.1 mmol/L cBase 7.4 mmol/L. Ultrasound of the abdomen showed evidence of a markedly distended stomach and the first part of the duodenum with no signs of pyloric stenosis. Barium meal revealed an abnormally distended stomach, paucity of distal gas, and triple bubble sign consistent with a diagnosis of congenital duodenal obstruction  and serum chemistry values including potassium , including pylorospasm and gastroesophageal reflux . As in oWith the current advances in medicine, in utero detection of congenital duodenal obstruction through prenatal ultrasound is possible. Some prenatal markers include polyhydramnios, double bubble sign, and associated congenital anomalies. However, nearly half of the cases are not diagnosed until after birth and a third of them are identified later on in infancy. Therefore, clinicians must have a high index of suspicion when regurgitations persist, even low volume, in nonseptic neonates to avoid missing the diagnosis. We thereby stress the importance of taking a careful and complete history and performing a thorough physical examination as the foundation of diagnosis.Delayed diagnosis of proximal bowel obstruction may lead to dehydration and clinically significant metabolic disturbances due to the ongoing loss of gastric secretion with persistent emesis. Thus, in view of the history, clinical examination, and venous blood gas findings, it is highly likely that this patient had been suffering from the consequences of the delayed diagnosis of congenital duodenal obstruction. Kilbride et al. reported that infants with delayed diagnosis of congenital duodenal obstruction have had poorer outcomes and that 12.5% of them died as a consequence of metabolic and pulmonary disturbances related to proximal bowel obstruction .Finally, although proper assessment of vomiting is challenging to paediatricians, particularly in a nonseptic newborn, this report aims at reminding clinicians to be vigilant when emesis persists in the newborn, regardless of the volume, and that it is particularly vital to further evaluate those before discharge to avoid delays in operative intervention.This case suggests that a palpable olive mass in the epigastric region may not be a distinct disease characteristic of hypertrophic pyloric stenosis but may instead be a presenting sign of proximal bowel obstruction. We also emphasize the importance of a thorough history taking and physical examination, followed by rapid and effective diagnostic techniques, such as point-of-care ultrasound. Moreover, it is vital that clinicians maintain an open diagnostic mind when approaching newborns with persistent vomiting as it could mimic less serious diseases.


Intra-abdominal gossypiboma, a cotton-based retained foreign body after an abdominal surgery, is associated with various clinical manifestations and complications. Its infrequent occurrence and unpredictability make its early diagnosis particularly challenging. We herein present an atypical case of intra-abdominal gossypiboma mistaken for a jejunal tumor.A 33-year-old female presented to the emergency room with an acute episode of progressive abdominal pain and distention, nausea, and vomiting for 20 hours. She had undergone an urgent cesarean section due to fetal tachycardia seven years prior. The initial diagnosis of small bowel obstruction (SBO) due to a jejunal tumor was established by computed tomography. Subsequent to successful medical management of the SBO, a laparoscopy-assisted resection of the mass and the adherent jejunal segment was conducted, culminating in a primary side-to-side jejunojejunostomy. Examination of the excised tissue revealed an approximately spherical fibrous mass, 6 × 6 × 5 cm in dimension, embedded in the jejunal wall, housing a 20 × 20-cm gauze. Postoperative recovery and routine follow-up ensued without complications.In light of this case, the need for clinicians to maintain an elevated awareness and suspicion of gossypiboma should be accentuated when evaluating an intra-abdominal mass, especially in patients with a prior history of high-risk laparotomy. Laparoscopic surgery stands out as a technically proficient and minimally invasive strategy for diagnosing and treating intra-abdominal gossypiboma. Besides, it is imperative to emphasize the importance of meticulous surgical procedures and postoperative protocols to prevent such oversights, reaffirming the need for consistent intraoperative counts and checks of surgical items. Gossypiboma, derived from “gossypium” (Latin for cotton) and “boma” , was first documented by Wilson in 1884 . By defiThe abdominopelvic cavity was the most frequent body cavity where the foreign body left (54%), followed by vagina (22%), thorax 7%), and others  . Notably%, and otIn alignment with the principles of the CAse REport (CARE) guidelines , we hereIn November 2022, a 33-year-old Han Chinese female patient was referred to our emergency room with progressive abdominal pain  and distention, nausea, and vomiting for 20 hours. She had undergone an urgent cesarean section because of fetal tachycardia 7 years previously. There were no underlying comorbidities or family history of malignancies with her. On admission, she was subfebrile with slightly abnormal vital signs . Physical examination showed abdominal distention, slight rebound tenderness in the periumbilical area, and a painless palpable mass in the lower quadrant with metallic bowel sounds. The result of digital rectal examination was unremarkable.Blood tests indicated a systemic infection with an elevated total leukocyte count , percentage of neutrophils , and C-reaction protein . Contrast-enhanced computed tomography (CT) of the abdomen and pelvis demonstrated dilated proximal bowel loops and multiple air-fluid levels due to a 6.1 × 5.7-cm, approximately spherical, well-delineated heterogeneous cystic and hyperdense jejunal mass and mild intestinal volvulus, without indicators of intestinal ischemia or necrosis  met the inclusion criteria –35. OverThe optimal approach to dealing with this iatrogenic surgical complication is prevention. For surgeries associated with high risk of retained surgical sponge (RSS) as mentioned above, repeated sponge counting at the key time point  should be advocated and emphasized . All theAs mentioned above, intra-abdominal gossypibomas are associated with unpredictable clinical presentations and the possibility of a long interval between the previous surgery and current episode . It may Furthermore, gastrointestinal endoscopy, as well as cystoscopy and ureteroscopy, can be applied to identify transmural migration and sometimes remove the RSS , 39. ForIn conclusion, our case report accentuates the need for clinicians to maintain an elevated awareness and suspicion of gossypiboma when evaluating an intra-abdominal mass, especially in patients with a prior history of high-risk laparotomy. Laparoscopic surgery stands out as a technically proficient and minimally invasive strategy for diagnosing and treating intra-abdominal gossypiboma.The original contributions presented in the study are included in the article/The studies involving humans were approved by Biomedical Ethical Committee of West China Hospital, Sichuan University. The studies were conducted in accordance with the local legislation and institutional requirements. The participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.YH: Writing – original draft, Project administration. WY: Writing – original draft, Project administration. WD: Writing – original draft. QM: Writing – review & editing, Data curation, Investigation, Visualization. TY: Writing – review & editing, Formal Analysis, Software. YY: Writing – review & editing, Formal Analysis, Software. YL: Writing – review & editing, Supervision. BZ: Supervision, Writing – review & editing, Conceptualization, Funding acquisition, Methodology. MZ: Supervision, Writing – review & editing, Conceptualization, Funding acquisition, Methodology.


Chordoma is a rare, aggressive tumor that is believed to originate from notochord remnants. It can occur anywhere from the clivus to the sacrum and often recurs even after resection and radiotherapy. We present a unique case that initially suggested a different pathology based on imaging and presentation but was found to be a chordoma on gross and pathological analysis.An 11-year-old girl presented outpatient for scoliosis evaluation and was found to have what appeared to be a right L4 peripheral nerve sheath tumor on MRI, causing dextroconvex scoliosis. She underwent a gross total resection via a retroperitoneal approach and was found to have what appeared to be an extraosseous, extradural, extra-spinal canal lumbar chordoma. Immunohistochemical features on surgical pathology were consistent with chordoma. The patient was referred to radiation oncology for adjuvant radiotherapy and pediatric hematology/oncology for recurrence monitoring.Our case is the first to present in such a manner, was shown to be external to the spinal canal, encasing the nerve root, and was the first such case in a pediatric patient. We reviewed the growing body of literature on spinal extraosseous chordomas and their characteristics within the pediatric patient population. We also reviewed chordoma pathogenesis theories as well as current and future treatment options. Chordomas are malignant, aggressive tumors derived from notochord remnants that occur most commonly in the sacrococcygeal region and the clivus, with a small subset occurring in extraosseous and/or extra-axial sites.The diagnosis of conventional chordoma is made with brachyury immunostaining on pathology.Pediatric chordoma may present with back pain and scoliosis with symptoms disproportionate to CT findings.Pediatric chordoma may also present as an extraosseous, extradural, extra-spinal canal lumbar mass, mimicking a peripheral nerve sheath tumor.Chordomas are malignant, aggressive tumors derived from notochord remnants that occur most commonly in the sacrococcygeal region and the clivus, with a small subset occurring in extraosseous and/or extra-axial sites. They are characterized histologically by the presence of brachyury , 2, 3, 4An 11-year-old Caucasian female was referred to neurosurgery for evaluation of scoliosis and a 1-year history of back pain. The pain was right-sided and radiated down the lateral aspect of her right lower extremity and was not relieved by over-the-counter medications, including acetaminophen, naproxen, and ibuprofen. She also described occasional tingling of her right toes and the feeling that her right knee would “give out” when walking. She denied experiencing any bowel and bladder dysfunction. She also denied left-sided pain and any inciting event or injury.At the time of initial presentation, her non-contrasted CT lumbar spine and her thoracic spine X-rays showed dextroconvex scoliosis with a Cobb angle of 15°, with no other abnormalities noted by the radiologist. Given the mildness of her scoliosis, her back and the right leg pain was unlikely to be secondary to her scoliosis alone. Thus, a lumbar MRI was ordered to rule out a herniated disc or other causes of nerve impingement. Her MRI showed a 5.3 cm T2/STIR hyperintense, lobulated, heterogeneous enhancing lesion widening the right L4-L5 neuronal foramen with scalloping of the posterior right L4 vertebral body and extending into the paraspinal soft tissues, most suggestive of a right L4 peripheral nerve sheath tumor (shown in Fig. The MRI findings and plan for surgical resection were discussed with the patient and her mother on follow-up. Given the lateral location of the mass, a retroperitoneal approach was planned with the assistance of pediatric surgery. Intraoperatively, we found that the mass appeared to encase the right L4 nerve root, which was spared along with any other neural elements we encountered. The mass also had a very friable capsule and contained old hematoma. We dissected the mass from the L4 nerve root with great difficulty and followed it down to the vertebral body, achieving a gross total resection (confirmed on postoperative imaging, shown in Fig. The final pathology on the permanent sample showed that the mass was consistent with conventional-type chordoma. Histologic sections of the soft tissue mass of the right L4 vertebra show a lobular pattern with fibrous septa separating the lobules. The lobules consist of abundant basophilic myxoid stroma with short cords of eosinophilic epithelioid cells, which are admixed with vacuolated cells resembling lipoblasts and physaliphorous cells. The neoplastic cells show diffuse and strong nuclear positivity for brachyury immunostaining, immunoreactivity to pan-cytokeratin, and epithelial membrane antigen, as well as focal immunoreactivity to desmin and S-100. The tumor cells show retained nuclear staining of BRG-1 and INI-1. These features are shown in Figure Upon receiving the final pathology results, the patient was referred to pediatric hematology/oncology and radiation oncology. Adjuvant proton beam radiation was chosen to minimize acute and long-term toxicity, and the patient completed a full course. She received 74 Gy administered in 37 fractions over 51 days and routine postradiation monitoring for recurrence. She was last seen for follow-up at 3 months post-operation and reported that she has been doing well since surgery, with an improvement in her pain and weakness. However, scoliosis films at 3 months showed worsening of her curvature with a Cobb angle of 51°. Because she is still growing and undergoing radiation treatments, we referred her for bracing temporarily. We plan for a close follow-up for safe scoliosis surgery as soon as possible.To our knowledge, this is the first report of a pediatric, extraosseous, extradural chordoma that was also found to be external to the spinal canal and encasing a nerve root. Extraosseous chordomas in the pediatric population are exceedingly rare, even more so for those in the lumbar spine. In a review of the literature to find similar cases, we specifically examined thoracolumbar or sacrococcygeal chordomas in the pediatric population. Of those we found (shown in Table Given the findings on preoperative imaging, chordoma was low on our differential, and we counseled the patient's family on treatment for a peripheral nerve tumor. However, if we had concerns that the mass was a chordoma prior to surgery, we would have had a different conversation regarding treatment, postoperative course, and prognosis. For extraosseous chordomas, it has been shown that complete resection is more often attainable, likely due to lack of bony involvement , 23, 24.Another unique aspect of our case was that we reported histology and immunohistochemical staining results, which have yet to be reported in other pediatric extraosseous, extradural cases of chordoma. Currently, there are two theories on chordoma pathogenesis: either originate from notochord remnants or undergo a malignant transformation of a benign notochordal tumor into a chordoma , 31, 32.With the difficulty of attaining complete remission in up to half of known cases and the adverse outcomes of repeated RT treatments, investigations into immunotherapy and chemotherapy treatments have been done. Many chemotherapeutic agents have been tried in the past but only anecdotal successes have been noted, and only Al-Rahawan et al.  reportedWe present the unique case of an extraosseous, extradural, extra-spinal canal chordoma in a pediatric patient who initially presented for scoliosis and back pain. Based on imaging characteristics, the mass was initially thought to be a peripheral nerve sheath tumor, but it was confirmed to be a chordoma on surgical pathology to our surprise. The patient was treated with GTR and adjuvant RT, the primary treatment recommendation for this pathology. While extraosseous chordomas are less likely to recur and have a better prognosis, more medical treatment options are needed for this patient population. Thus far, EGFR inhibitors have shown promise, but developing other therapeutic agents is hindered by drug delivery challenges and few mutational targets.The Internal Review Board of the University of Oklahoma Office of Human Research Participant Protection does not require ethics approval for case reports or series that include less than 5 patients. No ethical approval was needed in accordance with national guidelines. Written informed consent was obtained from the patient's legal guardian for publication of this case report and accompanying images. All patient information and images were de-identified before publication.The authors have no conflicts of interest to declare.No funding sources were utilized in creating this manuscript.Helen Shi, MD, and Joanna Gernsback, MD, were responsible for article conception. Helen Shi, MD, Khairuddin Memon, MD, and Joanna Gernsback, MD, were responsible for the treatment of the patient. Figures and tables were created and edited by Benjamin Lee, BS, and Audrey Grossen, BA. Literature review was performed by Benjamin Lee, BS, and Audrey Grosse, BA. Sara Abu Mehsen, MD, Zhongxin Yu, MD, and Kar-Ming A. Fung, MD, PhD, were responsible for description of pathology. Khairuddin Memon, MD, was responsible for the description and interpretation of radiological findings. Benjamin Lee, BS, and Audrey Grossen, BA, were responsible for the drafting of the article. Helen Shi, MD, Benjamin Lee, BS, and Audrey Grossen, BA, were responsible for all article revisions. Benjamin Lee, BS, Audrey Grossen, BA, Helen Shi, MD, Sara Abu Mehsen, MD, Zhongxin Yu, MD, Kar-Ming A. Fung, MD, PhD, Khairuddin Memon, MD, and Joanna Gernsback, MD, were responsible for reviewing the submitted version of manuscript. Joanna Gernsback, MD, supervised the entire process of manuscript writing, editing, and submitting.All relevant data generated and analyzed during this study are included in this article. Further questions and inquiries can be directed to either the corresponding or submitting author.


This case report presents a unique clinical scenario involving a 32-year-old woman with a unicornuate uterus and a rudimentary horn, leading to recurrent fetal malpresentation. The patient, gravida 3, para 1, presented at 36 weeks of gestational age with contractions and vaginal bleeding. Clinical evaluation revealed a breech presentation and nonreassuring fetal heart tracings. An emergency cesarean section was performed, during which a unicornuate uterus with a rudimentary horn was identified and fused to the main uterine cavity on the left posterior aspect. The rudimentary horn bore a functional left tube and ovary. The surgical procedure was successful, resulting in the delivery of a healthy baby girl. This case underscores the importance of meticulous preoperative assessment, multidisciplinary collaboration, and informed consent in managing complex uterine anomalies to ensure optimal maternal and fetal outcomes. Uterine anomalies constitute a diverse spectrum of congenital malformations that can profoundly influence the course of pregnancy. Among these, the unicornuate uterus with a rudimentary horn is a rare and intriguing anomaly that presents unique challenges in obstetric management . This caUterine anomalies, though infrequent, encompass a diverse range of structural deviations from the normal uterine anatomy. These anomalies can be classified into various categories, such as fusion defects, septal malformations, and agenesis, each with its distinct impact on reproductive outcomes . The uniRecurrent fetal malpresentation, a common consequence of uterine anomalies, can complicate pregnancy and childbirth. Malpresentation refers to the deviation of the fetal presenting part from the vertex position, increasing the risks of prolonged labor, obstructed delivery, and potential birth trauma. In this context, the unicornuate uterus with a rudimentary horn introduces an additional layer of complexity to the understanding and management of recurrent fetal malpresentation .This case report presents a comprehensive analysis of the clinical course of a patient with a unicornuate uterus and a rudimentary horn, where the anomaly played a pivotal role in the recurrent presentation of fetal malposition. This case serves as a testament to the importance of understanding and addressing uterine malformations in obstetric practice and underscores the significance of sharing such clinical experiences to enrich the medical community's knowledge and expertise in managing challenging cases.The patient, a 32-year-old woman, gravida 3, para 1, had a history of one previous cesarean section. She had been married for seven years and had a three-year-old male child delivered through cesarean section due to breech presentation. Additionally, she experienced one spontaneous abortion at eight weeks of gestational age. Her medical history was unremarkable, with no history of gestational hypertension, diabetes mellitus, or other associated comorbidities.At 36 weeks of gestational age, the patient presented to the emergency department complaining of contractions and slight vaginal bleeding. On physical examination, several pertinent findings were noted. The linea nigra and striae gravidarum were visible on the abdominal skin. A previous cesarean section scar was evident. Fetal movements were visible upon inspection. The fundal height of the uterus reached the xiphisternum, indicating appropriate uterine growth. A longitudinal fetal lie was detected, with the fetal head positioned at the fundal pole and the buttocks at the podalic end, consistent with breech presentation Figure . Fetal hThe patient's most recent ultrasound scan revealed a single live intrauterine fetus at 36+3 weeks of estimated gestational age. The estimated fetal weight was 2.5 kg. The breech presentation was confirmed, and the placenta was situated anteriorly with adequate amniotic fluid levels. The thickness of the cesarean scar was measured at 3.5 mm.Given the clinical presentation and the findings from antenatal imaging, the patient was promptly prepared for an emergency cesarean section. Informed consent was obtained, and the patient was informed of the risks associated with the surgical procedure. These risks included those related to the cesarean section, the possibility of needing obstetric hysterectomy, potential placenta accreta, the requirement for blood transfusion, NICU admission for the newborn, and the option for tubal ligation during the surgery.The medical team involved in the patient's care included the attending obstetrician, a pediatrician, and an anesthetist. The pediatrician was informed about the case to ensure prompt assessment and care of the newborn. At the same time, the anesthetist was briefed about the patient's medical history and the potential need for specialized care during anesthesia administration.Under spinal anesthesia, a Pfannenstiel incision was made to access the uterus. The surgical team delivered a baby girl weighing 2.3 kg. Apgar scores were recorded at 8 and 9 at one and five minutes, respectively. The cesarean section was performed successfully and without complications.During the surgery, a thorough examination of the uterus revealed a unicornuate uterus with a rudimentary horn. The rudimentary horn was flattened and located on the right side of the unicornuate uterus Figure . It was The presented case underscores the complex nature of congenital uterine anomalies and their significant implications for pregnancy outcomes. In particular, the unicornuate uterus with a rudimentary horn presents intricate challenges that demand thorough understanding and careful management. Congenital uterine anomalies arise from anomalies in the development of Müllerian ducts during fetal life. These anomalies exhibit varying prevalence rates across different populations. While affecting 1-10% of the general population, the prevalence rises to 2-8% in infertile women and 5-30% in those with a history of miscarriages ,7. The pThe rarity of a unicornuate uterus, found in only 0.1% of the general population, presents unique challenges. Women with this anomaly experience poor reproductive outcomes, including a live birth rate of merely 29.2%, a high prematurity rate of 44%, and an ectopic pregnancy rate of 4% . The comIn the case presented, a rudimentary horn in conjunction with a unicornuate uterus introduces further complexities. The rudimentary horn is often non-communicating, and its occurrence is associated with ectopic pregnancies. The unique mechanism of pregnancy occurrence within a non-communicating rudimentary horn through transperineal migration of sperm or fertilized ovum is rare, with an estimated frequency of approximately 1 in 76,000 pregnancies. However, this anomaly poses a substantial risk of uterine rupture, necessitating vigilant monitoring .This case report underscores the paramount significance of meticulous preoperative assessment and multidisciplinary collaboration in managing complex uterine anomalies. Successful surgical intervention hinges upon careful navigation through intricate anatomy. In this case, the patient's sufficient blood supply to the dominant uterine cavity and the possible presence of normal endometrium within it likely contributed to her successful full-term pregnancy without significant fetal compromise.The presented case vividly illustrates the complexities and challenges associated with uterine anomalies in obstetric management, specifically the unicornuate uterus with a rudimentary horn. The recurrent fetal malpresentation, in this case, underscores the crucial role that uterine anatomy plays in shaping pregnancy outcomes. By meticulously recounting the patient's journey from presentation to surgical intervention, we have highlighted the importance of thorough preoperative assessment, multidisciplinary collaboration, and informed decision-making. Managing uterine anomalies requires a comprehensive approach integrating obstetric, surgical, anesthetic, and neonatal considerations. The identification of a unicornuate uterus and its associated rudimentary horn during a scheduled cesarean delivery showcases the necessity for obstetricians, gynecologists, pediatricians, and anesthetists to work in tandem to achieve favorable outcomes for both the mother and the newborn. This case report underscores the significance of vigilant antenatal care and imaging in early anomaly detection, allowing for tailored management strategies. The successful cesarean delivery with careful surgical navigation and attention to the unique uterine anatomy is a testament to the importance of surgical precision and patient-centered care in rare uterine malformations.