Achilles tendon ruptures often occur during physical activity where the tendon is frequently stressed. Although rare, rupture can also result from electric shock.We present the case of a 63-year-old female who presented with pain in the lower leg after enduring an electric shock. She was diagnosed with a ruptured Achilles tendon based on physical exam and ultrasound.This case highlights an uncommon mechanism for a relatively common injury. Because Achilles tendon ruptures are frequently misdiagnosed, clinicians need to be aware of unusual causes and use tools at their disposal to ensure timely and accurate diagnosis. Despite being the thickest and strongest tendon of the human body, the Achilles tendon (AT) is the one that most often completely ruptures.A 63-year-old female presented to the emergency department (ED) with pain just posterior to the left medial malleolus after experiencing an electric shock. She had stepped on an exposed wire on her way to bed after a shower and felt a shock followed immediately by a left calf spasm. She denied falls, syncope, or additional blunt trauma to her leg. Initially, she thought it was a minor cramp that would resolve. Although she was able to ambulate, persistent discomfort and new-onset bruising behind the malleoli prompted her to go to the ED the next day.On evaluation, her vitals were unremarkable. There was decreased plantarflexion both actively and when her calf was squeezed. An area of ecchymosis was present above her calcaneus and posterior to her medial malleolus. There were no burns or wounds noted at the electrocution site. Her labs, including a complete blood cell count, basic metabolic panel, and creatine phosphokinase were unremarkable. Her electrocardiogram revealed a normal sinus rhythm.The overall clinical picture was suspicious for AT injury, prompting the use of point-of-care ultrasound to evaluate the integrity of the tendon in both the sagittal and transverse planes. The images obtained supported the diagnosis of a complete AT rupture , and 3.The patient was placed in a posterior short leg splint with her foot in plantarflexion and discharged with crutches. She was instructed to follow up with an orthopedic surgeon.Achilles tendon ruptures are a relatively common injury with an estimated incidence between seven and 40 per 100,000 people per year.6The most frequent mechanism of injury is pushing off the forefoot while extending the knee, a movement commonly performed in sports such as basketball, soccer, volleyball, and gymnastics.1What do we already know about this clinical entity?Achilles tendon ruptures frequently occur during physical activity.What makes this presentation of disease reportable?Achilles tendon rupture resulting from an involuntary muscle contraction secondary to an electric shock is an uncommon and unusual mechanism of injury.What is the major learning point?Achilles tendon ruptures are frequently misdiagnosed. Simple physical exam maneuvers and ultrasound can help make the diagnosis.How might this improve emergency medicine practice?Familiarity with uncommon causes of Achilles tendon rupture and competency with point-of-care ultrasound may expedite time to diagnosis and prevent delay in proper management.While the exact etiology for tendon rupture is unclear, there are intrinsic and extrinsic factors that can predispose an individual to tendinopathy and injury.In a noteworthy scenario, our patient suffered a rupture from an electric shock. To the best of our knowledge, there has only been one other case report of AT rupture from an electric shock. In that case, a woman experienced a shock while blow drying her hair, causing her to jerk her leg uncontrollably and rupture her tendon.9Approximately 10,000 patients present annually to the ED for evaluation after experiencing an electric shock, which typically occurs at home or work.12The classic presentation of an AT rupture is sudden pain in the leg that may be associated with a pop or snap. Patients may report feeling as if they were struck in the calf or heel. They may struggle with walking and pushing off the affected leg. Approximately 25% of AT ruptures are initially misdiagnosed, frequently as ankle sprains.Clinical diagnostic tests that can be used to evaluate for AT rupture include palpating for a defect along the tendon, the calf squeeze test, Matles test, Copeland test, and O’Brien test. The calf squeeze test and Matles test have the highest sensitivity and, unlike the Copeland and O’Brien tests, do not require additional equipment.13If clinical testing is ambiguous or limited due to pain or body habitus, further imaging, such as clinical ultrasound (CUS) and magnetic resonance imaging (MRI), can be considered. Although MRI is the gold standard for imaging, it is more expensive and time consuming than CUS and less likely to be available.15To perform ultrasound evaluation of the AT, the patient should lie prone. The fibers of the tendon should be evaluated in both a sagittal and transverse plane. For assessment in the sagittal plane, the probe should be placed directly on top of and in line with the tendon. For assessment in the transverse plane, the probe should be placed perpendicular to the tendon. Using a high-frequency linear probe, the examiner should start scanning at the calcaneal tuberosity where the AT inserts and then slide the probe proximally to the myotendinous junction. A normal AT will have fibers that are linear, regular, and without interruption. A ruptured tendon may have fibers with thick irregular edges, interruptions along the course of the tendon, or anechoic areas that may represent hematoma.Once the diagnosis is made, the emergency clinician should splint the patient’s affected extremity in the equinus position (plantarflexion), provide crutches and analgesics, and refer the patient to an orthopedic surgeon for follow-up ideally within two days.Although Achilles tendon ruptures are common, not all result from obvious mechanisms. Uncommon and atypical mechanisms, such as an electric shock, can also lead to tendon injury. Emergency clinicians need to remain astute, so that they do not miss the diagnosis and delay proper treatment. If there is doubt regarding the diagnosis, ultrasound can help confirm the presence of an AT rupture.


The aim of this case report is to show that late diagnosis of vertebral osteoid osteoma gives rise to structural scoliosis which sometimes requires long-term management.We report a case of an osteoid osteoma in the thoracic spine associated with structural scoliosis. We describe a 14-year-old boy who complained chronic nightly left back pain and scoliosis. Spine’s X-ray was reported thoraco-lumber scoliosis without bone lesion.MRI as well as technetium-99 m total body bone scan and a computed tomography scan revealed a bony lesion in the upper left joint of T11 vertebra consistent with the diagnosis of OO. Anatomopathological study of the resection piece confirmed the diagnosis of OO. Surgical excision of the tumor resolved pains, but scoliosis needed an orthopedic treatment for 1 year.Through this case, it has been demonstrated that late diagnosed vertebral OO can be the cause of structural scoliosis. Clinical and radiological results indicate that OO resection is an effective and safe method of treatment.IV. The occurrence of scoliosis in older children and adolescents is most often idiopathic. Nevertheless, an etiology must be systematically ruled out in the face of the painful nature of scoliosis. The therapeutic strategy must be carried out according to a schedule taking into account two factors: scoliosis and its etiology. OO is one of the etiologies to think about.We report the case of a 14-year-old patient who consulted for spinal deformity. The patient reported nocturnal spinal pain which had progressed for 5 months. When standing, the shoulders were horizontal, the line of the thorns was deviated in italic “S” to the right Fig. . A CT scOO is a benign bone lesion with high incidence at the age between 10 and 20 . The lumWe encountered an unusual case of OO of the thoracic spine presenting as scoliosis that was difficult to diagnose. If the OO nidus exhibits close proximity to the neural canal, resection of the tumor is an effective and safe treatment method. The duration of symptoms and the age at the presentation time are the most important factors in the development of associated vertebral rotation, a structural scoliosis with a high magnitude of curve.


Nutcracker syndrome is a clinical condition in which there is compression of the left renal vein in its path between the abdominal aorta and the superior mesenteric artery. This phenomenon can cause abdominal or low back pain and hematuria. It is a rare clinical entity, although probably underdiagnosed. The diagnosis is essentially clinical and based on imaging, but necessarily a diagnosis of exclusion.We present the case of a 21-year-old boy who came to the Emergency Department with hematuria and low back pain. After exhaustive study and exclusion of other possible clinical entities, the diagnosis was confirmed to be nutcracker syndrome. Despite its usually benign expression, this entity should not be forgotten in the diagnostic process of cases of hematuria and low back pain, especially in young patients. Nutcracker syndrome (NCS) is a rare clinical condition caused by compression of the left renal vein (LRV) by the superior mesenteric artery (SMA) as it passes between the SMA and the abdominal aorta (AA) (anterior NCS) . AnotherThe extrinsic stenosis of the renal branch can cause different symptoms, with the most common being the absence of symptomatology; however, it can cause macroscopic hematuria, proteinuria, renovascular hypertension, flank pain, dyspareunia, dysmenorrhea, and pelvic varicose veins .There appears to be a bimodal distribution of cases. Most patients with complaints of hematuria and low back pain are teenagers or young adults who tend to be slender. The second peak incidence is in middle-aged women who present mostly with pelvic congestion syndromes .Diagnosis is challenging and is usually made after excluding other more common causes as there are no specific clinical diagnostic criteria .Imaging examinations are necessary to confirm the syndrome, and Doppler ultrasonography is the most widely used method -3. TreatA 21-year-old male, with no relevant previous pathological history and no usual medications, presented to the Emergency Department for lower back pain with one week of evolution and macroscopic hematuria with one day of evolution. He denied dysuria, pollakiuria, abdominal pain, nausea, vomiting, fever, and a history of recent trauma or vigorous exercise. He also reported that three years earlier he had an episode of mild macroscopic hematuria with spontaneous resolution, which did not motivate the use of health services.Physical examination revealed a bilaterally negative renal Murphy’s sign and no other changes. His blood pressure was normal. Analytical results showed mild microcytic and hypochromic anemia and no increase in acute inflammatory markers Table . Type IIUltrasonography showed no morphological changes in the kidneys or bladder.He was admitted to the Internal Medicine ward and started analgesic treatment with resolution of hematuria and low back pain in three days. Diagnostic investigation excluded hereditary thrombophilia, hyperhomocysteinemia, and antiphospholipid syndrome.Doppler ultrasonography showed a change in the caliber of the LRV between the aorta and the SMA after the origin (relation between the diameter upstream of the compression (6.8 mm) and in the compression (2.9 mm): 2:35), suggestive of NCS Figure .Once all other potential causes of hematuria and low back pain were excluded and Doppler ultrasonography findings were suggestive of NCS, a computerized tomography (CT) urogram and CT angiography were performed to confirm the diagnosis. CT urogram and CT angiography showed compression of the LRV in its path between the AA and SMA Figure  and an aThe patient was evaluated by the Urology team and was discharged with conservative treatment. Two months after this episode, he was observed at an outpatient clinic, remaining asymptomatic, without new episodes of hematuria and low back pain.The clinical presentation of NCS ranges from asymptomatic in most cases to various symptoms, including hematuria and abdominal pain . HematurThe diagnosis is hard and requires excluding other more common causes. Confirmation of the diagnosis requires imaging examinations. Doppler ultrasonography is the first-line diagnostic test with 69-90% sensitivity and 89-100% specificity . AlthougWhen Doppler ultrasonography is elucidative and the symptoms are mild, CT angiography is not essential. Important findings demonstrated by CT angiography are the Beak sign, a severe form of narrowing of the LRV at the aortomesenteric portion, and an angle between the SMA and the aorta <41° .Treatment can be conservative, surgical, medical, or endovascular. Conservative treatment is suggested for patients with tolerable symptoms, such as mild hematuria; surgical treatment is indicated in cases of severe haematuria associated with anemia, functional renal failure, severe pelvic pain, or ineffectiveness of conservative treatment after 24 months of clinical follow-up .NCS is a rare clinical condition that should be considered in patients with left flank or lumbar pain and isolated hematuria. The diagnosis is essentially clinical and based on imaging, but necessarily a diagnosis of exclusion. Doppler ultrasonography is the first-line imaging examination but CT angiography can be used to confirm the diagnosis. Treatment is usually conservative. In this case, knowledge of the syndrome was essential for its suspicion and subsequent confirmation, avoiding performing more invasive procedures.


The inverted papilloma is an uncommon unilateral nasosinusal benign tumor. The clinical picture presents nonspecific signs and symptoms, such as unilateral nasal obstruction, anosmia and headache. The diagnosis is established by anamnesis, physical exam, computed tomography and magnetic resonance imaging. Treatment is essentially surgical. This report has the objective of presenting an uncommon bilateral inverted nasal papilloma and making a literature review. The first report of this type of tumor in the nasal cavity was made by Ward et al. in 1854,,The inverted papilloma (IP) is a rare and benign nasosinusal tumor, bearing an incidence of 0.75 to 1.5 cases per 100 thousand inhabitants/year3–8. Representing 0.5 to 4% of all nasal tumorsIP originate from the nasal cavity lateral wall, and it secondarily affects the maxillary, ethmoidal, frontal and sphenoid sinuses. The primary involvement of the paranasal sinuses is extremely rare, happening only to 5% of the casesIPs are 4 to 5 times more frequent in males, with greater prevalence in Caucasians, between their 5th and 6th decades of life,,,,Although benign, the inverted papilloma is characterized by an aggressive growth, great invasion potentialSigns and symptoms are unspecific and may cause unilateral nasal obstruction, epistaxis, olfactory disorders and recurrent rhinosinusitisDiagnosis is carried out by history taking, otorhinolaryngological exam and image exam. CT scan (CT) and magnetic resonance image (MRI), of the nasal cavities and paranasal sinuses are important to assess the size, extension and anatomical relations of the tumor, and they also help differentiate them from other nasosinusal diseases and also in cases of orbital and/or intracranial complications.,,The main differential diagnoses are antrochoanal polyps, nasal cavity squamous polyp, fibrous dysplasia, gigantic cells granuloma and other neoplasiaTreatment is surgical. Surgical techniques and access must be broadly studied and individualized.A 51 year-old male complained of nasal obstruction for one year, associated with hyposmia and mucopurulent rhinorrhea. He did not complain of epistaxis, headache or visual impairment.The otorhinolaryngological exam revealed inferior nasal turbinate moderate hypertrophy and mucosa paleness, with mucopurulent secretion. Oroscopy and otoscopy did not show alterations.Video-nasofibroscopy showed a large amount of mucopurulent secretion, associated with a lobulated and polypoid tumor, of rough surface and firm, occupying the lateral wall of both nasal cavities.Nasal and paranasal sinuses CT scan revealed a hyperdense image occupying both nasal cavities, ethmoidal, frontal, sphenoid and right maxillary sinuses, enlarging the ostium-meatal complex in both sides and involving the rhino-pharynx. There were no signs of bone lysis .Figure 1The patient was then submitted to nasosinusal endoscopic surgery with bilateral total ethmoidectomy, frontal sinusectomy, sphenoidectomy and maxillary antrostomy with tumor extraction in two resection blocks together with most of the lamina papyracea bilaterally.The pathology exam revealed a bilateral inverted papilloma with no signs of cell atypia. It was carried out in blocks in another institution that confirmed our diagnosis of a bilateral inverted papillomaPost-operative evolution was satisfactory. Control was carried out every two months in the first 6 months after surgery through nasal endoscopic exam that showed a re-epithelized nasal cavity with no signs of disease recurrence. We then ordered nasal CT scan and MRI, but the patient has not returned yet.,,,The inverted papilloma, also called Schneider, papilloma, Ewing papilloma, transitional cells papilloma, epithelial papilloma villus cancer, transitional cells benign tumor and papillomatosis, is a benign sinusal tumor with undefined etiology,,,The name inverted is justified by the endophilic growth of the superficial epithelium to inside the adjacent stroma,The main theory on IP etiology proposes that Schneider membrane, which forms the nasosinusal tract mucosa, originates from the ectodermal invasion of the olfactory placoid. This membrane would then suffer a number of structural changes, causing a greater predisposition for neoplastic differentiation,Histologically, the papillomas can be divided in three types: fungiform or exophytic, which stems from the anterior septum and has the macroscopic aspect of a common wart, the columnar, which stems from the lateral nasal wall and the middle meatus, and the inverted, previously described, with three fold higher chance for malignization if compared to the columnar papilloma,,The clinical aspect of the IP is unilateral nasal obstruction (98%), rhinorrhea (17%), epistaxis (6%), anosmia (4%), headache and frontal pain. The tumor can extend to outside the nasal cavity in 7% of the cases, in 3% of the cases it extends to the nasopharynx and less than 2% to the pterygopalatine and intracranial fossaThe diagnosis must start by a detailed anamnesis, investigating environmental exposure, noxious habits, allergies and associated diseases, and by complete otorhinolaryngological exam. Endoscopic and radiological (CT and MRI) exams are fundamental for tumor study and diagnosis. Biopsy together with histopathology establish the diagnosis, however must not be carried out before prior histopathology exam in order to rule out the presence of a vascularized tumor (juvenile nasoangiofibroma) or lesions extending to the central nervous system ,,Nasal polyps, 25 times more frequent than IPs2, presents respiratory mucosa with stromal edema and eosinophillic infiltrate, and must constitute a differential diagnosis, just like the antrochoanal polyp, squamous polyp, nasal vestibule polyp, fibrous dysplasia, giant cells granuloma and neoplasias,Recently, Krouse,Nasal cavity and paranasal sinuses CT scan suggest IP when there is an image of soft tissue present from the middle meatus all the way to the adjacent maxillary antrum, through an enlarged maxillary ostium,MRI provides a more accurate assessment of tumor boundaries and implantation site, differentiating it from the adjacent inflammatory tissue and it is also the exam of choice for post-operative follow up,,,,Treatment is surgical. In the past, we used lateral rhinotomy, middle face degloving or medial maxillectomy with tumor en bloc resection. During the 80's, with nasosinusal endoscopic and microendoscopic surgeries, the procedures became less invasive; however, there has been an increase in tumor recurrence rates, counter-indicating the endoscopic approach when performed without the external accessRadiotherapy (RT) is controversial. Weissele,,,,Tumor recurrence usually happens in the first two years; however, in 17% of the cases it happens after 6 years of evolutionThe very rarity with which inverted papillomas affect the nasal cavities  makes this report so important, especially considering post-operative follow up because, despite being a benign tumor, the inverted papilloma is a very aggressive tumor. Surgical treatment must be careful, with previous radiological study of tumor boundaries, so as to carry out the best technique and thus, remove it completely and reduce recurrence risks.


Recurrent bleeding due to mild trauma, tongue biting, poor oral hygiene, and expansion of the lesions causing difficulty in chewing, swallowing, or speaking is common sequelae of vascular lesions of the tongue. Cryosurgery is a technique for destroying lesions by the rapid freezing method. The necrotic tissue that results is allowed to slough naturally after the lesion is frozen. Cryosurgery has been utilized in the medical and dentistry field to treat many lesions as it is successful and easy to perform. It has a number of advantages, including ease of use, minimal infection rate, and no bleeding intraoperatively. This report presents a case of a 15-year-old female patient with a hemangiomatous lesion on the tongue that was successfully treated by cryosurgery using a liquid nitrogen spray. The patient demonstrated complete resolution of the lesion with good wound healing during the one-year follow-up period. Cryosurgery is a technique for destroying lesions by rapidly freezing them in situ. The necrotic tissue that results is allowed to slough naturally after the lesion is frozen . The ensCryotherapy is considered the gold standard for treating many lesions in the oral cavity, such as trigeminal neuralgia, mucocele, leukoplakia, pyogenic granuloma, hemangioma, actinic cheilitis, human papillomavirus lesions, fibromas, and lichen planus. It is also considered a supplemental treatment in cases of osseous lesions with high recurrence rates, such as ossifying fibroma, keratocystic odontogenic tumor, central giant cell lesions, myxoma, and ameloblastoma . Tissue A 15-year-old female patient reported to the Department of Oral and Maxillofacial Surgery with a chief complaint of an asymptomatic blue-purplish lesion on the right lateral border of the tongue. History revealed that the onset of the lesion was five months ago and progressed slowly to the present size. There was no family history of malignancy and no episodes of recurrent bleeding from the lesion. Extraoral examination revealed no gross facial asymmetry, and the cervical lymph nodes were not palpable. Intraoral examination revealed an asymptomatic, purplish, sessile, nodular, firm-to-touch lesion measuring about 2.5 cm in its largest diameter on the right lateral border of the tongue  of the neck with contrast, and digital subtraction angiography were done, which revealed the lesion to be a low-flow lesion. MRI of the neck revealed a mildly enhancing, fairly defined, diffusion-restricting, T2/STIR hyperintense lesion with internal hypointensity  in the right anterior lateral aspect of the tongue involving the intrinsic and extrinsic muscles Figure . ThawingThe lesion appeared whitish in color immediately after the procedure, indicating the freezing process, and edema and erythema formation were visible in the treated area a few minutes later after thawing. The dentition was spared from contact with the liquid nitrogen, and it was ensured that the spray did not cause any liquid to collect and drain into the pharynx. Four sessions of liquid nitrogen cryotherapy were delivered at periodic intervals. The patient was periodically reviewed postoperatively at the first, third, sixth, and ninth weeks following each session of cryotherapy Figures -7.The patient experienced slight pain in the first few days after surgery, which was treated with oral analgesics for three days. During the healing process, there were no signs of infection. The patient was clinically healthy during the one-year postoperative follow-up period with no sensory abnormalities in the tongue, aberrant speech, or difficulty swallowing and no symptoms of lesion recurrence Figure . HoweverHemangiomas commonly occur in early childhood and infancy . It is aHemangiomas are hamartomatous lesions that are divided histologically into three types based on the size of the proliferating vascular spaces: capillary, cavernous, and mixed . The cavCryosurgery has been used successfully in a variety of specialties such as general surgery, ophthalmology, oncology, hepatology, dermatology, urology, and oral and maxillofacial surgery -7,11-13.To increase tissue damage, cryotherapy's basic technique includes rapid cooling, gradual thawing, and repeated freezing . A closeCryotherapy is regarded as a simple, rapid, and easy-to-execute procedure as a result of the prompt appearance of erythema and edema in the cryogenic exposed area. Following cryosurgery, there have been no reports of infection, respiratory issues, or adverse responses to the cryogenic agent. Mild pain was noted in cases, and it was treated primarily with peripherally acting analgesics . CryosurCryosurgery is a safe, comfortable, cost-effective procedure and readily accepted by patients. Liquid nitrogen cryotherapy can be utilized as the treatment of choice for vascular tumors such as hemangioma as it has many advantages over conventional surgery. Cryotherapy is currently the most preferred and effective therapeutic option for many benign mucosal lesions.


Mucosal melanomas represent about 1% of all melanoma cases. Primary malignant melanoma of the esophagus (PMME) is a rare and deadly condition, with only about 339 cases reported in the literature. Esophageal melanoma usually presents with progressively worsening dysphagia, and patients often present late in the disease course. Esophageal melanoma can be treated with surgical resection, chemotherapy, targeted therapy, or immunotherapy depending on the stage and tumor mutations. However, due to the rarity of the disease, no trials have been performed to deliver a gold standard of treatment. We present the case of a 76-year-old female who was diagnosed with metastatic primary malignant melanoma of the esophagus and underwent treatment with nivolumab, a PD-1 receptor antagonist. Melanomas are malignant tumors arising from pigment cells called melanocytes that most often occur on the skin . The incThe patient is a 76-year-old female nonsmoker with no reported medical history who presented to our hospital with six months of chronic, worsening, nonradiating epigastric pain that was 10/10 in severity that was not associated with food intake. The symptoms initially began as difficulty with swallowing liquids and early satiety. They progressed to difficulty with solids and eventually to sialorrhea, as she was unable to swallow and had to spit liquids out. During this period, she experienced nearly 40-pound weight loss with associated anorexia and fatigue. On our initial clinical interaction, she was noted to be cachectic, expectorating constantly, but in no acute distress. There was no palpable lymphadenopathy.Her initial laboratory results were remarkable for normocytic anemia secondary to anemia of chronic disease with components of iron deficiency and marked hypoalbuminemia. Hemoglobin was 10.3 g/dL, and albumin was 2.6 g/dL. CT scan of the abdomen was significant for an intraluminal esophageal mass measuring 4.9 × 4.2 cm axially and 12 cm in the craniocaudal dimension, originating in the subcarinal region and extending to the gastroesophageal junction. The mass compressed but did not occlude the right atrium and lower pulmonary veins. The patient underwent an esophagogastroduodenoscopy (EGD) for further evaluation, which showed a pigmented and lobulated esophageal mass starting at 21 cm from the incisors, with the endoscope unable to pass beyond 40 cm from incisors with the esophageal mass still being visualized (Figure After evaluation by a multidisciplinary team, a combined decision was made by the family to initiate palliative chemoradiation. Subsequently, the patient underwent placement of a gastrostomy tube to maximize the quality of life given dysphagia with severe malnutrition and anticipated radiation, which could likely worsen the dysphagia. After discussion at an interdisciplinary melanoma tumor board, the patient was started on single-agent nivolumab given an Eastern Cooperative Oncology Group performance status score of 4. She received two doses of nivolumab 480 mg, four weeks apart, with no improvement of symptoms, and continued decondition, suggesting progressive disease not responding to treatment. The patient never received radiation because she missed her appointment with the radiation oncologist. Thereafter, an informed decision was made by the family to transition to hospice care. Death occurred two months after the last dose of nivolumab. The time from initial presentation to initiation of treatment was about one month, and the death occurred about 4.5 months after diagnosis.The most common presenting symptom in PMME, as noted in the existing literature and shown in several case reports, is dysphagia ,5-9. ThiEGD can reveal a pigmented mass, such as our patient had . An ulceHistologically, the diagnosis of melanoma was supported by positive staining with SOX10, HMB-45, Melan A, and vimentin immunostains and positive staining with Fontana Masson special stain. HMB-45 is an indicator of active melanosome formation  and, witThe staging of cancer helps guide its treatment. PMME is treated with surgical resection if the disease is at a limited stage ,8,10, anPrimary malignant melanoma of the esophagus is a rare form of esophageal cancer. Prognosis is poor and depends, among others, on the stage at diagnosis. There is a high risk of recurrence even in those who had a primary surgical resection. Those with metastatic disease and good performance status are candidates for immunotherapy with PD-1 checkpoint inhibitors, although the efficacy of these drugs is variable. Further research is warranted to determine optimal treatment protocols.


Intraoral melanoacanthoma is a rare, reactive pigmented lesion that is mostly seen in black individuals with a tendency to occur more frequently in younger females. The color of melanoacanthoma may vary from brown to black, and it is commonly seen as a solitary lesion in the buccal mucosa. This lesion requires no treatment, and no malignant potential has been observed to date. The clinical presentation of multifocal oral melanoacanthoma (MOMA) (the rate of growth and the recurrence) is usually diagnostic. On the other hand, solitary oral melanoacanthoma might be difficult to diagnose, and a biopsy should be performed and examined by a well-trained oral pathologist. Here, we report two cases of MOMA with an unusual location in one case. Intraoral melanoacanthoma is a rare, mixed epithelial pigmented lesion that is mostly seen among blacks with a tendency to occur more frequently in younger females . These lHere, we report two cases of multifocal oral melanoacanthoma (MOMA) with an unusual location in one case. In addition, we review and summarize most of the reported cases of MOMA in the literature.Case oneA 36-year-old Saudi female patient presented to the Oral and Maxillofacial Surgery Department complaining of black pigmentation on her left and right buccal mucosa. She reported that the lesions had appeared suddenly for two weeks and had been gradually increasing in size. The lesions were not associated with pain or any change in sensation. She also denied any constitutional symptoms, including fever, chills, night sweats, appetite changes, and weight loss. The patient was a non-smoker. In addition, her medical history did not reveal any chronic illness or allergy, and there was no history of medication use. The patient had a history of dental implants and prosthetic crowns on the right and left posterior lower teeth a long time ago. Extraoral examination showed no significant findings.Intraoral examination revealed a brown-black pigmented lesion on the left and right buccal mucosa, with the left side being more diffuse Figure . The lesHistological examination showed a wedge of oral mucosa surfaced by acanthotic and spongiotic stratified squamous epithelium with numerous dendritic melanocytes throughout the thickness of the epithelium. Increased melanosis was seen at the basal and para-basal level with mild chronic inflammatory cell infiltration in the superficial connective tissue Figure .A two-week follow-up of the patient showed a new lesion on the left lateral side of the tongue with the same color as the buccal lesions but smaller in size Figure . The eigCase twoA 21-year-old black Saudi female dental student presented to the Oral Medicine Clinic complaining of rapidly growing brown discolorations on multiple areas in her mouth for three months. The patient was a non-smoker, medically fit, and did not take any medication. The extraoral examination was normal. Intraoral examination revealed multiple, diffuse, smooth brownish-black macules with irregular erythematous border on the right and left buccal mucosa Figure . FurtherMishima and Pinkus were the first to describe melanoacanthoma in 1960 as a benign mixed skin tumor of basal and prickle cell keratinocytes and pigment-laden dendritic melanocytes [Most of the previously reported cases of MOMA have been summarized in Table The lesion usually presents as brown/black macules of variable sizes, which might be raised or elevated. It usually involves two sites rather than being diffuse (three sites and more). The two most commonly involved sites are the hard palate and gingiva, followed by bilateral buccal mucosa. The least documented site to be involved is the floor of the mouth . Some asThe clinical presentation of MOMA (the rate of growth and the recurrence) is usually diagnostic, and a biopsy is rarely needed. On the other hand, solitary OMA might be challenging to diagnose based on clinical presentation only, especially with no history of onsite and recurrences. In such cases, the lesion might be mistaken as malignant melanoma based on the size of the lesion, border irregularity, and rapid growth; hence, a biopsy is indicated. In one of our cases, the diagnosis was made based on histological examination, while, in the other case, the diagnosis was made based on the history of recurrence and presentation of the lesions. However, even with histological examination, the diagnosis can be easily missed if not reviewed by a well-trained practitioner in the field of maxillofacial pathology. Histologically, the lesion has unique features of showing numerous dendritic melanocytes extending between the spinous epithelial cells with acanthosis and spongiosis of the epithelium.MOMA has been documented in association with local factors such as dental prosthesis ,13, chroDifferential diagnosis of MOMA includes drug-induced melanosis, systemic disease , syndromes , smoker melanosis, and racial pigmentation. Malignant melanoma is usually not included in the differential of MOMA as it develops as a solitary lesion and not as a multifocal lesion.However, some clinicians may hesitate to finalize the diagnosis based solely on the clinical examination. Recently, dermoscopy has been introduced to oral medicine clinics, allowing proper magnification and visualization of the morphologic features of oral pigmented lesions non-invasively . The proMelanoma has variable histological presentation which can mimic carcinomas, neuroendocrine tumors, sarcomas, lymphomas, and others . TherefoOnce the diagnosis of MOMA is confirmed, no further treatment is required as it is a self-limited process. Argon plasma coagulation has been used in one study to treat MOMA with no recurrence after a one-year follow-up . No maliIt is crucial that dental practitioners have sufficient knowledge and approach to diagnose MOMA. This will minimize the number of incision biopsies that have to be obtained from patients to reach a diagnosis. Additionally, it will spare the patient from unnecessary pain and expenditures for a lesion that can be easily identified based on clinical presentation.


Spontaneous haemoperitoneum (SH) is a rare but life-threatening condition with several well-recognized causes. SH often occurs in anticoagulated patients and requires urgent treatment to prevent mortality. SH is rarely associated with infective endocarditis (IE). To date, there are no reported cases of a haemorrhagic mesenteric abscess causing haemoperitoneum. We present the case of a warfarinized 30-year-old intravenous drug user with IE 2 weeks post-revision of a metallic valve who reported abdominal pain and subsequently suffered haemodynamic collapse. Abdominal computed tomography and damage-control laparotomy revealed haemoperitoneum secondary to an actively bleeding mesenteric nodule which was resected. Histopathology confirmed a haemorrhagic mesenteric abscess representing a rare sequela of IE and a novel cause of SH. Given that the incidence of IE appears to be increasing in people who inject drugs in Australia, the general surgeon should be aware of this clinical entity and the need for urgent intervention to prevent catastrophic sequelae. Metastatic abscesses secondary to septic embolization from infective endocarditis (IE) has been well reported in the literature. However, very few cases of mesenteric abscesses secondary to IE have been reported. To our knowledge, no cases of haemorrhagic mesenteric abscesses causing haemoperitoneum have been published. This case highlights rare and potentially lethal sequelae of IE in a warfarinized patient.Staphylococcus aureus bacteraemia. She had a history of intravenous drug use and previous mechanical aortic valve replacement (AVR). Echocardiogram as part of her initial workup demonstrated aortic root abscess with evidence of septic emboli in the brain, spleen and bilateral kidneys. She underwent revision of AVR and warfarin was re-commenced. She remained an inpatient for ongoing intravenous antibiotics. After 2 weeks, she complained of severe abdominal pain, shortness of breath and dizziness. Over a period of hours, she became progressively tachycardic and hypotensive. A haemoglobin taken several hours after onset of pain was 76 g/L, representing a 25 g/L decrease from her baseline. WCC was elevated at 15 and INR was subtherapeutic at 2.2. Abdominal computed tomography (CT) with IV contrast demonstrated contrast material extravasating within the small bowel mesentery consistent with active bleeding  is defined as intra-abdominal bleeding in the absence of trauma . It is aThe incidence of IE in Australia is increasing, particularly among IV drug users . IE can Indeed, to our knowledge, there is only one published report of a mesenteric abscesses secondary to IE where the proposed pathogenesis was infarction of the mesentery, with subsequent bacterial proliferation and abscess formation . Given tHaemorrhagic mesenteric abscess appears to be a unique cause of SH. Given that the incidence of IE appears to be increasing in people who inject drugs in Australia, the general surgeon should be aware of this clinical entity and the need for urgent intervention to prevent catastrophic sequelae. Our case highlights SH as an important differential in the warfarinized patient and introduces haemorrhagic mesenteric abscess as a unique cause of SH in patients with IE.


Portal and splenic vein thrombosis are uncommon, potentially fatal post-operative complications following splenectomy. These thrombotic events may be asymptomatic or present with non-specific symptoms. Therefore, imaging is important for diagnosis. The risk of thrombosis is linked to spleen size, pre-operative thrombocytopenia and surgical technique. We present the case of a 40-year-old man who underwent curative and diagnostic laparotomic splenectomy following chronic thrombocytopenia and concurrent splenomegaly who subsequently developed extensive portal and splenic vein thrombosis.Teaching Point: Portal and splenic vein thrombosis after splenectomy is a relatively uncommon but important diagnosis in which radiology has a pivotal role. Portal and splenic vein thrombosis are potentially fatal complications following splenectomy, with a study-dependent incidence ranging from 0.8% to 55%, and with a more recent study from 2018 reporting a rate around 8% 12. This co34A 40-year-old man presented with thrombopenia, found during a routine pre-operative workup for an unrelated procedure, and splenomegaly. The patient was known to have thrombopenia for 10 years. A previous workup for thrombocytopenic purpura was performed, proving negative as immunoglobulin therapy was unresponsive. An FDG-PET-CT only showed hypermetabolic splenomegaly. A splenectomy with diagnostic and therapeutic intention was suggested to exclude low-grade lymphoma, but no action was taken at the time. At the current presentation, an open splenectomy was performed. There was no perioperative complication. The enlarged spleen had a weight of 1.89 kg. Postoperatively, the patient presented with a 10-day persisting fever despite antibiotics; he was referred for a computed tomography (CT) exam to exclude late postoperative complications or infection. The CT exam, performed 15 days postoperatively, revealed splenic vein thrombosis, extending into the intrahepatic segment of the left branch of the portal vein  and 1b–cAs most cases are clinically asymptomatic or non-specific, radiology is crucial in diagnosing splenic and portal vein thrombosis. Contrast-enhanced CT exam is superior to ultrasonography 5. Preope121Portal or splenic vein thrombosis treatment is controversial. In some centres, treatment consists of intravenous heparin or low-molecular weight heparin (LMWH) followed by oral anticoagulants for 3–6 months, while others recommend simultaneous LMWH and warfarin followed by warfarin for 3–6 months 5. In acuProphylaxis is also the subject of discussion, post-operative anticoagulation being standard in some, but not all, institutions 5.Portal and splenic vein thrombosis are uncommon and often asymptomatic post-splenectomy complications, and contrast-enhanced CT exam is the preferred imaging modality. If the patient presents with pre-operative risk factors for thrombosis, including a splenic vein diameter >10 mm or a spleen weight estimated >500 g, a follow-up exam to diagnose and treat this severe condition could be taken into consideration [


To our knowledge, this is one of the rare literature reports of an ileal GIST complicated with mesenteric gangrene. We reported successful surgical treatment. Infection of GIST is an extremely rare complication that should be treated with no delays to avoid rupture and peritonitis. Ileal gastrointestinal stromal tumors are rare. Mesenteric gangrene is an extreme rare complication. Diagnosis is difficult. Surgical treatment should not be delayed.  Question:What is shown in the intraoperative view?Answer: Ileal gastrointestinal stromal tumor causing mesenteric gangrene.3 and the C reactive protein was raised to 65 mg/L. Computed tomography (CT) scan showed irregular wall thickening of the small intestine proximal to a hydroaeric mesenteric collection. We performed a laparotomy and found an ileal tumor (Figure A 45‐year‐old male patient was presented to the emergency department with acute abdominal pain. On examination, he had a temperature of 38.5°C. There was periumbilical tenderness. Laboratory studies showed a white blood count of 13800 cells/mmr Figure . We perfThe histopathologic examination showed spindle cells type GIST of the small intestine. There was a proliferation of cells with elongated nuclei and eosinophilic cytoplasm arranged in fascicles and expressing Dog1 in the immunohistochemistry Figure . It was Postoperatively, the patient was treated with Imatinib. He had an uneventful recovery with a follow‐up of 6 months. To the best of our knowledge, this is one of the rare literature reports of an ileal GIST complicated with mesenteric gangrene. We reported successful surgical treatment. Infection of GIST is an extremely rare complication that should be treated with no delays to avoid rupture and peritonitis.The authors declare that they have no conflict of interest.Hazem Beji and Mahdi Bouassida collected the data and involved in writing the manuscript. Slim Zribi edited the manuscript. Hassen Touinsi involved in validation.This work meets ethical and legal guidelines.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


Neck tumors in newborns are very rare. Teratomas usually include all three germ cell layers, as well as tissues that are not native to the anatomic site of genesis. Teratomas of the head and neck make up a smaller percentage of congenital teratomas. Because of the external compression that oropharyngeal or neck masses produce, they can cause serious airway obstruction. In addition, the larynx or trachea may have an underlying lesion. We presented a mature, 1-day-old newborn with an isolated giant neck tumor and difficulty breathing. The intubation was successfully done and the entire mass was completely removed. Early neonatal life is explored to emphasize this challenge briefly with several interesting instances, including prenatal diagnosis, therapeutic alternatives, and ex-utero intrapartum therapy (EXIT) techniques. •Teratoma should be evaluated as part of the differential diagnosis of neck masses, and confirmed or excluded before deciding on definitive therapy, despite its rarity. It is critical to distinguish between the various diseases because they have an impact on prenatal counseling, antenatal care, and postnatal care . DiffereIn children, teratomas are the most prevalent type of germ cell tumor . The sacThe ectoderm, mesoderm, and endoderm make up their histology. Airway obstruction is a common complication of neck teratomas . In addiTeratomas of the head and neck can be highly vascularized, resulting in life-threatening blood loss . To secuNeck tumors in newborns are very rare. Teratomas of the head and neck make up a smaller percentage of congenital teratomas. We presented a mature, 1-day-old newborn with isolated giant neck tumor and difficulty breathing successfully managed with surgıcal excision.2A 1-day-old baby was brought to our hospital with a large neck mass and difficulty breathing. We urgently underwent endotracheal intubation due to difficulty breathing and were transferred to the neonatal intensive care unit. The mass was not discovered in utero, however, a polyhydramnios was mentioned during the gestational ultrasonography (US) scan. During the pregnancy, the mother did not consume any nicotine, alcohol, or medications. A large neck tumor was discovered during a physical examination .Fig. 1ClChest radiography, ultrasound of the abdomen, and echocardiogram were unremarkable. The tumor extended from the auricle to the clavicle on both sides of the neck. The US revealed a mass lesion containing hypoechoic, cystic areas of approximately 80 × 55mm in the middle and left side of the Neck. A neck Magnetic Resonance Imaging (MRI) was recommended and showed a massive well-defined multi-loculated complicated neck mass with considerable mass impact. The solid component has intermediate T2 and T1 signal intensities, whereas the cystic component exhibits high T2 and low T1 signal intensities. The esophagus and airway have been shifted .Fig. 2AxOnce the baby has been stabilized in the neonatal intensive care unit,  surgical intervention was undertaken. A left-sided incision was made over the tumor, and the tumor was securely attached to the tracheal cartilages in the midline. During the procedure, the tracheal cartilage was intact. The large mass was completely removed, and no other complications were encountered during the procedure A,B,C,D. On postoperative day 7, a gastrostomy tube was used to keep the patient fed. On Day 45, the patient was discharged. During a one-year follow-up, the patient remained well, growing normally, and had no evidence of recurrence.This work has been reported in line with the SCARE 2020 criteria .3Most teratomas appear in the sacrococcygeal region, whereas, teratomas in the neck region comprise 2%–9% of all congenital teratomas ,7. TeratThe tumor compresses the oropharyngeal structures, causing airway obstruction in the fetus, which impairs swallowing and causes polyhydramnios. The tumor has the potential to displace and force the fetal lungs upward, resulting in severe lung hypoplasia, it's also one of the reasons for this newborn's death. Fortunately, our patient does not suffer from lung hypoplasia. The EXIT procedure is a surgical technique that permits a newborn to remain on placental support while the airway is secured .Unfortunately, there was no accurate prenatal diagnosis in our baby, and the tumor was only identified by ultrasound after the mother underwent a cesarian section. As a consequence, our patient was unable to undergo an EXIT procedure due to post-delivery detection. Despite this, intubation was performed to secure the airway, and mechanical ventilation was initiated.If the airway is secured prior to surgery, patients with cervical teratoma as an isolated anomaly can do well after postnatal excision. Patients with pulmonary hypoplasia are unable to be oxygenated, in addition to having a large cervical mass, and they die of respiratory insufficiency despite maintaining an adequate airway .It's important to get a diagnosis as soon as possible when dealing with a newborn with a large lump in the neck region. Cystic hygroma, hemangioma, lipoma, dermoid cyst, congenital goiter, cervical meningocele, occipital encephalocele, bronchogenic cyst, branchial cleft cyst, thyroglossal duct cyst, and teratoma are all possible diagnosis for a congenital neck tumor ,11.4Teratoma should be evaluated as part of the differential diagnosis of neck masses, and confirmed or excluded before deciding on definitive therapy, despite its rarity. Early neonatal life diagnosis is crucial to emphasize this challenge briefly with several interesting instances, including prenatal diagnosis, therapeutic alternatives, and ex-utero intrapartum therapy (EXIT) techniques.According to our hospital rule, Ethical approval is only required in articles but not case reports.There is no funding source for this study.All authors contributed toward writing, analysis, drafting, and revising the paper and they gave final approval of the version to be published, and agree to be accountable for all aspects of the work.The parent of the patient previously consented to use their medical and surgical data in this study. This study was carried out in accordance to the Helsinki Declaration contents.1.Name of the registry: Not applicable2.Unique Identifying number or registration ID: Not applicable3.Hyperlink to your specific registration (must be publicly accessible and will be checked): Not applicableIsmaıl Mohamed Ali.Not commissioned, externally peer reviewed.The authors declare that there is no competing interest related to the study, authors, other individuals or organizations.


Spontaneous cerebral dissections in children are rare and can be associated with the formation of pseudoaneurysms. The management of these pseudoaneurysms is controversial as they can be treated either by surgery or endovascular techniques. On rare occasions, they may spontaneously thrombose. We present a 12-year-old male without a history of trauma who developed an intracerebral hematoma secondary to a ruptured pseudoaneurysm of the middle cerebral artery that showed a rapid spontaneous complete thrombosis. Five days after his initial diagnostic cerebral digital subtraction angiogram, a follow-up study showed no evidence of the previously observed pseudoaneurysm. Two months later, a computed tomographic angiography of the brain showed no evidence of the pseudoaneurysm. Thrombosed pseudoaneurysms should be closely followed by neuroimaging studies as they may subsequently recanalize. Spontaneous cerebral dissections in children are rare . These dThe management of these cerebral pseudoaneurysms is controversial as they can be treated either by surgery or endovascular techniques . On rareA healthy 12-year-old male with no significant past medical history or drug allergies was playing basketball at his aunt's house when he suddenly developed a severe headache accompanied by nausea and vomiting. He denied any head trauma to his aunt and mother. He was given an over-the-counter analgesic and went to bed, awakening in the early morning hours with another episode of a sudden severe headache that subsided without medication. He went back to bed, but a couple of hours later, his mother noticed that he had wet his bed. When awakened, he was drowsy and confused. His mother took him to the local hospital. He had two tonic-clonic seizure episodes immediately after his arrival and was intubated to protect his airway. A head computed tomographic (CT) scan without contrast showed a 6.0 x 3.2 x 3.0 cm right frontal intracerebral hematoma with rupture into the frontal ventricular horn extending into all the ventricles, causing a 1.2 cm shift of the midline  showed a dissection at the M1 segment of the right MCA with an associated 7 mm dissecting pseudoaneurysm Figure . DespiteFive days later, the patient's condition improved, opening his eyes to the pain and making flexion withdrawal of the extremities to painful stimuli. Repeated cerebral DSA on the fifth day showed no evidence of the previously observed pseudoaneurysm Figure . A trachMost cerebral pseudoaneurysms occur after head trauma, severe infections, congenital vessel defect, hereditary connective tissue disorders, and allergic arteritis ,10,11. SThe true natural history of intracranial pseudoaneurysms is unclear, and complete spontaneous thrombosis is considered an uncommon event ,8,9,16. Surgical options for pseudoaneurysms include direct clipping, suturing, wrapping-clipping, ligating the parent artery, and trapping with or without bypass ,5,16,17.Spontaneously thrombosed pseudoaneurysms should be closely followed by neuroimaging studies to detect if partial or complete recanalization has occurred ,9. In chSpontaneous cerebral dissections or pseudoaneurysms in children are rare. Their management remains controversial, and in most cases, they are managed by surgery or endovascular techniques. In some cases, conservative management can produce thrombosis of the pseudoaneurysm. However, patients with thrombosed pseudoaneurysms must be closely followed by neuroimaging studies as they may subsequently recanalize.


Although infantile hepatic hemangioma and hepatic mesenchymal hamartoma are relatively common in benign pediatric liver tumors, coexistence of the two tumors is rare. Placental mesenchymal dysplasia is also a rare disorder. We report the case of a baby girl born after a pregnancy complicated by placental mesenchymal dysplasia, who developed both infantile hepatic hemangioma and hepatic mesenchymal hamartoma.The patient was born at 32 weeks and 5 days of gestation for impending placental abruption, weighing 1450 g. Liver tumors, composed of both hypervascular solid and large cystic lesions, were detected after birth and markedly increased to create abdominal distention within 9 months. Diagnostic imaging suspected the coexistence of infantile hepatic hemangioma and cystic hepatic mesenchymal hamartoma. Following propranolol therapy for infantile hepatic hemangioma and needle puncture of a large cyst, the cystic lesions and adjacent hypervascular lesions were partially resected via laparotomy. Pathological findings confirmed the coexistence of hepatic mesenchymal hamartoma and infantile hepatic hemangioma, which had no association with androgenetic/biparental mosaicism. The postoperative course was uneventful, and the tumor had not regrown after 3 years.Although the coexistence of infantile hepatic hemangioma and hepatic mesenchymal hamartoma associated with placental mesenchymal dysplasia is extremely rare, the pathological and pathogenetic similarities between these disorders suggest that they could have derived from similar embryologic origins rather than being a mere coincidence. Further follow-up is required, with careful attention to the potential for malignant hepatic mesenchymal hamartoma transformation. Liver tumors comprise only 1–2% of all pediatric tumors, and one-third are benign . The coePlacental mesenchymal dysplasia (PMD) is a rare disorder characterized by placentomegaly, dilatation of chorionic vessels, and areas of villous cystic changes . The incA healthy 30-year-old gravida 1, para 1 woman was diagnosed with PMD at 15 weeks of gestation with prenatal fetal ultrasound (US); her pregnancy was otherwise uneventful. At 32 weeks and 5 days of gestation, emergency cesarean section was performed for impending placental abruption. A baby girl was born with a birth weight of 1450 g and Apgar scores of 8 and 9 at 1 and 5 min, respectively. The delivered placenta was enlarged (23.6 × 23.5 × 6.5 cm) and heavy (1209 g), and the maternal surface had multiple cystic villi  revealed coexisting multifocal cystic and solid components in both hepatic lobes  was started to promote regression of the hemangioma component. Her clinical condition temporarily improved and the volume of some hemangioma components was reduced, however; the propranolol effect was limited, and the cystic lesion returned to its original size. Laparotomy was performed at age 10 months. During the procedure, the two largest cystic tumors, located in the right and left hepatic lobes, respectively, were excised along with their adjacent small solid tumors. Postoperative recovery was uneventful, and propranolol treatment was continued until 1 year of age. At the age of 4 years, the patient was doing well, and follow-up MRI showed no HMH regrowth and a trend to decreasing IHH volume.The left lobe cyst measured 115 × 105 × 70 mm, and the right lobe cyst was 100 × 62 × 48 mm. On the cut section, both lesions contained spongy, white, and solid components with multicystic spaces that were incompletely separated by loose, fibrous septa and filled with mucinous material Fig. A. MicrosThe coexistence of IHH and HMH is rare, and there have been no reports of PMD associated with these two pathologies. The patient described in our report was well for some time after birth and received regular follow-up. However, at the age of 9 months, the rapid growth of IHH and HMH caused abdominal distension and poor oral intake. The combination of propranolol therapy for IHH and partial tumorectomy for HMH resulted in extensive tumor volume reduction without post-surgical regrowth. Although the etiology of IHH, HMH, and PMD has not yet been fully elucidated, their simultaneous occurrence in our patient suggests that some common etiological mechanisms underlie the three pathologies.It has been previously speculated that HMH could contain hemangiomatous elements. However, to the best of our knowledge, the coexistence of IHH and HMH has been reported only six times in the literature Table  4–7]. A–7. A4–7]Moreover, our patient was also diagnosed with PMD. There have been reports of HMH associated with PMD –11. HMH More recently, it has been suggested that HMH and PMD could be caused by ABM. First, a mixed cell population consisting of normal and androgenetic  cells was identified in the PMD placenta , 14. TheHemangiomas (liver and skin) in neonates have been associated with PMD  and placIn our patient, the combination of propranolol therapy for IHH and tumorectomy for the two largest HMH lesions diminished the progressive abdominal distension during infancy. Three years after the surgery, the HMH showed no regrowth, and the IHH was gradually regressing. Still, the optimal treatment and prognosis for our patient remain unclear. IHH is known to regress over time , 17 and Although the coexistence of IHH and HMH in infants born to women with PMD is extremely rare, the pathological and pathogenetic similarities between these disorders suggest that they could share an embryological origin, rather than being mere coincidence. The treatment and prognosis of these conditions remain unclear, and careful follow-up and additional treatments should be considered.


Gastric lymphangioma is one of the highly rare benign tumors characterized by multilocular or unilocular lymphatic spaces. Herein, we report a case of lymphangioma in the gastric antrum.A 77-year-old male patient who had been experiencing epigastric discomfort for a year was presented to our hospital. A gastric subepithelial lesion was diagnosed by upper endoscopy and was entirely excised via diatal subtotal gastrectomy. Endoscopic ultrasonography revealed an echoless homogenous echo pattern in the third wall layer. A lymphangioma was diagnosed by pathologic investigation of the resected specimen. The PubMed, Embase and Web of Science databases were reviewed for literature in English while using the keywords of “gastric lymphangioma” or “lymphangioma of stomach” or “gastric lymphatic cyst” or “lymphatic cyst of stomach” and the results were discussed.Gastric lymphangioma is a rarely occurring submucosal tumor that should be considered when diagnosing subepithelial lesions in the stomach. Literature reports lymphangioma as a benign microcystic lymphovascular lesion located mostly in the axilla, groin, and neck and is characterised by dilated lymphatic channels A 77-year-old man complained of epigastric discomfort for almost one year. He had an esophagogastroduodenoscopy in our hospital in September 2019 and a gastric subepithelial lesion (SEL) on the posterior wall of the stomach antrum was diagnosed. The lesion was sessile, broad-based and elevated in size, measuring 3.0 cm in diameter during white-light endoscopy. The surface of the overlying mucosa was red–orange normal stomach mucosa with no erosion or ulcer . They may be asymptomatic and can be identified incidentally . Sometimes, they may have various chief complaints, even could cause gastrointestinal hemorrhage Under white light endoscopy, the tumour is red–orange typical gastric mucosa with no ulcer or erosion; it is pliable upon compression with biopsy forceps. A large tumor may be translucent and lustrous. The depth and size of a lesion could be assessed with EUS. Under EUS, a cystic homogeneous echoless mass with hyperechoic inner septations in the third layer is the hallmark appearance of a gastric lymphangioma. The use of EUS examination can assist distinguish lymphangioma from gastric GIST , the most common gastric SEL. However, in the two cases composed of multiple small lymphatic spaces Microscopically, gastric lymphangioma is characterized by localized proliferation of thin-walled, dilated lymphatic channels of diverse diameters observed in the current case. In the overlying squamous epithelium, there is no dysplasia. In most cases, determining the accurate diagnosis based on histological findings is not difficult. A negative immunoreactivity for FVIII, positive immunostaining pattern in lymphatic endothelial cells for D2-40 could help establish the diagnosis of lymphangioma in cases where it has to be distinguished from hemangioma.Depending on the size of the tumor, different treatment options for gastric lymphangioma may be used. A large symptomatic tumor can be surgically removed. Endoscopic therapy was previously not used to resect gastric lymphangiomas. Kazuoki Hizawa et al. To summarize, gastric lymphangioma is a rare submucosal tumor that should be considered when diagnosing gastric SEL. The use of EUS for preoperative diagnosis and evaluation of tumor size and depth is critical.


We report a case of a 61-year-old lady presenting with several weeks of progressive left-sided weakness, and found to have a foramen magnum meningioma. She was counselled on surgical resection of the tumour, and a preoperative computed tomography angiogram (CTA) was obtained for operative planning purposes. CTA demonstrated incidental bilateral internal jugular vein (IJV) stenosis, with enlarged extracranial collateral vessels and elongated styloid processes. The main surgical concern was potential injury of the extracranial collateral vessels during operative exposure, which may compromise her intracranial venous outflow in light of the IJV stenosis. A doppler ultrasound scan of the IJVs was performed, which demonstrated that blood flow was still present through both vessels. Through careful soft tissue dissection during surgery, potential complications and injury to the extracranial collaterals were avoided.We performed a literature review of the incidence of IJV stenosis, its associated conditions, and potential surgical implications. Complications from injury to vital collateral extracranial vessels should be considered during preoperative planning in patients with anatomical variants or risk factors for IJV stenosis, as seen in this case. Internal jugular vein (IJV) stenosis is being increasingly associated with certain neurological conditions, including idiopathic intracranial hypertension (IIH), cerebrospinal fluid (CSF) disorders, multiple sclerosis, and Alzheimer’s disease . The rolWe report a case of a 61-year-old right-handed lady who presented to a local accident and emergency department with a several-week history of gradual heaviness affecting her left arm, on a background of 4 months of left-sided paraesthesia. She had no right-sided symptoms.On further questioning, she also described frequent coughing, difficulty clearing her throat and reaching high-pitched sounds during singing, and a few weeks of dysphagia. Her past medical history included acute idiopathic thrombocytopenic purpura, complicated by pneumonia 10 years previously. She is a keen horse-rider. On examination, she had a normal gait, with negative Romberg and Unterberger’s tests. There was a reduced sensation of light touch in her left hand. Her tongue was symmetrical, with mild fasciculations in the neutral position, and she had a slight uvular deviation to the right. Examination of lower cranial never function by flexible naso-endoscopy did not reveal any abnormality.CT scan demonstrated a left-sided mass at the level of the foramen magnum, measuring 3×16×22 mm, with brainstem compression. Magnetic resonance imaging (MRI) demonstrates a homogenously contrast-enhancing intradural, extramedullary lesion, causing compression of the medulla and upper cervical spinal cord  raised a possibility of bilateral jugular vein obstruction just below the skull base Figure , with enThere was also a finding of bilateral elongated styloid processes of 4 cm, with associated bilateral IJV stenosis. The reduced calibre of both IJVs continued throughout the neck, and the enlarged collateral vessels were seen to drain directly from the sigmoid sinuses. The concern at this stage was that the paraspinal venous channels were the primary intracranial venous outflow, with potential risks of venous hypertension, infarction and brain swelling in the event of venous injury during the surgical exposure. Following discussion with vascular surgery, a doppler ultrasound of the neck vessels was performed, which demonstrated patency and a reasonable blood flow through both IJVs. There was no evidence of underlying thrombosis Figure .Although this was reassuring, the patient was counselled in detail about small risks of potential venous infarction, surgical access difficulties and incomplete resection.Surgical approachA midline hockey-stick incision curving to the left was used, with careful dissection in the midline raphe. There was no breach of the deep cervical dilated veins on the surgical exposure. A left suboccipital craniectomy was performed, with C1 hemilaminectomy, and a part of the left occipital condyle was drilled down to facilitate surgical access. After applying the retractors, flow through deep veins was confirmed with intra-op ultrasonography. A hockey-stick dural incision was made. The tumour was firm, extra-axial, with a good arachnoid plane and was debulked internally to minimise retraction on adjacent structure Figure . The durThe patient was discharged home on postoperative day 5 with no neurological deficits. Immediate postoperative MRI showed no residual tumour  typically manifest with pain and dysphagia, usually after a tonsillectomy and formation of localised scar around the styloid process. When the IJV is compressed between the elongated styloid and C1 transverse process, “Eagle Jugular Syndrome” can arise, which can also incorporate intracranial hypertension symptoms -13. The Surgical relevanceThe surgical relevance of IJV stenosis pertinent to this case report is the presence of enlarged venous plexus collaterals within the cervical paraspinal muscles that were at risk of compromise in a posterior cervical approach. This was only picked up incidentally during a preoperative CT neck angiogram. The true incidence of intracerebral venous outflow obstruction is still unknown and is most likely underdiagnosed due to the associated nonspecific clinical symptoms. Based on Jayaraman et al.’s paper, the incidence of incidental dilated paraspinal venous collaterals secondary to IJV stenosis can be up to 8% . Whilst Given the relatively high incidence of IJV stenosis and collateral vessels, and the increasing number of pathologies associated with IJV stenosis, damaging collateral venous drainage in a posterior cervical approach and potentially precipitating other associated neurological conditions should be considered during operative planning in patients with anatomical variants or risk factors.We described the case of a patient with incidental bilateral IJV stenosis and associated dilated deep cervical venous plexus, during preoperative investigations for a craniocervical junction meningioma, and discussed potential surgical implications. Patients with anatomical variants or risk factors for IJV stenosis and dilated deep cervical veins should be counselled on potential associated complications when undergoing posterior cervical approach surgeries.


Masses of the mediastinum develop from anatomic structures that pass through or are normally located within this space; they may also occur secondary to metastatic spread from malignancies from other locations. The anterior compartment of the mediastinum can give rise to thymomas, which tend to be restricted to the thymus as well as nearby structures. Their symptoms are associated with their size and impact on nearby structures. This patient, a 22-year-old male with no medical history, presented to the emergency department for evaluation of a cough ongoing for 3 weeks. Chest X-ray and computed tomography (CT) scan of the thorax demonstrated a mass measuring up to 17.2 cm. Afterwards, a CT-guided biopsy was performed, which revealed findings consistent with a benign thymic neoplasm, though due to the specimen being scant, patient was referred to cardiothoracic surgery for resection and excisional biopsy of the mediastinal mass. Masses of the mediastinum arise from organs normally located within the mediastinum or traverse through this space . SymptomOf these, thymic carcinomas and thymomas are the most common neoplasms that arise from the thymus. Approximately 20% of all mediastinal neoplasms are thymomas with a majority of these patients being between the ages of 40 and 60 years of age . In addiThis report examines a rare surgical case of a 22-year-old male patient that underwent en-bloc resection of an enlarged mediastinal mass, which was determined to be a thymoma.A 22-year-old male patient with no significant medical or surgical history and no smoking history presented to the emergency department for evaluation of persistent cough for 3 weeks. The cough was initially associated with chest pain, which then subsided. He denied fever, chills, nasal congestion, shortness of breath and sore throat. A viral panel including COVID-19 was negative and patient was vaccinated against COVID-19. Chest X-ray  demonstrIn the operating room, the patient was placed in the left lateral decubitus position exposing the right side of the chest. Upon entering the pleural cavity, a significant amount of bloody pleural fluid was seen. After fluid aspiration, the mediastinal mass was seen compressing the right lung . SamplesThe patient was then turned to the supine position to resect the mediastinal mass, which started with a median sternotomy. The right pleural cavity was entered through the mediastinum, and the mass was mobilized from its adhesions to the chest wall. The lysis of adhesions was continued medially until the hilar structures were visualized. However, mobilization could not be achieved successfully without removing part of the lung that was extremely adherent to the mass. After removing this portion of the lung, the mass also demonstrated adherence to the right lateral aspect of the pericardium. Although multiple attempts were made to preserve the right phrenic nerve, a portion of it, along with part of the pericardium and small portion of normal-appearing thymus, had to be sacrificed due to surrounding inflammation and fibrosis. The mass was then able to be resected and removed, which was then sent for permanent pathologic examination . before Pathology from the permanent specimen demonstrated a partially encapsulated thymoma along with fibrotic bands that were composed of epithelioid epithelial cell proliferation mixed with many small lymphocytes. Ultimately, the mediastinal mass was determined to be a mixed type B2 and focal B3 thymoma.The patient was discharged 1 week after surgery. During follow-up visit 1 month following discharge, the patient denied any shortness of breath or any acute symptoms. At this time, the patient was cleared to resume all activities, including heavy lifting. He has been scheduled to receive adjuvant radiation therapy with a repeat CT scan of the thorax in 6 months.According to the Masaoka Classification of Thymic Tumors, type B2 thymomas demonstrate macroscopic invasion into the thymic or surrounding tissues, and type B3 shows invasion into nearby organs such as the lung or pericardium . UltimatThis case warrants discussion of tumor size in thymoma cases, especially since it is considered a critical prognostic factor . Althoug


Benign schwannomas are uncommon and their intraosseous location is even rarer counting for less than 1% of all benign primary bone tumors. They exceptionally occur in the oral cavity with the tongue being the most common site of involvement. We report here a case of intramandibular schwannoma derived from the inferior alveolar nerve, in a 57-year-old patient with a 3 months history of inferior left lip paresthesia. The oral examination showed a firm, painless and non-pulsatile swelling located in the inferior vestibule. The panoramic X-ray revealed a circumscribed and homogeneous radiolucent image. Treatment consisted of total excision of the tumor with preservation of the nerve bundles. The histological examination confirmed the diagnosis of schwannoma. The patient recovered a normal sensory function 6 months post-operatively without any recurrence up to 2 years after surgery. The treatment of intramandibular schwannoma is basically surgical with the conservative approach being the most advocated by majority of authors. Schwannomas, also called neuromas or neurilemomas, are benign nerve tumors developed from schwann cells, which forms the myelin sheath surrounding the axons of peripheral nerves. This myelin sheath allows a faster and saltatory conduction velocity in the axons involved ,2.Schwannomas are uncommon and their intraosseous location is even rarer counting for less than 1% of all benign primary bone tumors . MandibuWe report here a unique case of a mandibular intraosseous schwannoma and its surgical management.Patient information: a 57-years-old patient, with no particular pathological history, referred to us for paresthesias of the inferior alveolar nerve evolving since 3 months.Clinical findings: exobuccal clinical examination did not find any swelling or lymphadenopathy, and Vincent's sign was positive. The intrabuccal examination showed a small painless vestibular lump on the left mandible, covered with a healthy-looking mucosa. The patient was totally toothless.Diagnostic assessment: the orthopantomogram revealed a circumscribed, homogeneous radiolucent image, well limited by a clear and continuous condensing wall, on the left horizontal branch. The mandibular canal appears to be enlarged in contact with the lesion.Therapeutic intervention: excision of the lesion was performed under general anesthesia after surgical breach of the external mandibular cortical bone, revealing a lesion surrounded by a fibrous capsule in the mandibular canal. Although the lesion was connected to the lower alveolar neurovascular bundle, it easily detached from the nerve fibers, allowing them to be preserved (reserved .The mass and the fragments were sent for pathological examination, which confirmed the diagnosis of a schwannoma as it displayed areas of cell proliferation of moderate density interspersed with predominantly lymphocyte tissue within a fibrohyaline shell. The Antoni A areas were predominant; made of spindle-shaped and bipolar, epithelioid, well-organized cells grouped in parallel, arranged in clusters, with a compact palisade-like arrangement of nuclei within a collagen-rich connective tissue; occasionally alternating with the Antoni B areas.Follow-up: postoperatively, the patient maintained left labiomental paresthesia for a period of 6 months before its total resolution, supported by vitamin supplementation. Up to two years post-operatively, no recurrence was noted.The schwannoma´s most common location is the upper and lower limbs, they exceptionally occur in the oral cavity with the tongue being the most common site of involvement . IntraosThey occur at any age, most often in the fourth and fifth decades with a female predilection. They are generally asymptomatic and have a slow progression. Exceptionally, they can cause aesthetic or functional repercussions. The latter can range from a simple transient primary local paresthesia, same as in our patient, to a total loss of sensitivity of the inferior alveolar nerve . RadioloHistologically, the tumor is surrounded by a fibrous capsule consisting of two cell populations; the first so-called Antoni A type cell population which is characterized by a hypercellular proliferation of bipolar fusiform elements, within a rich network of collagen fibers and reticulin. The cells group together in such a way that the nuclei form a palisade. The juxtaposition of several palisades, like a barrel stave, is known as the verocay nodule. The second population, known as the Antoni B type, concerns fewer and less well-ordered cells within a looser connective tissue. The cells are more rounded with reduced cytoplasm and rounded nucleus resembling lymphocytes. This shape could represent type A degeneration .Conservative surgery is the treatment of choice, consisting of excision or enucleation of the tumor. Schwannomas are well encapsulated, ovoid, eccentric with respect to the nerve trunk and their excision is carried out without any difficulty. Nerve preservation throughout surgical removal deserves special attention from the surgeon. The literature suggests that the nerve should be separated from these tumors and preserved when the lesion is removed . HoweverReported cases of mandibular schwannoma remain very rare. This benign neurogenic tumor requires a thorough anamnesis, clinical examination, imaging and pathological examination to obtain the histological origin. Its treatment is based on surgical resection with a conservative approach to the nerve bundles whenever possible.


Allied disorders of Hirschsprung’s disease (ADHD) mainly present with bowel obstruction, intestinal dilatation, and chronic constipation, while recurrent spontaneous pneumoperitoneum was rarely reported. We aimed to report a case of recurrent spontaneous pneumoperitoneum caused by ADHD.A 59-year-old female patient presented with progressive and severe constipation in the past 30 years. She suffered from abdominal discomfort, which was described as ‘gurgling’ during the last three years. Radiography showed free-air and intestinal dilatation, without any other diseases, and she was identified with recurrent spontaneous pneumoperitoneum. Gastrointestinal transit test indicated gastrointestinal motility disorder, and anorectal manometry confirmed the presence of rectal anus-suppressing reflex. Subtotal colectomy was performed to relieve apparent constipation, and the postoperative pathological examination of the colon demonstrated proliferation of nerve fibers and hyperplasia of myenteric plexuses, as well as a relatively scarcity of ganglion cells in the myenteric plexus. Based on the presentations and the postoperative pathology, she was diagnosed with ADHD. The recurrent spontaneous pneumoperitoneum was regarded as the gas escape from dilated intestines, which was in high pressure. All the symptoms and her mental state were improved after the treatment with gastrointestinal decompression and enteral nutrition. However, during follow-up visits, she had intestinal infection, and suffered from severe diarrhea and water-electrolyte imbalance, and the patient eventually died at 17 months after the diagnosis.ADHD could be a rare cause of recurrent spontaneous pneumoperitoneum, and are mainly undiagnosed or misdiagnosed. A full-thickness biopsy of the gastrointestinal tract  and differential diagnosis are recommended for the definitive diagnosis. While the ADHD have shown a poor prognosis, timely and long-term treatment with intestinal decompression and nutritional therapy could help relieve symptoms and provide a better quality of life for such patients.The online version contains supplementary material available at 10.1186/s12876-022-02376-w. Allied disorders of Hirschsprung’s disease (ADHD) refer to a disease group that can clinically resemble Hirschsprung’s disease, featuring bowel obstruction, intestinal dilatation, and chronic constipation despite the presence of ganglion cells in the terminal rectum . HoweverA 59-year-old woman was admitted to our hospital in June 2019, with chief complaints of constipation for 30 years and recurrent abdominal discomfort for 3 years.The patient presented with constipation 30 years before her admission. Symptomatic treatments for constipation were given, while symptoms became progressive and severe during these years. In 2016, for no apparent reason, she started to have abdominal discomfort, which was described as ‘gurgling’. She denied having fever or vomiting. She was admitted to a local hospital for further examination. Subdiaphragmatic free-air was shown in the upright conventional abdominal radiography, and free-air in the peritoneal cavity was presented in the computed tomography (CT) scan of the abdomen  and hepatic hemangioma were also diagnosed. There was no remarkable family history for similar conditions.2. Superficial lymph nodes were not palpable. The examination of lung and heart showed no abnormality. The abdomen was distended, tympanitic, and tender. The liver dullness boundary disappeared, and the shifting dullness was negative. Bowel sounds were active with no abnormality in the rectal examination.Physical examination at the time of admission revealed that the body mass index was 15.6 kg/mAfter admission, blood test revealed that pre-existing renal dysfunction was worsened with an increase in creatinine level from 127 to 161 μmol/L  due to insufficient intake. The blood routine test showed no significant differences in data measured before and after admission, and the liver function showed no abnormality. The erythrocyte sedimentation rate and C-reactive protein level were normal. The autoantibody and M protein was negative. Meanwhile, no sign of tumor or inflammation, gastrointestinal perforation, peptic ulcer or pneumatosis cystoides intestinalis was found in the imaging data  of the proximal bowel .ADHD were first reported by Ravitch as “pseudo Hirschsprung’s disease” in the Annals of Surgery in 1958 , and havIn this case, the patient had typical symptoms of chronic constipation, serious abdominal distention, and recurrent intestinal obstruction . The imaExcept for these typical symptoms, this patient presented with spontaneous pneumoperitoneum, which is the rare manifestation of ADHD. Perforation of gastrointestinal tract and laparoscopic surgery are common causes of pneumoperitoneum . Non-surAs for treatment, intestinal decompression and enteral or parenteral nutrition are currently recommended. Radical surgery is controversial and no drug can be recommended to effectively improve functional gastrointestinal disorders or symptoms . In thisTo our knowledge, this is the first case of ADHD presented with recurrent spontaneous pneumoperitoneum. The patient underwent an surgery and visited doctors from nationwide for the cause of recurrent spontaneous pneumoperitoneum. She was finally diagnosed with ADHD and her symptoms were improved through conservative treatment. ADHD is rare and mainly undiagnosed or misdiagnosed. Physicians are advised to consider ADHD in these patients after differential diagnosis. A full-thickness biopsy of the gastrointestinal tract  is recommended for the definitive diagnosis . While tAdditional file 1. The timeline of disease in this case.


In the case of a congenital wall defect including liver parenchyma, there is still an ongoing debate on the definition of the abdominal wall defect and best care practice. Even though congenital abdominal wall defects are frequently diagnosed in prenatal screenings, controversy on the underlying etiology, embryology and underlying anatomy remains. Prenatal distinction between omphalocele and hernia into the cord cannot always be obtained; however, due to its clinical relevance for postnatal management and counseling of parents, accurate diagnosis is essential. (2) Case Presentation: We describe the uncommon postnatal finding of a pediculated accessory liver lobe with gallbladder in a preterm with umbilical cord hernia, which was prenatally diagnosed as omphalocele. Postnatal examination revealed an amniotic sac with a diameter of six and a small abdominal wall defect of three centimeters in diameter. Postnatal management included resection of the accessory liver lobe and gallbladder and closure of the defect. (3) Results and (4) Conclusions: Throughout the literature, the distinction between umbilical cord hernia and omphalocele has been variable. This has led to confusion and difficulties regarding postnatal treatment options. In order to achieve an accurate prenatal and/or postnatal diagnosis, the morphological differences and clinical manifestation of umbilical cord hernia and omphalocele need to be assessed. Further embryological studies are warranted to understand the underlying embryological pathology of omphalocele and umbilical cord hernia and offer appropriate treatment. In consideration of possibly severe complications in the case of the torsion of a pedunculated accessory liver lobe, we strongly recommend primary removal once pre- or intraoperative identification has been made. Accessory liver lobes (ALL) are a rare finding and have mostly been encountered as incidental findings in adulthood, during abdominal surgery or autopsy ,2. In raWhile the anatomic variations of liver vessels and bile ducts are common, ALLs are extremely rare and present as an extranumeric liver lobe composed of physiologically intact liver parenchyma. Due to the rare encounter of this anatomic variation, they are hardly considered in the differential diagnosis of uncommon hepatic findings. However, clinical episodes of vascular damage to the ALL  or the oIn order to be able to distinguish abdominal wall defects, the definition of these two separate entities needs to be evaluated.Currently, an umbilical cord hernia is defined as an abdominal wall defect with a diameter smaller than 4 cm, containing nothing but intestinal loops. On the contrary, in omphaloceles, the defect of the abdominal wall must measure over 4 cm to be defined as an omphalocele. In the case of an omphalocele, the amniotic sac may contain intestinal loops along with other visceral organs, such as the stomach or liver . UnfortuWe report a case of a male newborn with the prenatal diagnosis of a small abdominal wall defect. On prenatal ultrasounds, the herniation of the liver into the defect was suspected; therefore, parents were counseled on postnatal management. The patient was delivered at 35 + 6 gestational weeks with a birth weight of 2750 g by cesarean section due to placenta accreta. Postnatal examination showed a small abdominal wall defect of three centimeters in diameter and an intact amniotic sac with a protuberance above the umbilical ring measuring six centimeters a and 1b,Since the patient was hemodynamically stable and there were no signs of the alteration of blood flow in the liver parenchyma, and surgical treatment was scheduled for the following day. On postnatal day one, the child underwent surgical exploration and the primary repair of the UCH. We identified separated but intact liver tissue with an embedded gallbladder separated from the abdominal liver and connected via a six-centimeter pedicle containing blood vessels and biliary ducts. The pedunculated liver tissue was adherent to the amniotic sac, and we identified at least two cleaved liver segments, most likely liver representing segments V and VI with an embedded gallbladder. Primarily, the ALL was relocated into the abdomen and the abdominal wall was closed to allow time for further diagnostic imaging. Sonography identified a normal portal vein and hepatic artery. The ALL was identified in the left lower quadrant of the abdomen. Bowel malrotation was excluded. After thorough investigation of the literature, it was decided to remove the ALL in order to prevent the torsion of the pediculated ALL, as described in the literature. An intraoperative cholangiography identified a very long cystic duct draining into a normal common bile duct. The exploration of the pedicle revealed at least eight vascular structures including the cystic artery and duct a and 2b.This case report presents us with two separate challenges. First, the definition and distinction between and umbilical cord hernia and an omphalocele, and secondly, the evaluation of the optimal management of a pedunculated accessory liver lobe in pediatric patients.Throughout the literature, UCH and omphalocele have not been separated as two different embryologic entities. This has led to confusion and difficulties regarding treatment options. Recent publications describe UCH as being associated with a higher mortality, cardiac anomalies and neural tube defects , which aIn order to obtain an accurate prenatal and/or postnatal diagnosis, the morphological differences and clinical manifestation of UCH and omphalocele need to be distinguished. Several studies claim that UCH solely affects the midgut and not visceral organs, such as the liver ,12, therHowever, whether the defect is determined to be an UCH or an omphalocele, in cases of the herniation of visceral organs, such as the liver, into the amniotic sac, we recommend immediate surgical exploration in order to prevent the strangulation of liver tissue, which may lead to ischemia and possible subsequent injury to the otherwise intact liver parenchyma and biliary tract. Accessory liver lobes are a rare anatomic variant. They present as a supranumeric liver lobe of intact hepatic parenchyma. While accessory liver lobes have some form of continuity with the original liver, ectopic liver lobes are completely separated and can be found in various locations . RegardiIn pediatric patients, several reports of herniated liver parenchyma in regard to abdominal defects, such as umbilical cord hernias, can be found in the literature. They describe their findings as accessory liver lobes with an attached gallbladder that were only connected via vessels and bile ducts to the liver ,8. To ouRegarding the management of the herniation of liver parenchyma into the abdominal wall defect, the repositioning of the herniated liver parenchyma into the abdomen ,19,20 seHowever, Elmasalme et al. reported a life threatening event of the torsion of an accessory live lobe in a six month old child, which was initially treated as a presumed pneumonia . The chiGoor et al. intended to classify the anomalies of the biliary tract depending on the biliary drainage from the lobe into the normal biliary tree and on the presence of a common capsule . Three dTo our knowledge, there is no case in the current literature reporting of complications during or after the removal of a pediculated accessory liver tissue. In consideration of the possibly severe complications in case of torsion as mentioned above, we strongly recommend primary removal of a pedunculated accessory liver lobe once pre- or intraoperative identification has been made.Cases of UCH with accessory liver lobes connected via a pedicle to the abdominal liver are extremely rare. Further embryological studies are warranted in order to explore and understand the underlying embryological pathology of omphalocele and umbilical chord hernia. This is essential to properly distinguish both entities and to understand the development of accessory liver lobe formation.The correct prenatal diagnosis of umbilical cord hernia should be aspired to allow adequately counseling of parents and to decide on prenatal management. One should be aware of the possibility of pediculated accessory liver lobes, and these should actively be searched for and surgically removed in order to prevent torsion and subsequent hepatic ischemia and spare the child from additional unnecessary procedures. The presence of an accessory liver lobe should be taken into consideration in children with a history of an omphalocele or umbilical cord hernia closure.