Gastrointestinal stromal tumors (GISTs) in the third portion of the duodenum are rare. Intussusception and obstruction are rarely caused by GISTs because of their tendency to grow in an extraluminal manner. Herein, we report a case involving segmental duodenectomy in a patient with duodenojejunal intussusception secondary to a primary GIST.A 91-year-old woman with a history of iron-deficiency anemia presented with vomiting and anorexia. Preoperative imaging suggested duodenojejunal intussusception secondary to a GIST in the third portion of the duodenum. Segmental duodenectomy with end-to-end duodenojejunostomy without reduction of the intussusception was performed. At 6 months after the surgery, the patient’s anemia had improved and she had no abdominal symptoms.Adult intussusception requires surgical resection because most of the patients have intraluminal lesions. The location in relation to the Vater papilla, tumor size, and resection margin should be considered when selecting the type of surgical resection for duodenal GIST. Limited resection appears to be better than pancreaticoduodenectomy with respect to postoperative complications. Considering the age and performance status of this patient, a less invasive maneuver was selected.Duodenal GISTs can be a rare cause of intussusception. Thus, a limited surgical resection procedure should be considered in such cases. SinceHerein, we report a case involving segmental duodenectomy in a patient with duodenojejunal intussusception secondary to GIST in the third portion of the duodenum. This work has been reported in line with the SCARE criteria .22. Her abdomen was soft and flat with previous operative right pararectal and right subcostal incision scars. On blood examination, her hemoglobin and albumin levels were low   . Contrasctively) a. Two mactively) b. Esophactively) c.Table 1Considering her age and physical status, we planned to resect only the duodenum unless the metastatic liver tumor had subcapsular localization and a risk of rupture. Laparotomy revealed lymph node metastasis, but no peritoneal metastasis and no exposure of the liver tumors to the abdominal cavity was observed a. The liThe resected specimen showed a smoothly marginated tumor measuring 40 × 40 × 15 mm with an erosive lesion on the mucosal side and two elastic soft tumors on the serosal side a. Histol3In this report, we described the diagnosis and management strategies in a rare case involving duodenojejunal intussusception secondary to primary GIST in the third portion of the duodenum. Although gastroduodenal, jejunoileal, and ileocolic intussusceptions have been reported, to our knowledge, this is the first report of a patient with duodenojejunal intussusception secondary to GIST . Since GIn adults, intestinal invagination or intussusception is rare, accounting for 5% of all intussusceptions and 1% of all intestinal obstructions . There iIn this case, the preoperative diagnosis was intussusception accompanied by duodenal GIST; thus, we planned limited resection (LR). The Cattell-Braasch maneuver, which permits the surgeon to elevate the right colon and entire small bowel cephalad, was considered in order to observe the third portion of the duodenum completely if the GIST had been located behind the superior mesenteric vessels and transverse mesocolon . HoweverThe peritoneum and liver are the most common sites of metastasis, whereas regional lymph node metastasis is rare (0–10%), except in Carney’s triad ,8,13,17.Immunohistochemical staining for CD117 (c-kit), CD34, desmin, smooth muscle actin (SMA), and S100 should be performed . The posGISTs considered high risk include those >5 cm in diameter and with mitotic count >5 per 50 high power fields (HPFs), those >10 cm in diameter whatever the mitotic count, and those of any size with high mitotic count (>10 per 50 HPFs) . The med4In conclusion, duodenal GIST can cause duodenojejunal intussusception, and in this rare situation, LR without initial reduction can be performed safely. As demonstrated in this case, even elderly patients can have improved quality of life with limited surgical resection.4.1The patient and her family were concerned about whether surgery would adversely affect oral ingestion or other activities of daily living (ADL). Therefore, they agreed to limited surgical resection that would allow oral ingestion. Furthermore, they did not provide consent for a hepatectomy for treatment of the liver metastasis owing to the age of the patient. The surgery was performed with no postoperative complications other than an infection of the surgical site. After the surgery, the patient was able to ingest food, and returned to her normal ADL. The patient was informed about the regular medical follow-up.The research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This case report was approved by the Research Ethics Committee of the Ofuna Chuo Hospital (No. 2019-003).Written informed consent was obtained from the patient for publication of this case report.Goshi Fujimoto: Surgeon of the patient’s procedure described in the case report, concept and design of study, acquisition of data, drafting the manuscript, revising the manuscript, and approving the final version of the manuscript.Shunichi Osada: Assistant during the patient’s surgery described in the case report, revising the manuscript, and approving the final version of the manuscript.https://www.umin.ac.jp/ctr/) with the unique identifying number UMIN000037656.This study was registered as a case report in the UMIN Clinical Trials Registry (Goshi Fujimoto.The datasets supporting the conclusions of this article are included within the article.Not commissioned, externally peer-reviewed.None.


Congenital pulmonary airway malformation (CPAM) is a rare developmental deformity of the lower respiratory tract. The disease occurs more in newborns. However, on rare occasions, CPAM can be found in adults. Radiologic features of CPAM include cystic or solid mass pattern. In an elderly patient, CPAM can be easily misdiagnosed as lung cancer.A 66-year old woman was admitted with complaints of chronic cough, expectoration. Her past history was unremarkable with no history of tuberculosis or smoking. Physical examination was normal. Computerized tomography of the chest showed an irregular cystic lesion in right lower lobe.Histopathological results confirmed the diagnosis of CPAM.The right pulmonary wedge resection was performed via thoracoscopic surgery.On follow up 1 year later, the patient is asymptomatic.CPAM is rare in adults, and imaging cannot accurately distinguish CPAM from thin-walled cystic lung cancer. Hence, histopathology is mandatory to confirm the diagnosis. The disease is often found in newborns. However, on rare occasions, it can be found in adults. A common complication of CPAM is recurrent pulmonary infection. It may be diagnosed in adults because of complications or suspected malignancy.,5 So it is rarely discovered in asymptomatic adults, the preoperative diagnosis is challenging because congenital cystic adenomatoid malformations (CCAM) can be confused with other more common lesions. Therefore, we report a case of a 66-year-old woman with cystic lesion in the right lower lobe which was highly suspicious of lung cancer. Surgical resection of the lesion followed by histopathological confirmed a CPAM.Congenital pulmonary airway malformation (CPAM) is a rare developmental deformity of the lower respiratory tract.2A 66-year old Chinese woman, born in Beijing, was admitted with complaints of cough, expectoration since for 3 months. She denied fever, night sweats, loss of weight, chest pain, or hemoptysis. Her past history was unremarkable with no history of tuberculosis or smoking. Physical examination was normal. Clinically, electrocardiography and routine laboratory tests were normal. Contrast-enhanced computerized tomography of the chest showed 35 × 22 mm irregular cystic lesion in right lower lobe, wall thickness of 0.5 mm to 3.5 mm, smooth inner margin, septum was seen within the cyst. The lesion also showed mild distortion of surrounding lung tissue with no evidence of abnormal arterial blood supply Fig. A–C. The 3 Incidence of CCAM of the lung was found to be 0.0029% to 0.004% in newborns.,7 However, on rare occasions, it can be found in adults.CPAM, formerly known as CCAM, was first described by Ch’in and Tang in 1949. The pathogenesis is not clear but segmental bronchial atresia has been attributed as the primary pathogenetic defect that results in the development of a CCAM distal to the defect. Type 1 is thought to originate in the distal or proximal bronchioles. Type 1, the most frequently occurring type (60%–70%), was a lesion consisting of single or multiple large cysts (more than 2 cm in diameter) lined by ciliated pseudostratified columnar epithelium. The walls of the cysts contain prominent smooth muscle and elastic tissue. Mucus-producing cells are present in 32% of the cases, and cartilage in the wall was seen in 5% to 10%. Relatively normal alveoli may be seen between the cysts. Ninety-five percent of type 1 patients have only 1 lung lobe involved. Type 2 was composed of multiple small cysts (less than 1 cm in diameter) lined by ciliated cuboidal to columnar epithelium and was associated with other anomalies. There were no mucous cells or cartilage. Type 3 was composed of regularly spaced bronchiole-like structures separated by masses of cuboidal epithelium-lined alveolus-like structures. In 2002, Stocker added type 0 and type 4 to 3 types of CCAM classification, both of which were very rare. Meanwhile, CCAM was renamed CPAM. Each type of CPAM has unique pathological features. The clinical manifestations of CPAM mainly depend on the lesion of the size, number, location, and compression of surrounding tissues. In newborns, most of them presenting clinical symptoms may include fever, cough, progressive dyspnea, cyanosis, or hemoptysis. In adults, presenting clinical symptoms may also include cough, expectoration, chest tightness, and dyspnea. CPAM are often found when they cause recurrent pulmonary infection.,5 Clinical symptoms are not obvious when the lesion is confined to a single lobe or lung segment. Imaging examination is an important way of CPAM, which is characterized by bronchiolar adenomatous hyperplasia and cyst formation. Its imaging pattern is described into 3 types:In histopathology, CPAM is a hamartoma-like lesion. Stocker has described 3 types of CCAM classification on the basis of histopathologic appearance in 1977.(1)single or multicystic pattern,(2)nonhomogeneous mass consistent with the gross configuration of numerous evenly spaced cysts usually less than 1 cm size,(3)a cystic or solid mass. Final diagnosis and classification required pathological confirmation. It is not clear why this case has gone undetected. We hypothesized 3 possible causes: first, the lack of symptoms of recurrent respiratory infections; second, the limitation of imaging lesions; and third, the absence of annual routine chest imaging examination. These may explain that the patient was not detected early. In our case, the patient was elderly female presented in 6th decade, with no history of recurrent pulmonary infection. Her computerized tomography (CT) chest showed irregular cystic lesion in peripheral right lower lobe, with uneven wall thickness and septum within the cyst. These features raised possibility of malignancy. Therefore, it needs to be differentiated from lung cancer. There is a kind of lung cancer presenting as thin-walled cyst or cavity has been described in various case reports.–17 It usually presents as peripheral lung lesion, most of them were adenocarcinomas. These are more common in females than male. CT features suggestive of malignancy include uneven thickening of the thin-walled cavity, irregular margin, septum in cavity, nodule formation, presence of short spicules, blood vessel convergence signs, signs of lobulation or pleural indentation. Most important is increasing size of cyst or cavity with thickening of its wall. Histopathology shows that carcinoma cells grow randomly along the cyst wall.–17 Hence, the need for surgical resection and pathological examination is reaffirmed. However, in our case, diagnosis of type 1 CPAM was confirmed with histopathology with no evidence of malignancy. Type 1 CPAM has good prognosis; however, a risk of malignant transformation was reported to have an association with a mucinous bronchioloalveolar carcinoma.–20 Currently, lobectomy is considered to be the best method for treating it.Radiologic features of type 1 CPAM include involvement of single lobe, predominantly lower lung fields with left lung more than right.We report a unique case of pulmonary cystic lesion masquerading pulmonary malignancy, diagnosed as CPAM in patient presenting unusually in 6th decade. It poses a diagnostic challenge, broadens the thinking of clinicians and increases the understanding of CPAM. Though meticulous imaging may help to differentiate it from malignancy, histopathology is mandatory to confirm the diagnosis.Data curation: Qi Leng, Zhenwu Li, Peng Pang.Formal analysis: Zhenwu Li, Peng Pang, Xiaoli Qi.Resources: Qi Leng.Supervision: Ying Zhao.Validation: Qi Leng, Zhenwu Li.Writing – original draft: Ying Zhao, Xiaoli Qi.Writing – review and editing: Ying Zhao, Yongxiang Zhang, Xiaoli Qi.


A pediatric patient of 12 years consulted for a left scrotal mass of 2 months of evolution. After suspecting a cystic content due to positive transillumination, on ultrasonography a scrotal cyst separated from the testis, of 5 cm in its maximum length, was confirmed. Due to size, parental anxiety, and the referred short evolution, excision was decided. Given the clinical radiological findings, a scrotal incision was chosen, obtaining complete excision. Biopsy confirmed the diagnosis of simple epididymal cyst (EC). ECs usually present as painless, scrotal swelling in adolescents as a result of dilatation of the efferent epididymal tubules. Many cases (up to 60%) regress spontaneously. In these, average time to involute ranges from 4 to 50 months. Although cases of cyst torsion have been described (with pain derived from ischemia and inflammation), conservative management has been suggested in the majority, both in pediatric and in adult series. Surgery is recommended in some patients, due to testicular pain or increased paratesticular mass, as was our case. The most common extratesticular lesions encountered in children include Morgagni hydatid torsion, epididymitis, paratesticular rhabdomyosarcoma, epididymal cysts/spermatoceles, and varicocele. As opposed to intratesticular masses, most extratesticular masses are benign. Examples of rare extratesticular lesions in adults include lipomas (most often arising from the spermatic cord), adenomatoid tumors (most often found in the epididymis), sarcoidosis, liposarcoma, leiomyosarcoma, malignant fibrous histiocytoma, mesothelioma, and lymphoma –3.A pediatric patient of 12 years consulted for a left scrotal mass of 2 months of evolution. It was not painful, and he did not present any other associated symptoms. On examination, testes appeared normal in size and location, but a 4 cm oval soft scrotal mass was palpated in the left scrotum, adjacent to the testis. It was of elastic consistency, mobile, not adhered, and separated from the left testis. Given the suspicion of epididymal cyst (EC), after positive transillumination, ultrasound was requested. An uncomplicated scrotal cyst separated from the testis, of 5 cm in its maximum length, was confirmed. Due to the size, parental anxiety, and the referred short evolution, preferred excision was decided. Given the clinical radiological findings, where a malign tumor was not suspected, a scrotal incision was chosen, obtaining complete excision of the cyst, adjacent to the epididymis head. Figures EC usually presents as painless, scrotal swelling in adolescents as a result of dilatation of the efferent epididymal tubules. On many occasions terms ‘EC' and ‘spermatocele' have been used interchangeably to describe the same entity. The only means of differentiating these 2 lesions is aspiration of the cyst content, as EC does not contain sperm , 4. HistPrevious reports revealed EC in 5% of pediatric patients undergoing scrotal ultrasound and in 15% of boys undergoing ultrasound for a palpable mass , and in Etiology of EC remains unknown. The result of endocrine disrupting agents acting fetally or postnatally may play a role in EC development, and obstruction to the flow of sperm content has also been described. Epididymal epithelium is dependent on a relatively high concentration of androgens for normal function. This dependence includes secretory activity, fluid resorption, and cytological integrity. Recently, an apparent increase in the number of cases has been pointed out by some authors, theoretically caused by increasing exposure to estrogenic compounds in the environment . On the Pain and scrotal mass are the most common clinical findings in patients with EC.Many cases (up to 60%) regress spontaneously , 4. In tOur case illustrates a rare specimen of EC of a considerable size in a preadolescent male, showing the ease to perform the excision through a scrotal incision.


Streptococcus pyogenes being a possible, though uncommon etiology. Even more uncommon are mycotic aneurysms secondary to group A strep purulent pericarditis and bacteremia. We report a case of an 18-year-old female with a history of strep pharyngitis develop Streptococcus pyogenes purulent pericarditis with subsequent ventricular fibrillation (VF). Following initial stabilization, she ultimately developed a 4.8 cm mycotic aneurysm of the ascending aorta, with resultant compression of the pulmonary trunk and right pulmonary arteries.Purulent pericarditis is a rare disease in the era of antibiotics, with  Purulent pericarditis is a rare but rapidly progressing and in some cases deadly event. Rapid detection via imaging and high clinical suspicion are paramount. Purulent pericarditis can, in limited cases, complicate clinical course by instigating mycotic pseudoaneurysm formation in the aorta. Proper imaging and prompt treatment with antibiotic therapy and either endovascular or open surgical repair are vital. We present a case of group A strep purulent pericarditis that resulted in formation of a mycotic pseudoaneurysm in an otherwise healthy 18-year-old female. She underwent subsequent urgent surgical repair with complete recovery. To our knowledge, this is the second reported case of purulent pericarditis complicated by mycotic pseudoaneurysm.An 18-year-old female with an allergy to penicillin and a past medical history of migraines presented to the emergency department with pleuritic chest pain and dyspnea on exertion. Three months prior she had an upper respiratory infection. Her initial workup was unrevealing, including negative troponin and normal complete blood count. Her electrocardiogram (ECG) revealed normal sinus rhythm. A computed tomography angiogram (CTA) chest had no significant findings. She was diagnosed with atypical chest pain and discharged home.Streptococcus pyogenes. Antibiotic coverage was weaned to intravenous cefazolin monotherapy. She continued to drain 240–360 milliliters of purulent pericardial fluid daily and was on norepinephrine for pressure support. Post resuscitation, she developed acute renal failure that required intermittent renal replacement therapy secondary to acute tubular necrosis. Antibiotic coverage was broadened to vancomycin and cefepime after the development of acute respiratory distress syndrome (ARDS) secondary to multifocal pneumonia. She was gradually weaned from the ventilator and subsequently extubated. The remaining hospital course was uneventful, and she was discharged home on oral levofloxacin for completion of her antibiotic course.She returned three days later with worsening chest pain. ECG  was signThe patient returned to the ED several days later with shortness of breath and worsening back pain that radiated to her chest. The ECG  at that Our patient was again transferred to the ICU. A subsequent CT chest with contrast demonstrated pseudoaneurysm expansion to 5.3 cm. Emergent cardiothoracic surgery with circulatory arrest was performed. The operation revealed a 2 cm × 1 cm wall defect in the distal ascending aorta, extending into the arch of the aorta. Intraoperative transesophageal echocardiography revealed right ventricular remodeling resulting in a severely strained D-shaped ventricle and severely dilated RVOT of 4 cm . AdditioStaphylococcus aureus comprising the majority of cases. Other commonly reported organisms include Streptococcus pneumoniae, Haemophilus influenzae, viridans streptococci, and anaerobic bacteria [β-lactam agents or combination therapy with a β-lactam and aminoglycoside [Purulent pericardial effusions are a relatively uncommon occurrence, particularly in the post antibiotic era. A retrospective study in Spain, done between 1972 and 1991, indicated an incidence of 33 in a population of 593,600 [bacteria . Patientbacteria –12. Dissbacteria . Despitebacteria . PreviouMycotic pseudoaneuryms are rarely described following purulent pericardial effusions and only comprise a small minority of all aneurysms (0.7%) . HematogPurulent pericarditis is a rare and serious condition with a high chance of complications and mortality. We describe a rare case of group A streptococcal purulent pericarditis with multiple complications, including mycotic pseudoaneurysm formation and resultant compression on the pulmonary vasculature. While unusual, mycotic aortic pseudoaneurysms are a reported complication following purulent pericardial infection. Appropriate and early antibiotic therapy was key in our patient's recovery as well as swift pseudoaneurysm identification and surgical intervention.


An osteoma is a benign, slow-growing, osteogenic neoplasm with a low recurrence rate that is typically characterized by the proliferation of a compact or cancellous bone. It can be peripheral, central, or extraskeletal. Usually asymptomatic, peripheral osteomas in the maxillofacial region commonly arise in the paranasal sinuses and mandible and rarely occur in the zygomatic arch, with only six previously documented cases in the literature. Here, we present the management of a solitary peripheral osteoma of the right zygomatic arch in a 72-year-old woman and a review of the literature. Osteoma is a benign, slow-growing, osteogenic neoplasm characterized by the proliferation of a compact or cancellous bone . It was Osteomas almost exclusively affect the maxillofacial skeleton, particularly the paranasal sinuses and mandible, and can be classified based on the location from which they arise. Central osteomas arise centripetally from the endosteum, peripheral osteomas are caused by centrifugal growth of the periosteum, and extraskeletal soft tissue osteomas develop within the muscles , 4, 5, 7Clinically, peripheral lesions appear as unilateral, pedunculated, and asymptomatic mushroom-like masses and can produce pain, trismus (when there is nerve involvement), limited mandibular movement, malocclusion, swelling, and facial asymmetry , 10–13.Computer tomography (CT) is the current gold standard for the diagnosis and surgical management of an osteoma. A peripheral osteoma is seen as an oval, radiopaque, well-circumscribed mass attached to the cortex by a broad base or pedicle , 13, 14.We describe a rare case of peripheral osteoma in the zygomatic arch and present a review of the literature.A 72-year-old woman was referred to the division of Maxillofacial Surgery, Città della Salute e della Scienza Hospital, University of Turin , for an enlarged preauricular mass on the right side of her face. The lesion had slowly been growing for 3 years . There wUnder general anaesthesia, the zygomatic arch was accessed via a preauricular incision with temporal extension (Al-Kayat and Bramley's modifications ). After The patient's postoperative course was uneventful with only temporary dysesthesia along the V3 branch of the trigeminal nerve. The symptoms resolved spontaneously after 2 months. The patient was discharged home 5 days after surgery. CT scans 1 year after surgery showed normal bone architecture of the right zygoma, good symmetry, and no signs of relapse Figures . No clinPeripheral osteomas of the maxillofacial region most frequently occur in the paranasal sinuses but can also be found in the jaw bones, external auditory canal, orbit, temporal bone, and pterygoid processes , 13, 17.Several authors , 10, 13 Similar to Furlaneto et al. , we beliMost cases of tumor excision have been performed with zygomatic arch preservation, with the exception of a case reported by Quintans et al.  , in whi


Obturator hernias are a rare subtype of intra-abdominal hernias that present with considerable challenges in both diagnosis and surgical repair. Delays in diagnosis and management significantly increases the Morbidity and Mortality.An elderly 70-year-old female presented with Lower abdominal pain radiating to the medial side of the left leg over a year that has become persistent and continuous over the 3 day period. Patient was found to have a Richter's type of strangulated obturator hernia on diagnostic laparoscopy. A laparoscopic assisted resection of the strangulated small bowel and repair of the Hernia was performed. Postoperatively patient had an uneventful recovery.Surgical treatment is the standard of care for an obturator Hernia. Increasing number of laparoscopic repairs are being performed in the recent past. Minilaparotomy greatly facilitates the resection and anastomosis of a dead bowel without jeopardizing cosmesis and mesh placement at the time of repair.Heightened awareness of the condition combined with our proposition of a Hybrid repair achieves optimal results in an emergency setting. A Laparoscopic assisted repair of a strangulated hernia provides a durable repair and at the same time offers the patient benefits of minimally invasive surgery. They occur more commonly in thin, elderly females between the ages of 70–90 and with a higher prevalence in Asian countries . Obturat2A 70 year old female with chronic obstructive pulmonary disease (COPD) on home oxygen with no history of previous surgeries presented with lower abdominal pain for a year which has become constant and severe for 2 days. She had nausea, vomiting and was not passing flatus for 3 days. On physical exam vital signs were within normal limits. Abdomen was mildly tender in the suprapubic region with no peritoneal signs. There are no palpable hernial swellings or lymph nodes. On adduction and medial rotation of left thigh, patient complained of increased pain in her medial left leg. Laboratory test showed increased lactic acid level. Abdominal CT scan  was doneA small transverse pfannensteil incision (5 cm) was made just above the pubic symphysis and the loop of gangrenous small bowel was delivered out and resected with a GIA stapler. After that peritoneum and the fascia was closed and pneumoperitoneum recreated. Subsequently a Vicryl woven mesh cut to appropriate size was tacked around the left obturator canal to buttress the hernial defect. The neurovascular bundle and the obturator foramen was clearly visible laparoscopically and was protected. No entrapment of bowel or omentum was observed at the end. Pneumoperitoneum was desufflated and ports were removed. Post operatively patient made an uneventful recovery.3Obturator hernias were first described by Arnaud de Ronsil in 1724 and the first successful surgical repair of these was performed by Henry Obre in 1851 . Concomi4The obturator foramen which is the largest foramen in the body is formed by the pubis and ischium and is covered by an obturator membrane anterosuperiorly. The foramen is closed by a strong quadrilamellar musculoaponeurotic barrier consisting of an internal and an external obturator membrane and an internal and an external obturator muscle. The membranes are joined below and separated above and are encased by the muscles on both sides. The obturator canal is situated in the large cranial part of the foramen. The obturator canal is approximately 2 to 3 cm long, running obliquely downward and upward. The obturator nerve and blood vessels pass through this canal and are covered peripherally by fatty tissue. Usually this fatty tissue helps fills the space in the obturator canal, thus, decreasing the risk for protrusion of small bowel. With severe cachexia and/or malnutrition, there is loss of this fatty tissue which can increase the risk for an obturator hernia to develop ,8,9. Thr5Hernia of a skinny old lady” underscores the phenotype of the Obturator Hernia, seen in the elderly and emaciated women. The hernia has a female predilection because they have a broader pelvis with a larger triangular obturator canal opening with a greater transverse diameter. Prior pregnancy is cited as a common risk factor [The eponym “k factor ,4,9. OH k factor . Three sk factor ,4. Patiek factor . Ultraso6Surgical repair of the Hernia is the standard of care for Obturator Hernia. Several open and laparoscopic techniques are described in the literature to repair the Hernia ,12. As tConventionally open approaches are used. Four different types Abdominal, retropubic, obturator, and inguinal approaches are described . In an eRecently Laparoscopic techniques  are increasingly being used for the repair of OH . As fami7Awareness of the pathophysiology of Obturator Hernia is important. Delays in diagnosis and treatment can significantly heighten the morbidity and mortality. Rarity of the condition and the absence of telltale clinical signs imposes challenges in early diagnosis and treatment. However a heightened awareness of the condition, dedicated cross sectional imaging and an early operative repair provides the best long term outcomes.No conflicts of interest.All the authors report no financial acknowledgments involved for this case report and declare no potential conflicts of interest.Study exempt from ethical approval.Written informed consent was obtained from the patient for publication of this case report and accompanying image.Study concept and design: Suman B Koganti MD.Data collection: Casey Joe MD.Data analysis and interpretation: Suman B Koganti MD, Vinayak Gowda MD.Writing the paper: Casey Joe MD, Suman B Koganti MD.None.Vinayak Gowda MD.Not commissioned, externally peer-reviewed.


Tailgut cysts arise from the remnants of the tailgut during the embryonic period. Although malignant transition of tailgut cysts is very rarely observed in the clinic, this congenital condition should be carefully monitored for early diagnosis and appropriate treatment, especially when the tailgut cysts are malignant.Here, we report the case of a 33-year-old man with retrorectal adenocarcinoma originating from the tailgut cysts. Magnetic resonance imaging (MRI) showed many cystic masses in the posterior rectal space, the largest of which was approximately 100 mm × 59 mm × 53 mm in size and compressed the rectum. The patient underwent surgical resection of the masses located in the retrorectal and anterior sacral spaces. Histological and immunohistological examinations confirmed adenocarcinoma transition of the tailgut cysts. The patient recovered well and was discharged 10 days after surgery.We have reported a rare case of retrorectal adenocarcinoma originating from tailgut cysts. MRI, histological, and immunohistological examinations are vital for the diagnosis of tailgut cysts. Complete surgical resection of the tumor should be better performed. Tailgut cysts, also known as retrorectal cystic hamartoma, arise from the remnants of the tailgut during the embryonic period . This coRetrorectal tumors are very rare, occurring with an incidence rate of approximately 1/40,000 , 4. FurtHere, we present a very rare case of retrorectal adenocarcinoma originating from tailgut cysts. We also discuss the characteristics, diagnosis, and surgical management of these cysts.A 33-year old man attended our hospital with a sacral mass (80 mm × 59 mm × 53 mm) detected by magnetic resonance imaging (MRI) two days previously. The patient defecated once every 4 to 5 days. The patient had no nausea or vomiting, no abdominal pain or distention, no blood in stools, and no rectal tenesmus. The patient attended our hospital for surgical treatment. During the course of the disease, the general condition, diet, and mental state of the patient were good. There was no history of cough, urinary frequency and urgency, or any other abnormal sensations. Based on the digital rectal examination (knee-chest posture), the mass was palpable within 4 cm at the 12 o’clock position. The mass was solid with a clear boundary, good mobility, and no tenderness. However, the upper edge of the mass could not be felt. In addition, there was no blood or pus staining on the surface of the glove. The MRI examination showed numerous short T1 signals and long T2 signals and many long T1 signals and mixed T2 signals in the posterior rectal space  infiltrating the smooth muscle wall and surrounding fibrous tissues Fig. . The immTailgut cysts are a very rare congenital polycystic lesion that always occur in the presacral space, the borders of which are the presacral fascia  posteriorly, the fascia propria of the rectum and mesorectum anteriorly, levator ani muscles inferiorly, the peritoneal reflection superiorly, and the iliac vessels and ureters laterally –10. The Prasad et al. proposed that tailgut cysts can be theoretically classified as cystic teratomas , 17. AltIn the diagnosis of tailgut cysts, different imaging methods are often faced with numerous differential diagnosis challenges . In the Surgical management is typically required for nearly all presacral tumors, and preoperative imaging serves as a road map for optimal resection. Although the absolute risk is unclear, some benign presacral tumors may undergo malignant progression, thus necessitating prompt surgical planning once a presacral tumor is diagnosed. Although malignant degeneration/progression may occur, urgent resection of simple, cystic-appearing lesions with features consistent with benign etiologies is unnecessary . HoweverThe spectrum of presacral pathology is broad, but the most commonly seen lesions are developmental cysts, ranging in size from a few centimeters to lesions that completely fill the pelvis . SmallerHere, we report a rare case of retrorectal adenocarcinoma originating from tailgut cysts. The diagnosis was confirmed by MRI, histological, and immunohistological examinations. Complete surgical resection of the tumor was performed, and the patient recovered well. We also describe the characteristics, diagnosis, and surgical management of retrorectal tumors.


Carney complex is a rare autosomal dominant disorder characterized by multiple tumors, including cardiac and extracardiac myxomas, skin lesions, and various endocrine disorders. We are reporting a 21-year-old female patient with past surgical history significant for excision of a cutaneous myxoma who presented with multicentric cardiac myxomas involving the four cardiac chambers. She also presented with endocrinal disorders in the form of an enlarged right lobe of the thyroid, hyperthyroid state, and an incidentally noted adrenal cyst; hence, she was diagnosed with carney complex syndrome. Cardiac myxoma is the most common benign cardiac tumor. It usually presents as a sporadic isolated condition in the left atrium of middle-aged women . A condiA 21-year-old female patient presented with progressive exertional dyspnea and irregular palpitations for 3 months. She had past surgical history significant for excision of a cutaneous myxoma in her left arm. Physical examination revealed a high jugular venous pressure and a diastolic murmur. An electrocardiogram showed atrial fibrillation. Laboratory investigations were within normal limits except for a low TSH and elevated free T3 and T4. Transthoracic echocardiography (TTE) showed a large echogenic mobile mass with central constriction attached to the interventricular septum (IVS), occupying the entire right atrium and right ventricle (RV) and obstructing the flow of the tricuspid valve. There were two other masses of the same echogenicity: one was occupying the left ventricle (LV) and the other was in the left atrium attached to the interatrial septum at the site of fossa ovalis . The lefThe patient underwent surgery where all three masses were excised. However, the tricuspid valve was inseparable from the RV mass; hence, it was replaced with a tissue prosthesis. The masses were grossly reddish grey in color, fleshy, and gelatinous in consistency . The hisPrimary intracardiac tumors are extremely rare in comparison to secondary tumors. However, myxoma is the most common primary cardiac neoplasm . CardiacPRKAR1A). However, twenty-five percent of the cases occur sporadically secondary to de novo mutation [Carney complex (CNC) is one of the familial forms inherited in an autosomal dominant fashion. CNC is most frequently associated with mutations in the protein kinase A type I-alpha regulatory subunit gene (mutation . CNC is mutation .Cardiac myxomas associated with CNC are characterized by the following: occurring at a younger age, multicentric, and a higher tendency of recurrence after resection in comparison to solitary ones , 4. Our CNC-associated skin lesions can present as a spotty skin pigmentation which occurs in more than 80% of patients  or a cutEndocrine abnormalities due to tumors of the adrenal and pituitary glands or testicular tumors are the most frequent systemic manifestations of CNC. Cushing's syndrome can occur in up to 45% of patients diagnosed with CNC . Our patThyroid disease is also common; almost 75% of patients with CNC will develop thyroid nodules typically early in life, and 25% will develop adenomatous disease. Less than 10% will develop thyroid cancer. Hence, fine-needle aspiration is indicated for patients with thyroid nodules. Regardless of the previous pathologies, most CNC patients are euthyroid . Our patPRKAR1A gene or a first-degree relative has CNC [In order to diagnose CNC, Stratakis et al. defined the diagnostic criteria . Diagnos has CNC , 10.PRKAR1A gene is more commonly used for diagnostic certainty. In addition, genetic screening of potentially affected family members of patients with CNC could be useful especially in younger family members. However, clinical surveillance is still advisable for family members even when a PRKAR1A pathogenic variant is not identified.Although the clinical diagnostic criteria are reliable, nowadays genetic testing for mutations in the In cases of cardiac myxomas, surgical resection is the treatment of choice with a 6-month follow-up via TTE afterwards due to the high incidence of recurrence. In addition, a multidisciplinary approach is recommended in order to screen for endocrinopathies and malignancies. Clinical surveillance is advisable for family members and possibly genetic testing. In our patient, clinical screening of the family was not significant; hence, we concluded she developed a de novo mutation.Multiple intracardiac myxomas account for less than 5% of all cases of myxoma. Its presence should warrant screening for endocrinal disorders and should be treated by complete surgical excision with a generous safety margin in addition to close follow-up as it carries high risk of recurrence.


To highlight the importance of histopathological evaluation of a lid mass to prognosticate the disease. We report a case of ulcerative squamous cell papilloma with clinical features suggesting malignancy.A 65-year-old man presented with a rapidly enlarging mass in the left upper eyelid, with clinical features suggesting a squamous cell carcinoma. However, a repeat histopathological examination showed no malignant cells. The patient was diagnosed with squamous cell papilloma. He was followed-up for 30 months and no recurrence was observed. No such case has previously been reported in the literature.This report highlights the need for histopathological examination of all eyelid lesions to enable surgeons to prognosticate the disease. Feeder vessels, hyperkeratosis, leukoplakic plaque, keratinization, scaling, and ulceration are considered as features suggestive of malignancy. Nevertheless, atypical malignant behavior of a benign eyelid lesion is unusual. Herein, we report the case of a lesion presenting with typical malignancy features that was histopathologically found to be benign. To the best of our knowledge and based on a review of the literature, ulcerative squamous papilloma of the eyelid has not been reported thus far.A 65-year-old man presented with a mass on the left upper eyelid that had been progressively enlarging for the past seven years. A pedunculated growth measuring 20 Histopathological examination revealed no atypia; rather, the lesion was composed of papillae with vascularized connective tissue covered by acanthotic epithelium. Hence, it was diagnosed as ulcerative squamous cell papilloma [Figure 3]. A repeat examination with repeat tissue cuts and histopathology slide preparation performed by another pathologist did not change the diagnosis. The patient was followed-up for 30 months and no recurrence was observed.,4Ocular papillomas favor sites where the epithelium undergoes transition. They can occur at either the limbus or the eyelid margin. Papillomas typically arise from the basal cells but, rarely, may form through the proliferation of squamous cells. Squamous cell papilloma is the most common benign lesion of the eyelid, constituting 13–19.5% of benign eyelid tumors. Our case revealed an absence of atypia on repeat examination. Furthermore, the patient was followed-up for two and a half years with no recurrence.For squamous cell papilloma, having surface ulceration and indurated margins is not unusual. In the present case, the clinical appearance suggested malignancy, but histopathology changed the diagnosis. Kersten et al reported that the majority of the initially benign lesions were found to be malignant on repeat histopathology.,7 This also emphasizes the need for histopathological examination of all eyelid lesions to enable surgeons to prognosticate the disease and advise patients accordingly.Although rare, case reports of surface pigmentation and hyperkeratosis in squamous papilloma exist.,9 The long-time closure of the eyelids, and the need for a second surgery are the major disadvantages of this procedure.The gold standard treatment for eyelid carcinomas is surgical resection with clear margins followed by reconstructive procedures. Cutler–Beard flap surgery is a successful procedure for superior eyelid tumors with wide tissue loss.The patient gave consent for reproduction of the photographs in the journal.The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that his name and initial will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.Nil.There is no conflict of interest.


Axillary region is one of the areas where primary hydatid cyst is rare. In this study, we present a case of isolated axillary hydatid cyst in a 40 year-old female patient having applied to our polyclinic with swelling and pain on right axillary. During the breast examination of the patient on right axillary, well-circumscribed semi-mobile mass lesion was detected. On mammary ultrasonography, both breasts were natural. There was necrotic lymphadenopathy on right axillary that was roughly 10×10 cm sized, and locally included cystic patency. Axillary LAP excision was planned for histopathologic diagnosis. Patient was taken to the operation. After it was understood that cyst was hydatic, it was excised with germanium membrane by encircling it with savlon compresses. In order to differentiate primary secondary on post-operative patient, the patient was taken to thoracic and abdomen tomography. No cystic lesions were detected on tomography. Having no problems on follow-ups, the patient was discharged on 3 post-op days with recommendations, and with starting albendazole 10 mg/kg. Hydatid cyst disease is one of the zoonoses caused by Echinococcus granulosus showing the wide geographic spread in the world . In humaA 40 year-old female patient applied to our polyclinic with swelling and pain on right axillary which had been continuing for about 2 months. During the breast examination of the patient who had no breast cancer cases in her family history, no features were detected on both breasts and left axillary. On right axillary, well-circumscribed semi-mobile mass lesion was detected. No features were found on biochemical investigations. On mammary ultrasonography (USG), it was reported that both breasts were natural, and there was necrotic lymphadenopathy (LAP) on right axillary that was roughly 10×10 cm sized, and locally included cystic patency. Axillary LAP excision was planned for histopathologic diagnosis. The patient was taken to the operation. By right axillary incision, skin and subcutan were passed. Cystic mass lesion was at axillary area. While trying to take of the lesion, capsule was perforated. Rock water and female vesicles were drained out .After it was found out that cyst was hydatic, it was excised with germanium membrane by encircling it with savlon compresses. In order to differentiate primary secondary on postoperative period, the patient was taken to thoracic and abdomen tomography. No cystic lesions were found on tomographies . Having Hydatid cyst disease is still an important health issue in agricultural communities, and Turkey is one of these countries .In humans, the disease is usually localized to the liver and lungs; and 13.9% of the cases have a rare localization of the hydatid cyst . Among tPatients with axillary hydatid cyst are usually at the age range between 25 and 45, and they are often presented with pain and swelling at the axillary area. Our case was a 40 year-old woman. Her physical examination revealed that she had a semi-mobile palpable lump in her right axilla. Imaging methods were very helpful in the diagnosis of the disease, often revealing some specific signs of the disease. Ultrasonography, computed tomography and magnetic resonance imaging can be used to visualize the cyst’s relation to the surrounding tissues, internal luminal wall of the cyst, and intraluminal daughter cysts .Echinococcus granulosus infection can lead to structural deformities in the tissue, and particularly the unilocular hydatid cysts with moderate or hyper densities can be confused with other pathologies like sarcoma or conglomerated lymphadenopathies (In our case, considering the possibility of breast cancer metastasis as the cause of the lesion, the patient was examined with bilateral breast and axillary ultrasonography. Breast and axillary ultrasonography was reported as bilaterally normal breast tissue, and necrotic lymphadenopathy at the right axillary area which included few cystic cavities. Over time, opathies –13. The Serological tests may aid in the diagnosis, but serological tests have high false positivity rates , 9. SincAlthough our region is an endemic region for hydatid cysts and frequent occurrence of hydatid cysts, it has been ruled out that the lesion might be hydatid cyst at ultrasonografi due to the rare localization of the cystic site. After the ultrasonography examination, it could be more appropriate in our case to confirm the diagnosis with an advanced imaging modality such as magnetic resonance imaging or computed tomography, and to conduct proper preoperative medical preparations.The disease was treated with total excision of the cystic lesion following prophylaxis with albendazole. Besides, other commonly affected sites should also be investigated because of the fact that primary axillary hydatid cyst disease is extremely rare. Seeing that our case was diagnosed intraoperatively, no preoperative prophylaxis was administered. The lesion was excised totally. The patient was scanned with thoracic and abdominal tomography at the post-operative period, and her treatment was completed because any other pathology was not detected.Although primary axillary hydatid cyst is an extremely rare disease, it should be considered among the differential diagnosis for patients presented with axillary swelling symptoms in the endemic areas.


Uterine fibroids are incredibly common, benign smooth muscle tumors which range in severity of symptoms from asymptomatic to debilitating. While pain is frequently a symptom, degeneration and necrosis of uterine fibroids can rarely present as acute abdomen. The authors present the case of a pregnant female at 19 weeks' gestation, whose clinical and radiographic presentation mimicked that of ovarian torsion, ultimately requiring exploratory laparoscopy for definitive diagnosis. Uterine fibroids, also referred to as leiomyomata, are benign smooth muscle masses of the uterus, with the prevalence rates exceeding 80% in some female populations . While tA 30-year-old primiparous female at 19 weeks' and 2 days' gestation by anatomical fetal ultrasound presented to the emergency department with a chief complaint of left lower quadrant abdominal and pelvic pain for the previous five days. The patient denied vaginal bleeding, discharge, or fluid leakage. Vital signs at triage were a blood pressure of 141/65 mmHg, a heart rate of 65 beats per minute, a respiratory rate of 16 respirations per minute, an oxygen saturation of 100% on room air, a temperature of 98.1 degrees Fahrenheit, and a pain level of 4/10. Physical examination was pertinent for a gravid uterus consistent with gestational dating and mild tenderness to palpation of the left lower quadrant of the abdomen without guarding or rigidity. Laboratory workup including complete blood count, basic metabolic panel, urinalysis, and quantitative hCG studies was unactionable. With concern for diagnosis of left ovarian torsion, a transabdominal ultrasound study was immediately performed, confirming a single, living intrauterine pregnancy with a fetal heart rate of 152 beats per minute. The study also revealed a left-sided, 5.8 x 4.0 x 5.8 cm exophytic fibroid at the left uterine fundus without internal vascular flow, replicated on an anatomical scan performed the day prior. While a pedunculated fibroid was listed as a possible diagnosis, no stalk was visualized on this lesion and the left ovary was also unable to be visualized, leading to the suspicion of ovarian torsion. Obstetrics and gynecology (OBGYN) was consulted for further evaluation and stated low current clinical suspicion for acute ovarian torsion at that time and arranged for next day clinic follow-up. Upon emergency physician re-evaluation of the patient, the patient appeared to be more tender than on previous assessment. An emergent pelvic magnetic resonance image (MRI) was ordered for continued suspicion of ovarian torsion. The MRI again revealed a left adnexal mass, with suggested internal hemorrhage and continued concern for ovarian torsion given no vascular flow present on sonogram Figure , 1B. WitOvarian torsion during pregnancy has an estimated incidence of five in 10,000 pregnant patients which is five times higher as compared to non-pregnant women, and may pose an immediate threat to the health of the mother, the pregnancy, and future fertility potential . SimilarPrevious studies portray conflicting data regarding uterine fibroids changes during pregnancy, with data to support both increasing and decreasing size with hormone fluctuations . However Diagnosis of uterine fibroids is often accomplished with ultrasonography, and absence of flow may be demonstrated in torqued or necrotic lesions, which is a rare complication. Similar to other ultrasonography, sensitivity can vary with operator dependence, and as in the case described, a pedunculated fibroid may be mistaken for an adnexal mass . Pelvic Pelvic and abdominal pain due to fibroids in pregnancy is most often successfully treated conservatively with analgesia; however, surgical management in select cases may be pursued. While no unanimous consensus has been adopted, several guidelines for identifying surgical candidates have been proposed based on factors such as fibroid type, size, location, refractory symptoms, and proximity to the placenta ,8.In a retrospective case series, in which pregnant women underwent surgical myomectomy for recurrent pain, large or rapidly growing lesions, or myomata in the lower uterus or those affecting placental site, women gave birth to healthy weight babies with good APGAR scores, and without increased risk of preterm delivery, as delivery with previous myomectomy has demonstrated lower five-minute APGAR scores, increased rates of cesarean section, and increased rates of primary postpartum hemorrhage . This deNecrotic fibroid lesions are a rare source of acute abdominal pain in the emergency department and may mimic ovarian torsion in both patient presentation and radiographic evaluation. Emergent consultation with OBGYN is necessary for further evaluation including possible diagnostic laparoscopy for definitive diagnosis. With a formal diagnosis of uterine fibroids, analgesia is often sufficient for the treatment of symptoms. While surgery is generally avoided in pregnancy, carefully selected patients may safely benefit from myomectomy without adverse risks to the unborn child.


Radical cystectomy and urinary diversion remains the definite management for muscle invasive bladder urothelial cancer. Internal herniation caused by ureteral adhesion is an extremely rare complication after the procedure. To the best of our knowledge, this is the first case report of small bowel obstruction and internal herniation occurring between bilateral ureters and urinary diversion after robot-assisted radical cystectomy (RARC).A 64-year-old woman presented with symptom of small bowel obstruction such as nausea, vomiting, and abdominal fullness after RARC and Indiana pouch. Another 61-year-old man presented with left obstructive hydronephrosis and recurrent pyelonephritis after RARC and ileal conduit.Both patients received computed tomography scans and the results were suggestive of small bowel herniation between bilateral ureters and urinary diversion.The 2 patients underwent open ureterolysis and internal hernia reduction. During the operation, bowel loop herniation between the interureteral spaces were found.Both patients recovered smoothly after second operation.The incidence of internal herniation may increase by the growing use of RARC. Suitable stoma position, appropriate length of ureter dissection, and retroperitonealization can help prevent this complication. The difficulty has increased with the introduction of the laparoscopic or robotic method. Myers et al had reported that perioperative complications after Indiana pouch creation or similar continent diversion may be complicated by wound infection (12%–33%), bowel leakage (1%–6%), and ureteral stricture (1%–21%). However, internal herniation caused by ureteral adhesion is extremely rare. We report the first case, to our knowledge, of small bowel obstruction and internal herniation occurring between bilateral ureters and urinary diversion after the procedure of robot-assisted radical cystectomy (RARC). Both patients have provided informed consent for publication of the cases.Among the diversion methods, continent cutaneous diversion by Indiana or Kock pouch creation serves as an alternative option for patients in whom continence is desired in the setting of a nonfunctional urethra or a positive intraoperative urethral margin precluding the ability to perform orthotopic diversion.22.1A 64-year-old woman had undergone RARC with Indiana pouch creation for urinary bladder urothelial carcinoma (UBUC). The Indiana pouch construction was performed extracorporeally with an 8-cm incision from the extended robotic trocar wound. Her left ureter passed underneath the sigmoid mesentery as a standard procedure. She recovered well after the procedure. However, on the postoperative day 38, she presented to the emergency department with chief complaints of fever and left flank soreness. Her body temperature was 38.9 °C. Physical examination revealed left costovertebral angle knocking tenderness. Laboratory test revealed leukocytosis 12,500/μL) and elevated C-reactive protein (14.28 mg/dL). Urine analysis revealed pyuria (208/μL). Abdominal computed tomography (CT) showed bilateral hydronephrosis, periureteral fat stranding, and pouch bowel wall swelling. She underwent bilateral percutaneous nephrostomy catheter insertion for obstructive uropathy and received antibiotic treatment for acute pyelonephritis. Twenty days later, she developed aggravated abdominal distention, nausea, vomiting, and constipation. Abdominal radiography revealed a distended bowel loop consistent with bowel obstruction. Abdominal CT demonstrated an obstruction ileus with a distal intestinal transition zone and internal herniation between bilateral ureters and Indiana pouch (Fig. 2,500/μL During laparotomy, bilateral ureteroenterostomy sites were first identified, and then 1 segment of the ileal herniation adhered between bilateral ureters was detected (Fig. 2.2Another 61-year-old man underwent RARC with ileal conduit for UBUC. He experienced left pyelonephritis postoperatively. Abdominal CT 30 days after the surgery revealed left distal ureteral stricture with hydroureteronephrosis. The stricture was around 4 cm between the peritoneal window beneath the sigmoid colon and the ureteroenteric anastomosis site Fig. A. FlexibDue to recurrent pyelonephritis, he underwent open ureterolysis 6 months after RARC. During the procedure, we noted a small bowel loop herniating from the space between bilateral ureters and the conduit. The ureteral stricture was surrounded by the herniated loop, and adhesion between the ureter and intestine was observed Fig. B. After 3,9 To the best of our knowledge, this is the first case report of small bowel obstruction and internal herniation between ureters and urinary diversion after RARC.Small bowel obstruction resulting from internal herniation between ureters and urinary diversion is extremely rare after radical cystectomy. Only 2 similar cases have been reported in the literature. This trocar position is at the umbilicus level which is much higher than the usual position. The tented pouch/conduit and ureters, therefore, acted as a “hang rope” for the herniated segment. Abdominal X-ray of the case 1 patient showed a distended small bowel. Because of the high position of the stoma site, the bilateral ureters formed a U-shaped bend and lead to strangulation of the hernia loop (Fig. Three factors may contribute to this rare complication. First, for cosmetic purposes we used the robotic trocar on the patient's right side as the position of the diversion stoma, rather than performing another incision.oop Fig. . Second,With the growing use of RARC for UBUC, the complication of small bowel internal herniation beside ureters may be more common in the future. Suitable stoma position, appropriate length of ureteral dissection, and retroperitonealization can help prevent such a catastrophic morbidity.Conceptualization: Chao-Hsiang Chang.Investigation: Wei-Juan Li, Guang-Heng Chen.Supervision: Chao-Hsiang Chang.Writing – original draft: Li-Hsien Tsai.Writing – review & editing: Po-Fan Hsieh.


Torpedo maculopathy is a rare, benign, and congenital macular lesion that typically appears in a ‘torpedo-shape’ and is located at the temporal macula region. This study aimed to describe in detail regarding torpedo maculopathy in a Chinese woman using multimodal imaging.A 30-year-old Chinese woman with occasional yellowish-white macular lesions in her right eye during a routine examination was presented to our hospital. She had no other symptoms, and the best-corrected visual acuity of both eyes was 6/6. Funduscopic examination revealed a torpedo-shaped and mild hypopigmented lesion in the temporal macular area of her right eye. Infrared fundal (IR) images showed visible lesion contour, transverse elliptical, and with a tip pointing towards the central fovea of the macula. Microperimetry visual field appeared normal. The spectral-domain optical coherence tomography (SD-OCT) showed a normal inner retina, with mild thinner outer retina and retinal pigment epithelium in the temporal macular area, and correspondingly increased choroidal reflectivity. Other OCT findings included outer retinal loss/attenuation with significant atrophy of an intact ellipsoid zone. OCT angiography (OCTA) of choroid capillary layer revealed increased density of choroidal vasculature in corresponding to the area of the lesion, while the superficial and deep layers revealed normal vasculature. Fundus autofluorescence (FAF) revealed normal signal with slight hyperautofluorescence at the nasal lesion margin. Fundus fluorescence angiography (FFA) of the lesion showed variegated fluorescence and no leakage and change in the morphology during the whole imaging process.This is the first report to include a thorough and detailed description of torpedo maculopathy by using fundal photograph, IR, microperimetry visual field, OCT, OCTA, FAF, and FFA. Multimodal imaging provides precious and detailed information to further clarify the characteristics and development of this rare disease. Torpedo maculopathy is a rare, benign, well-defined and congenital retinal pigment epithelium (RPE) disease that typically appears as a ‘torpedo-shaped’ lesion in the temporal macula. It was first reported by Roseman and Gass in 1992  due to iHowever, the etiology of torpedo maculopathy remained unknown. Many imaging devices have been used to observe the lesion. We herein used multimodal imaging for describing torpedo maculopathy in a Chinese woman. To our knowledge, this is the first report in the world to observe torpedo maculopathy by using fundal photographs, IR, microperimetry visual field, optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), fundus autofluorescence (FAF), and fundus fluorescence angiography (FFA). We hypothesized that this case might be in a very early stage or is a mild type of torpedo maculopathy.A 30-year-old Chinese woman with yellowish-white macular lesion in right eye during a routine examination presented to our hospital. She had no other symptoms, and had no pain or vision loss in her right eye. The patient has no traumatic history. Her past medical history and ophthalmic history were negative. The initial best-corrected visual acuity (BCVA) was 6/6 for both eyes. The cornea was clear, and the anterior segment was normal. Pupils were equal, round and reactive to light with no afferent pupillary defect. There was no cataract in both eyes. The initial intraocular pressure (IOP) was 14 mmHg in the right eye and 13 mmHg in the left eye. Funduscopic examination of the left eye was unremarkable. A spindle-shaped yellowish-white and hypo-pigmented lesion of about 0.5 disc diameter vertically and by 1 disc diameter horizontally was located in the temporal macular area with a tip pointing towards the central fovea of the macula Fig. . The SD-The treatment for this patient was closely observed. The patient was followed-up every 3 to 6 months. Her BCVA remained still 6/6 in both eyes, and the fundus lesion still remained intact during follow-up at 15 months.Torpedo maculopathy is a rare, benign, well-defined and congenital maculopathy, which is usually asymptomatic, but occasionally found during routine examination. It often occurs in patients without any relevant medical history and is most commonly unilateral, although bilateral cases have been reported previously . The torTill now, the etiology of this disease still remained unclear. Few studies have been reported to explain the pathogenesis of this lesion. Pian et al.  assumed Microperimetry precisely revealed the correlation of retinal sensitivity and fundus lesion, and also detected microscotoma within the central visual field that may not be detectable with other standard perimetry methods . PublishUsing SD-OCT, the outer retina was disorganized, and showed significant atrophy of RPE with an intact ellipsoid zone in this case. We thought that the RPE still preserved the function of ingesting photoreceptor cell outer segment, and so the ellipsoid zone still remained intact and the patient has normal visual function. Evan et al.  identifiOCTA non-invasively detects the movement of red blood cells to reveal the retinal and choroidal vascular system. Therefore, alterations in choriocapillaris can be visualized by using OCTA. Papastefanou and his colleagues  have desAutofluorescence signal is predominantly derived from lipofuscin within the RPE . In our FFA of the lesion showed variegated fluorescence and no leakage and change in the morphology during the whole imaging process. This demonstrated no choroidal neovascularization (CNV), while few other cases showed the existence of CNV . LesionsDifferential diagnosis such as posterior uveitis should be carefully addressed. Posterior uveitis is also known as choroiditis, which is ill-defined, and characterized by vitreous exudation and choroidal vasodilatation, resulting in CNV and visual reduction. OCTA, FFA and FAF examinations of posterior uveitis are distinguished from torpedo maculopathy. Early hypo-fluorescence followed by late leakage on FFA was observed in active choroiditis lesions. However, healed lesions presented hypo-fluorescence during the early phase on staining but no leakage was observed in the late phase. According to FAF, ill-defined hyper-autofluorescence was observed in the active choroiditis lesions, while rounded edges and hypo-autofluorescence within the lesion were shown in healed choroiditis . These aHowever, there are several limitations in this study. The sample size of the study was small, and indocyanine green angiography was not performed as it helps in better understanding of the differential diagnosis of this disease. Besides, no further follow-up examination is considered to be a limitation for this study.A case of torpedo maculopathy by using fundal photographs, IR, microperimetry visual field, OCT, OCTA, FAF, and FFA has been presented for the first time. it might be in a very early stage or is a mild type of torpedo maculopathy. The natural development of torpedo maculopathy still remained unclear, whether it develops very slowly or does not develop. Multimodal imaging provides precious and detailed information to easily diagnose and understand this rare disease. This is a very rare disease, and requires more case reports worldwide and longer follow-up time to further understand the etiology, characteristics and development of this lesion.


Gastric duplication cysts are uncommon congenital anomalies found primarily in children and rarely seen in the adult population. Accurate diagnosis of cysts before resection is difficult even using the most advanced imaging techniques.In this report, we describe a 28-year-old Moroccan patient with a history of autoimmune hemolytic anemia who presented with an asymptomatic abdominal cystic mass detected during abdominal computed tomography performed before splenectomy. Magnetic resonance imaging performed for accurate characterization showed a high-signal-intensity cystic mass on T2-weighted images, located between the patient’s stomach and spleen. The patient underwent a complete cyst resection during exploratory laparotomy. The histological examination showed a cyst lined by three different epithelia with bundles of smooth muscle, which suggested a gastric duplication cyst.We report a case of gastric cyst duplication in an adult with autoimmune hemolytic anemia, and we discuss this rare association, radiological findings, and the unique histological findings of this case. A gastrointestinal duplication is defined as a spherical cystic structure lined by a mucous membrane with smooth muscle in its wall . It may We present a case of a 28-year-old white man with an 8-month history of clinical symptoms of anemia, such as fatigue and breathlessness, without any abdominal complaint. He had autoimmune hemolytic anemia treated by corticosteroids for more than 6 months but with a relapse of disease after steroid remission. For this reason, he was a candidate for a splenectomy. His occupation is a student. He did not smoke tobacco or consume alcohol, and he was taking prednisone, bisphosphonates, vitamin D, and calcium. No other immune disorders were found. On admission, his blood pressure was 90/70 mmhg, his heart rate was 70 beats/minute, and his body temperature was 36 °C. Physical examination of the patient revealed a severe pale conjunctiva and icteric sclera with no evidence of abdominal mass or other physical abnormalities. Laboratory analysis results on admission are shown in Table Abdominal computed tomography (CT) performed before the splenectomy showed a large cystic mass between the stomach and the spleen with no evidence of communication with the stomach or pancreas. Endoscopic ultrasound (EUS) showed a cystic mass located along the greater curvature with no mucosal abnormality or communication with the gastric lumen. Magnetic resonance imaging performed for more characterization showed a cystic mass between the stomach and spleen with a high signal intensity on T2-weighted fat-saturated magnetic resonance images Fig.  and periThe patient underwent exploratory laparotomy with complete cyst resection and splenectomy. At gross examination, the cystic mass, measuring 8 × 5.5 × 4 cm, was well-circumscribed, unilocular, and filled with a mucoid yellowish fluid. Histologically, the cystic mass was lined by gastric epithelium with pyloric glands Fig. , transitThe histological examination of splenectomy showed pronounced cord congestion with reactive follicular hyperplasia and increased deposition of hemosiderin. After 10 months, there were no signs of local complication, and the patient was successfully weaned from steroids and had complete resolution of hemolytic anemia with negative direct antiglobulin tests.in utero ischemic events [In this report, we present a rare association of a hemolytic autoimmune anemia and an asymptomatic GDC in a 28-year-old man. This association has not been reported in the literature so far. We also describe a unique histological morphology of this cyst, which was lined by three different types of epithelium. Duplications of the alimentary tract are rare and occur in 1 in 4500 births [c events . The majc events .There are two types of duplication: tubular when the lumen is contiguous with stomach lumen and cystic when the lumen is not contiguous with stomach lumen . More thOur patient exhibited a unique histological finding: The lining epithelium was composed of both squamous and respiratory mucosa with the gastric epithelium, which has not been reported so far in the same cyst. The cyst lining may undergo erosions, ulceration, and regenerative changes due to increased fluid pressure. These changes may lead to bleeding into the cyst or perforation into the peritoneal cavity. These cysts are usually asymptomatic and are diagnosed incidentally, as observed in the cases in this report. However, in some cases, they have no specific symptoms, such as epigastralgia, vomiting, anemia, or recurrent episodes of pancreatitis. They may also manifest as complications such as gastric outlet obstruction, gastric perforation, and carcinoma arising in the cyst .Our patient’s case represents a very rare association between GDCs and hemolytic autoimmune anemia. Hemolytic anemia is commonly associated with lymphoproliferative neoplasms, drugs, connective tissue diseases, and infections and are very rarely reported in association with ovarian dermoid cysts and mesenteric dermoid cysts.There is only one reported case of a rare association between an enteric duplication cyst and autoimmune hemolytic anemia. In that case, there was an adenocarcinoma arising in the cyst, which refers to a paraneoplastic autoimmune hemolytic anemia . In our Although most of the diagnoses are made postoperatively, several imaging modalities have provided some informative findings. CT and EUS are very useful in identifying GDCs . ClassicEUS is useful in distinguishing between the intramural and extramural lesions of the stomach. Compared with CT, magnetic resonance imaging can provide additional information about cyst content. However, the nature of the fluid in the GDC is different in each case according to bleeding, chronic inflammation, or infection. Treatment is complete excision of the cyst to avoid the risk of possible complications such as perforation, hemorrhage, and the risk of neoplastic transformation , 13.A GDC is a rare congenital anomaly that is difficult to diagnose before complete resection. It should be considered in the differential diagnosis of abdominal cystic lesions. Its association with hemolytic autoimmune anemia raises the possibility of a morbid association and requires more reported cases.


Sirenomelia is a rare developmental malformation and is incompatible to life. The incidence of sirenomelia, as recorded in the literature, is estimated to be approximately between 1.5 and 4.2 per 1,00,000 births. Around 15% of sirenomelia cases are associated with twin pregnancy, most often in monozygotic cases with an incidence of 7%. In monozygotic twins, the risk of sirenomelia is nearly 100–150 times higher as compared to dizygotic twins or singleton pregnancies. Until now, only two cases of sirenomelia associated with hypoplastic left heart have been reported in the literature. Here, we present a monozygotic twin pregnancy, where one fetus was diagnosed with sirenomelia associated with hypoplastic left heart syndrome and the co-twin was absolutely healthy. Sirenomelia is a rare developmental malformation and is incompatible to life. It is characterized by a spectrum of fusions in varying degrees of the lower extremities, agenesis or dysgenesis of the kidneys, absent external genitalia, imperforate anus, lumbosacral and pelvic bone anomalies, and a single umbilical artery. Cardiac and central nervous system anomalies have also been reported sporadically .The alternate name of sirenomelia is the mermaid syndrome, as the fused lower extremities in sirenomelia cases resemble mermaids from the Greek and Roman mythology, which were depicted as having the head and upper body of a human and the tail of a fish .The incidence of sirenomelia, as recorded in the literature, is estimated to be approximately between 1.5 and 4.2 per 1,00,000 births. This anomaly is predominantly observed in male fetuses with a male-female ratio of 3 : 1 [Around 15% of sirenomelia cases are associated with twin pregnancy, most often in monozygotic cases with an incidence of 7%. In monozygotic twins, the risk of sirenomelia is nearly 100–150 times higher as compared to dizygotic twins or singleton pregnancies .Until now, only two cases of sirenomelia associated with hypoplastic left heart have been reported in the literature by Sirtori et al.  and TurgHere, we present a monozygotic twin pregnancy, where one fetus was diagnosed with sirenomelia associated with hypoplastic left heart syndrome and the co-twin was absolutely healthy.A 32-year-old, gravida 8, para 5, aborta 2 monochorionic diamniotic twin pregnancy (MCDA) was referred as a high-risk pregnancy to our fetal assessment clinic at 25 weeks of gestation for detailed fetal ultrasonography (USG).The woman had no history of any past medical illness. All her previous pregnancies had resulted in healthy live births at term by vaginal delivery. This pregnancy had been conceived naturally, and she had not been detected to be hypertensive or diabetic. Furthermore, there was no history of consanguinity with her husband.The USG revealed an MCDA twin pregnancy with discordant growth (28%) of the twins. One fetus was detected to have left hypoplastic heart, with a single umbilical artery, the absence of the kidney and bladder, and lower extremity deformities with an absence of feet. The other fetus was observed to have a normal growth and biophysical profile. The placentas appeared fused in the posterior uterine wall with barely detectable dividing membranes. The amniotic fluid index was grossly normal for both fetuses. Fetal echocardiography was performed and revealed hypoplastic left heart, transposition of the great arteries (TGA), and pulmonary atresia.The patient and her husband were informed regarding the poor prognosis of the anomalous twin fetus. At 36 weeks of gestation, after antenatal steroid coverage, an elective caesarean section was performed for the patient in view of the MCDA twin with malposition of the first twin in accordance with the hospital protocol.The neonate was born with severe growth restriction with a weight of 1300 gm and cyanosed with a poor Apgar score. As a result, soon after the delivery, it was shifted to the neonatal intensive care unit (NICU). Physical examination of the baby revealed microcephaly (head circumference 29 cm), low-set ears, right hand with polysyndactyly, and complete fusion of the entire lower extremities with an absence of feet (Figures Radiologic examinations revealed vertebral deformity at the midthoracic spine, partial sacral agenesis, bilateral hypoplastic iliac bones, complete fusion of both femurs , fused pNeonatal echocardiography was performed, and the diagnosis of hypoplastic left heart syndrome, TGA, and pulmonary atresia was made. USG abdomen and pelvis demonstrated an absence of the bladder and both kidneys with other normal abdominal organs. Chromosomal analysis confirmed the female karyotype 46,XX. The neonate survived for 3 days under human care. An autopsy was not performed due to cultural and social constraints. The other twin was healthy, weighing 2500 gm, and was discharged in good condition after routine preterm care.The phenotypic characteristics of sirenomelia are associated with varying degrees of severe multiorgan abnormalities commonly involving the urogenital and gastrointestinal systems. Other observed abnormalities in sirenomelia involve lumbosacral and pelvic malformations, which include sacral agenesis, malformed vertebrae and hemivertebrae, absent or malformed external and internal genitalia, imperforate anus, and CNS abnormalities. Pulmonary and cardiac defects are less commonly encountered in sirenomelia .Maternal diabetes mellitus, teratogenic drug or agent exposure, or genetic predisposition is suspected to be the causal factors of sirenomelia. However, the underlying mechanism is still unclear, and many hypotheses have been generated to explain its aetiology. Among the various theories, two important hypotheses, the vascular steal hypothesis and the defective blastogenesis hypothesis, have been described in the literature to explain its pathogenesis. The first theory explains that the abnormal vascular pattern of abdominal and umbilical vasculatures in sirenomelia results in a primary vascular defect that leaves the caudal part of the embryo hypoperfused. The second hypothesis states that, during the blastogenesis of the embryo, damage to the caudal mesoderm leads to overall malformation of the caudal body , 6, 7. SDuring prenatal sonographic diagnosis of sirenomelia, the first sign is progressive oligohydramnios in the second trimester secondary to renal agenesis or abnormalities. The condition is easier to diagnose in the first trimester when the amniotic fluid volume is usually normal and unrelated to the fetal urine production , 7.Surprisingly, in our case, the amniotic fluid volume was normal in both amniotic sacs at 25 weeks of gestation.In a study involving nine sirenomelia cases, it was observed that five cases had a normal amniotic fluid volume .In another reported case, initially at 18 weeks, marked oligohydramnios was found, but the liquor volume improved week by week. By 25 weeks, an amniotic fluid index of 11.1 cm was seen .In previous studies of sirenomelia in twin pregnancies, expectant management is suggested despite there being a potential risk of preterm delivery or harm to the healthy co-twin in cases of intrauterine death of the affected twin, especially in monozygotic twins. This is owing to the presence of communicating blood vessels between the two fetoplacental circulations .Our case was managed conservatively, and both fetuses were successfully delivered by elective caesarean section at 36 weeks of gestation. The sirenomelic baby survived for 3 days. Although most affected newborns can survive for a few hours, the presence of normal amniotic fluid volume may be the reason of improved respiratory condition that favoured the ex-uterine survival of the infant for few days in our case .Survival of affected infants depends on the degree of the visceral anomalies and the presence of the kidney and renal functions. Death is usually caused by obstructive renal failure secondary to renal agenesis or dysgenesis .There is no robust evidence in the literature pertaining to the association of cardiac anomalies with sirenomelia. In sirenomelia cases, type IV truncus arteriosus is seen commonly but other cardiac abnormalities are encountered sparsely. Till date, only two cases of sirenomelia with hypoplastic left heart syndrome have been reported in the existing literature , 6.In our case, at 26 weeks of gestation, hypoplastic left heart, TGA, and pulmonary atresia were detected by fetal echocardiography, which were confirmed by neonatal echocardiography later on. Ours is the third reported case of sirenomelia association with hypoplastic left heart syndrome.In this case, the aetiology of sirenomelia remains unclear. There was no history of maternal diabetes or other risk factors, and as the co-twin fetus was healthy, we can exclude environmental factors or teratogenic agent exposure from aetiological factors as both twins would have been equally exposed to the predisposing factors. The causal factor may be associated with genetic aberration in our case. Genetic counselling should be proposed to the parents, as the risk of recurrence is around 3–5% .


Coalition between the pisiform and the hamate is a rare congenital anomaly, often presenting as an asymptomatic incidental finding on radiographs. In some cases, it may become symptomatic, typically after trauma. We present a 13-year-old patient, with no history of trauma, presenting a bilateral painful coalition of the pisiform and hamate. Both of which were treated with excision of the pisiform resulting in complete pain relief. Pisiform-hamate coalition is a rare condition, which can become symptomatic even without any trauma or overuse activity, and is an important differential diagnosis in ulnar-sided wrist pain. Carpal coalition is a rare congenital anomaly characterized by the union between two or more bones . It is mA 13-year-old, right-hand dominant boy with African origins was referred to our consultation because of bilateral ulnar-sided wrist pain, with no history of trauma. At physical examination, tenderness was elicited by direct palpation of the slightly swollen skin over the pisiform on both sides. Symptoms could be reproduced by extreme ulnar deviation of the wrist. The sensation and motor function were preserved, and Hoffman-Tinel's sign along the ulnar nerve was negative. Radiographs from both sides showed a partial coalition between the pisiform and the hook of the hamate . The diaSurgical exploration was performed first on the more symptomatic left-hand side. Guyon's canal was opened and explored through a longitudinal incision. The ulnar artery and nerve were mobilized and retracted radially. The synchondrosis was exposed, and the pisiform was detached from the flexor carpi ulnaris tendon. The pisiform was removed entirely, and the remaining hook of the hamate was rounded with a rongeur. The wrist was then immobilized in a plaster cast for two weeks postoperatively. Nine weeks after the surgery, the patient reported to be pain free, and the other side was operated in the same way. At the last visit, one year postoperatively, the patient was pain free on both sides with a grip strength of 39 kg on the left side and 40 kg on the right side, and a wrist motion of 75° of flexion and 70° of extension on the left side and 75° flexion and 75° extension on the right side.Coalition of two or more carpal bones is a congenital anomaly, usually occurring between carpals of the same row  with lunate-triquetrum coalition being the most common one . Hamate-The present case is a type 1 coalition according to the classification of Devilliers Minnaar , showingThe removal of the pisiform which is not a very demanding procedure, commonly used for pisotriquetral osteoarthritis, was able to completely resolve the symptoms on both sides. This is why we would recommend this as the treatment of choice if facing this condition.Coalition between the pisiform and hamate is a rare congenital condition which is mostly an asymptomatic incidental finding. However, it can become painful with or without any history of trauma and is an important differential diagnosis in chronic ulnar-sided wrist pain. Surgical excision of the pisiform may completely relieve the pain.


Leiomyomatosis Peritonealis Disseminata (LPD) is an uncommon disease characterized by presence of Multiple Leiomyomas in the abdominal and pelvic cavity. Increasingly, literature supports that LPD is an iatrogenic condition. Malignant transformation of LPD is rarely reported. We hereby report a case of a middle-aged female patient who was diagnosed with malignant sarcomatous degeneration in LPD. Gynecologists may get inspiration from this case report. Leiomyomatosis Peritonealis Disseminata (LPD) is a disease with low incidence which is featured by multiple nodules in the abdominal and pelvic cavity. Malignant transformation of LPD is extremely rare. Here we report a middle-aged female patient who was diagnosed with LPD and malignant sarcomatous degeneration. Recently, increasingly literatures have supported that LPD is an iatrogenic condition due to the use of morcellation. As such we introduce an innovative method to solve this problem which can safely remove the large fibroids in laparoscopic myomectomy without the use of morcellation.A 47-year-old female, with a history of laparoscopic subtotal hysterectomy and bilateral salpingectomy ten years ago, was hospitalized in September 2018. The main reason of the surgery ten years ago was mutiple myomas and the corpus uteri was taken out after the use of morcellation. She presented a rapidly growing tumor in her pelvic cavity, which was about 9 centimeters in diameter when she was hospitalized. The woman's condition was evaluated with transvaginal three-dimensional sonography and magnetic resonance imaging (MRI) after admission. The ultrasound images depicted multiple, solid, hypoechoic mass lesions in pelvis . Color DThe patient underwent exploratory laparotomy with bilateral oophorectomy; residual cervix was also removed. All of the mass lesions varying in size from 1 cm to 9.5 cm were resected from the pelvis. These nodules scattered located on the surface of retroperitoneum, sigmoid colon and urinary bladder . All the2). Three months after the chemotherapy, she remained asymptomatic and no abnormal imaging results were found.Intraoperative frozen section revealed spindle-shaped tumor cells with histopathological atypia, which needed routine histologic examination and immunohistochemistry for diagnoses. The operation was successfully performed. Histopathological examination, after the surgery, confirmed the diagnosis of LPD with cellular leiomyoma and sarcoma degeneration. The pelvic nodules were composed of close-arranged spindle cells which were featured by nuclear enlargement, pleomorphism and atypia . On immuLPD was first reported in 1952 by Wilson , 4. SeveNowadays increasingly reports described laparoscopic myomectomy and morcellation as the contributing factor of LPD , 10. MorIn addition to this most important advantage, the new technique can also shorten operation time, reduce the operation cost and avoid the extension of abdominal incision to remove the tumor. In our experience, this method to remove myoma is easy to grasp and cost-effective. Further randomized controlled clinical trial is needed to evaluate the significance of this method in decreasing the rate of LPD.


Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. The age of presentation is most often during childhood or adolescence. MRI has a central role in the assessment of the RPON, especially to distinguish orbital, parasellar, or posterior fossa lesions that mimic symptoms of RPON. Actually, oculomotor nerve tumors may be masquerade as RPON so that MRI follow-ups are required to detect the possibility of tumor etiology.  We report a 16-year-old boy with a 7-year follow-up and multiple brain MRI data, previously diagnosed as OM. The last brain MRI, performed during an acute phase of oculomotor paresis with ipsilateral headache, showed a nodular lesion described as schwannoma of III cranial nerve. Then, we reviewed the literature on OM and RPON in pediatric age with a focus on brain MRI findings.  This review highlights the important role of serial brain MRIs in the long-term follow-up of RPON, especially in the cases with childhood onset, in order to not delay the diagnosis of a possible oculomotor nerve schwannoma. The cha(ICHD) 208 has been (IASP) . The dia variant , but in ition 198 of the I variant . Moreove variant , 12. The variant –13. In t variant  about thAn electronic literature search was conducted for all articles published up to December 2018, with a range of databases searched including PubMed, Scopus, and Web of Science. The following combinations of keywords were searched: “opthalmoplegic migraine AND magnetic resonance imaging” and “recurrent painful ophthalmoplegic neuropathy AND magnetic resonance imaging.” The initial search returned 227 records; we considered 117 publications after screening to remove duplication of papers from different sources of research. Research articles, case reports, and reviews describing patients under the age of 18 years have been included. As inclusion criteria papers needed to provide enough information to extract clinical features of headache and oculomotor involvement and a description of MRI findings, 28 publications for a total of 43 clinical cases were selected for inclusion in this review.We reported the case of a male patient, 16 years old, with a history of frontal headaches accompanied by photophobia, nausea, and vomiting since the age of 4. He had his first episode of migraine associated with ocular pain and ptosis in the right eyelid, lasting 24 hours, in Jun 2010 at the age of 6. Six months later (Jan 2011), he had a heavier and persistent episode of migraine associated with right ocular pain , right ptosis, and diplopia, so he was admitted to the Child Neuropsychiatry Unit, Department of Basic Medical Sciences, Neuroscience and Sense Organs, University of Bari “Aldo Moro,” Italy. Family history of migraine in both parents, multiple sclerosis in the father's family, and gastric neoplastic disease in the mother's family was referred. General physical examination was normal; neurologic examination showed paresis of the third and sixth cranial nerves. Laboratory investigation, including virological and organ-specific serum antibodies, and instrumental examination including awake/sleep electroencephalograph produced normal results. Brain and cervical-spine magnetic resonance imaging (MRI) enhanced after contrast administration and magnetic resonance angiography (MRA) produced normal findings. Diagnosis of “ophthalmoplegic migraine” was made according to ICHD 2, as discussed in a previous publication . At the To our knowledge, this is the first case reported in the literature of an adolescent suffering from headache and ophthalmoplegia with a 7-year brain MRI follow-up, raising the question on the relationship between RPON and schwannoma. The evolutive changes in the presentation of symptoms and the different findings over time in brain MRI images observed in this patient repurpose some open issues on the classification and pathophysiological mechanisms of RPON. The patient had his first clinical manifestations of recurrent headaches at the age of 4 years agreeing to diagnostic criteria of migraine without aura. Since he was 7 years old, the patient started to have migraine episodes with the same clinical features but lasting more time and with partial responsiveness to administration of paracetamol with association of right ocular pain, ipsilateral ptosis, and diplopia. After two episodes with these features and with MRI that showed normal findings in the image scans without any appreciable enhancement or thickening of the nerve, he was diagnosed with “ophthalmoplegic migraine” according to ICHD-II . Brain MIn conclusion, both case reports we presented and the literature review of other pediatric cases of OM/RPON, suggest an existing relationship between RPON and schwannoma. Further longitudinal studies on a larger number of cases are needed to clarify whether in some patients an ocular motor nerve schwannoma may closely mimic RPON or, alternatively, if the RPON may be considered a risk factor to develop over time a schwannoma. Anyway, in both these situations, negative findings at MRI during an acute attack of RPON (without any thickening or gadolinium enhancement) do not rule out the possibility of a future evidence of the tumor, considering that schwannomas are generally slowly progressive. Serial brain MRIs during acute exacerbations and even between attacks are essential in the long-term follow-up of RPON, especially in the cases with childhood onset, in order to not delay the diagnosis of a possible oculomotor nerve schwannoma.


Appendiceal metastasis from lung cancer is rare. However, it often causes acute appendicitis that requires emergency surgery. We herein report a thought-provoking case of appendiceal metastasis from lung cancer.A 71-year-old man was diagnosed with advanced lung cancer with multiple metastases and underwent chemotherapy. One month later, he developed acute appendicitis, and laparoscopic appendectomy was promptly performed. A swollen appendix and pus collection were observed during surgery. Histological analysis revealed an invasive adenocarcinoma in the appendix that infiltrated the mucosal, submucosal, and muscular layers. Positive immunostaining of thyroid transcription factor 1 indicated appendiceal metastasis of pulmonary adenocarcinoma, not a primary appendiceal malignancy. The postoperative course was uneventful, and the patient’s pulmonary internist resumed continuous chemotherapy after surgery.Although appendiceal metastasis from pulmonary adenocarcinoma is rare, it often results in acute appendicitis. Optimal therapy including emergency surgery should be performed without hesitation so that chemotherapy can be resumed as soon as possible. Lung cancer (LC) is one of the most common cancers worldwide and the leading cause of death in Japan. Metastasis occurs in half of LCs, and the most common sites are the lymph nodes, adrenal glands, liver, bone, and brain . UnusualA 71-year-old man was referred to our hospital from his primary physician because of suspected LC. Computed tomography (CT) revealed a primary tumor in the right middle lobe and metastases in the lymph nodes , brain, both adrenal glands, and bones . Laparoscopic survey of the abdominal cavity revealed no additional metastases .This patient received chemotherapy with carboplatin, paclitaxel, and bevacizumab. One month later, he presented with right lower quadrant pain when he visited our hospital to receive his scheduled chemotherapy. His serum level of C-reactive protein was clearly increased at 11.67 mg/dL, although his white blood cell count was within the normal range. Enhanced CT findings showed an enlarged appendix and fluid collection near the distal appendix Fig. . A diagn3Histological analysis by hematoxylin eosin staining revealed invasive adenocarcinoma in the appendix that infiltrated the mucosal, submucosal, and muscular layers. Positive immunostaining of TTF-1 indicated that the appendiceal metastasis was from pulmonary adenocarcinoma, not a primary appendiceal malignancy Fig. .Fig. 4PaThe postoperative course was uneventful, and the patient was discharged on postoperative day 7. The patient’s pulmonary internist resumed continuous chemotherapy after surgery.AA is a common disease worldwide, and primary appendiceal cancer often causes AA. However, secondary AA caused by appendiceal metastasis from LC is rare; the reported frequency of appendiceal metastasis of LC is 0.0005% . With reIn our case, it was difficult to distinguish metastatic pulmonary adenocarcinoma from primary appendix cancer based only on the pathological findings of conventional hematoxylin and eosin staining. TTF-1 plays an important role in the immunopathological diagnosis of lung and thyroid cancers. Positive immunostaining of TTF-1 strongly indicates lung or thyroid cancer. In contrast, gastrointestinal and colorectal cancers generally show negative immunostaining of TTF-1, and only 2.5% of colon cancers are reportedly positive for TTF-1 . TherefoAlthough enhanced CT has an advantage in detecting metastatic masses in the appendix , many pa