Simple mucinous cyst (SMC) of pancreas is a disease defined by the Baltimore Consensus in 2014. Pancreatic mucus-producing neoplasms are considered to be premalignant tumors, but SMC is not considered to have a risk of malignancy or recurrence.The case was a woman in her 50s with a chief complaint of abdominal pain. A blood exam showed an increase in the inflammatory response, and a slight increase of Amylase. CT showed a cystic lesion 80 mm in size at tail of the pancreas, and disproportionate fat stranding and ascites around it. We diagnosed peritonitis associated with the rupture of a cystic lesion accompanied by pancreatitis. Abdominal pain was improving, and we decided to proceed with the detailed examination. MRI showed a uniform hyper-intensity on T2WI, and a nodular-like hypo-intensity was observed inside, which was enhanced. During the follow-up, the lesion had gradually grown and re-ruptured. As we could not deny malignancy by image findings, distal pancreatectomy was performed. The intracystic fluid was browny and turbid, and Amylase, CEA and CA19-9 of the cystic fluid were elevated. We diagnosed it SMC by histopathological findings. Currently, she had no recurrence for 1 year.SMC is a type of true cysts, so rupture was rare. However, if the cyst wall becomes weak due to complications such as acute pancreatitis. It is probable that our case had pancreatitis and the cyst wall was weakened.SMC detected by rupture was very rare, so we report this case. •SMC of the pancreas in which surgery was required due to rupture was rare case.•This is unique that it looked like an nodule due to the gathering of cystic walls.•Non-neoplastic lesions should be considered if nodule grow and shrink over time. Abdominal computed tomography (CT) was performed and revealed a pancreatic cystic lesion. So, she was referred to our hospital for detail examination of pancreatic cystic lesion. On physical examination, there were tenderness and spontaneous pain on her entire abdomen. She had been treated for acute pancreatitis twice and didn't have any medical and family history. Blood examination showed increasing inflammatory response, WBC 10180/μl and CRP 23.6 mg/dl, and tumor markers, CEA, CA19-9 and CA125, were not elevated.The contrast-enhanced CT revealed a unilocular cystic lesion in pancreatic tail sized in 80 mm which had partial thicken wall. There were disproportionate fat stranding around cystic lesion , and ascThe operation was performed by specialists of pancreatic resection who had been in practice for over 10 years. In operative findings, there were adhesions due to repeated ruptures around the cyst lesion. The cystic lesion surface was smooth, and cysts contained fluid. We punctured the intra-cystic fluid and collected a brownish fluid. The biochemical examination of the intracystic fluid was Amylase 27,000 U/l, carcinoembryonic antigen 280 ng/dl, and carbohydrate antigen 7900 U/ml by biochemical examination. The operation time was 4 h 19 min, and the blood loss was 1400 ml.The pathological findings revealed that there were no nodules, and mucinous cells covered inside the cyst. Immunostaining was performed, but no ovarian type stroma characteristic of MCN was found. The cells were poorly atypical and no neoplastic growth was observed, and granulation tissue was growing in the stroma . FinallyThe course after the operation was uneventful. She discharged after 32 days after surgery. She had lived for a year after surgery without any evidence of metastasis and recurrence.3Two German pathologists, Kosmahl and Klöppel, and their colleagues in 2002 were the first to describe this type of mucinous cyst in the English-language literature SMC has been reported as 2.1– 3.4% in resected pancreatic lesions Although mucinous pancreatic cystic lesions are generally considered to be precursor lesions of pancreatic adenocarcinoma owing to their common association or coexistence with cancer In this case, we couldn't deny malignant potential because preoperative image findings showed the cystic lesion had solid components with blood flow. But histopathological findings revealed no nodule inside the cyst. When viewed retrospectively, nodular components were found inside the cyst at the first rupture, but the larger the cyst grew, the smaller the nodular components became in the follow-up CT. So, it seems that the cyst wall was gathered and looked like a nodule due to the shrinkage of the cyst due to the rupture. Intracystic nodules generally mean to be malignant in cystic pancreatic lesions, but if the nodules grow and shrink over time, it is necessary to consider the possibility like this case.Past report showed that analysis of cystic fluid may be useful in benign and malignant cysts. CEA level, CA19-9 level and K-RAS mutation are more frequently observed premalignant cystic lesion 4We experienced a rare and unique case of ruptured SMC which cyst wall was gathered and looked like a intracystic nodule due to the shrinkage of the cyst. Since there is no long-term observational study of SMC and it has one aspect of neoplastic lesions, follow-up is considered necessary in the future.CTComputed tomographyIPMNIntraductal papillary mucinous neoplasmMCNMucinous cystic neoplasmMRIMagnetic resonance imagingT1WIT1 weighted imageT2WIT2 weighted imageDWIdiffused weighted imageThis study of case report is exempt from ethnical approval in ethics committee of our institution.None.Clinical treatment: Naoko Sekiguchi, Shinsuke Nakashima, Masahiro Koh, Masami Ueda, Yujiro Tsuda, Tsukasa Tanida, Jin Matsuyama, Masakazu Ikenaga and Terumasa YamadaCollected data: Naoko Sekiguchi, Shinsuke NakashimaAssessment and discussion: Naoko Sekiguchi, Shinsuke Nakashima and Terumasa YamadaWrote the paper: Naoko Sekiguchi, Shinsuke Nakashima and Terumasa YamadaCorresponding author, Terumasa YamadaNone.Informed consent has been obtained from the patient and all identifying details have been omitted. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer-reviewed.None.


Lymphangioma is a rare benign neoplasm affecting mainly children. In this report we present a complicated case of polycystic omental lymphangioma in a 3 year old female presenting with acute abdomen. The patient underwent a laparoscopic-assisted excision of the lesion and had an excellent postoperative course. We discuss the effectiveness and advantages of this laparoscopic surgical approach in children and elaborate on the current literature. Lymphangiomas are rare congenital malformations of the lymphatic system, both of childhood and adulthood, with a prevalence of around 1, 1: 10,000 to 5, 3: 10,000 live births . Namely Most cases of lymphangioma are asymptomatic. However, probably owing to a smaller abdominal cavity, children more than often develop symptoms . Little A 3 years old female was transferred to our Emergency Department due to acute abdominal pain, for further investigation and potential surgical management, after a brief hospitalization in the Pediatric Department of a peripheral hospital. The child suffered from a gradually intensifying abdominal pain which began two days before presentation, accompanied by low grade fever appearing later during the course of the illness. On arrival, vital signs were stable, within normal limits and the clinical examination revealed tenderness to palpation, predominantly in the right lower quadrant, and a distended abdomen with abdominal guarding suggestive of peritonitis due to appendiceal perforation. According to the history of the present illness no other complaints indicative of gastrointestinal discomfort  were reported.3 with high levels of neutrophils. In this context a computed tomography (CT) scan of the abdomen was performed that demonstrated a giant multilocular cystic lesion occupying the entire abdominal cavity laterally, dislocating proximally the intestinal loops, measuring up to 20cm in length and 15cm in width  capillary; 2) cavernous and 3) cystic, which is also known as cystic hygroma . Abdominet al. [Omental lymphangiomas are rare abdominal malformations that due to the capacity of the abdominal cavity to expand do not develop symptoms early, unless complications arise . Ultrasoet al. . The samet al. .et al. in 2017, was managed the same way with equivalent results and no recurrence after five years [Surgical management consists usually of an open surgical exploration and excision of the lesion, with laparoscopic management gaining popularity over the years. According to two case series studies in children , 9, compve years .Lymphangiomas generally are known to have high recurrence rates after excision. In a case series study, a postoperative recurrence rate of 10% was noted, probably due to incomplete resection , while lOmental lymphangiomas indeed represent a rare entity with equivocal clinical presentation. Therefore, the clinical suspicion of a pediatric surgeon has to be increased, especially when dealing with abdominal differential diagnostic approach. Regarding the efficacy of laparoscopy-assisted excision, we suggest that it represents an adequate and safe approach for the management of such lesions, providing that the possibility of complete resection is not compromised and that the procedure is carried out by experienced laparoscopy surgeons.


Aggressive angiomyxoma (AAM) was identified as a distinct clinicopathological entity in 1983. Since then, a few cases of its occurrence in the scrotum have been reported. This case series was performed to increase clinicians' understanding of the clinical features and treatment of AAM in the scrotum.We evaluated the clinical presentations, treatments, and follow-up of two patients with AAM in the scrotum in our hospital and 34 cases reported in the literature.Among the 36 patients, the average age was 48.3 ± 20.6 years old (range from 1 to 81); the average maximum diameter of the tumor was 8.36 cm (1.6–25 cm); the site of one (2.78%) patient was located in the epididymis, two (5.56%) in the testes, five (13.89%) in the spermatic cord, and 28 (77.77%) in the scrotum. The clinical symptoms were generally non-specific and 20 patients inadvertently discovered their slow-growing painless masses. The treatments for all these patients were surgical excision once the tumor had been found and one case underwent excision followed by radiotherapy. The median follow-up time for the remaining 32 cases was 24.5 months (1 to 84 months). Recurrence occurred in three cases (9.09%) at the primary sites and no cases of distant metastasis.AAM of the scrotum can occur in middle-aged and elderly men. The clinical manifestation generally involves a long history of asymptomatic masses or swelling in the scrotum. Ultrasound is the most commonly used diagnostic technique but magnetic resonance imaging may be more effective. The mainly treatment is surgical excision and postoperative histopathological examination is still the gold standard for its diagnosis. Although it is locally aggressive, metastasis is extremely rare in males. Aggressive angiomyxoma (AAM) is a rare benign mesenchymal myxoid tumor that usually occurs in the pelvic soft tissues and perineum of women in the reproductive age . It was At present, AAM-related literature mainly consists of studies about women and a few case reports in men, lacking systematic analysis and summary of a certain number of cases in terms of AAM in the scrotum. To help recognize AAM occurred in scrotum better and reduce the rate of misdiagnosis, we herein describe two cases of it in Peking Union Medical College Hospital (PUMCH) and further review 34 cases in literature provides a retrospective analysis of their clinical manifestations, diagnosis, and treatments of this condition.Between January 1983 and December 2020, a total of 2 patients with AAM in the scrotum were diagnosed and treated at the PUMCH. The medical histories and preoperative auxiliary examinations were taken from medical records, including tumor marker test, US and MRI. Histopathological and immunohistochemical characteristics were also reviewed. The two cases underwent individualized surgical treatment and were diagnosed by postoperative paraffin pathological examination.PubMed and Geen Medical databases were searched for clinical case reports published from 1992 to 2020 using the keywords of aggressive angiomyxoma and scrotum. The included cases had full clinical data and clear pathological diagnosis. Finally, 34 cases met the inclusion criteria, including the two cases in our hospital, there were 36 cases included in total.Observed indexes: The ages, localization and sizes of the tumors, symptoms and duration, therapies and follow-up were documented and retrospectively analyzed.Case 1 was a 45-year-old male who was admitted to our hospital with a one-year history of a growing left scrotal mass. Physical examination on arrival at our hospital revealed a circular lump of about 1.5 cm in diameter palpated on the left side of the scrotum with no pain but mild tenderness. The mass had a clear boundary, moderate mobility, and normal overlying skin. The light transmittance test was negative, and the remaining physical examination and laboratory test results, including routine blood examination, liver and kidney function tests, blood coagulation parameters, and tumor markers , were unremarkable. The US of the scrotum showed that a 1.6 × 1.2 × 1.0 cm area of clear boundary and low echo could be seen on the left spermatic cord with a meager blood flow signal on color Doppler flow imaging (CDFI). On suspected lipoma of the spermatic cord, the patient underwent a tumor resection on his fifth day in the hospital. Intraoperatively, the mass was found to be approximately 1.2 × 1.0 cm  and besiCase 2 was a 64-year-old man who was admitted to our urology department on March 20, 2015 with a seven-month history of a right scrotal mass accompanied by obvious plummeting. Physical examination on arrival at our hospital revealed an enlarged right scrotum, nearly 10 cm in diameter with no pain or tenderness, and the scrotum did not disappear while lying flat. The light transmittance test was negative, and the other physical examination and laboratory test results were all unremarkable. The US of the scrotum revealed that a gelatinous uneven echo could be seen at the right spermatic cord and the upper part of the scrotum, and CDFI showed negligible blood flow signal. MRI showed an elliptical long T1 and long T2 signal mass in the right scrotum, with a clear boundary and size of about 9.6 × 6.9 × 6.2 cm, which presented as an iso-height signal on the diffusion weighted imaging sequence and small spots of enhancement on the enhanced scan . FinallyAmong these 36 patients, the average age was 48.3 ± 20.6 years old (range from 1 to 81) when AAM was diagnosed, and detailly five patients (13.89%) were <20 years old, 3 (8.33%) were 8 20–40 years old, 24 (66.67%) were 40–70 years old, and 4 (11.11%) 9 were >70 years old. With respect to the location of the tumor, one (2.78%) was in the epididymis, two (5.56%) in the testes, five (13.89%) in the spermatic cord, and the remaining 28 (77.77%) were reported in the scrotum. The average maximum diameter of the tumor was 8.36, while the minimum was 1.6, the maximum was 25, and 10 patients had tumors larger than 10 cm in size (28.6%) .Patients' clinical symptoms were generally non-specific. There were 20 patients who inadvertently discovered their slow-growing painless masses. The remaining patients experienced clinical symptoms such as swelling in the scrotum, hydrocele, inguinal hernia, lymphedema, and so on. And the range time from tumor discovery to treatment was 1 months to 20 years.In our report, all of the 36 cases underwent surgical excision once the tumor had been found and one of them underwent excision followed by radiotherapy. For 27 cases, local surgical excision was performed to remove the tumors while wide surgical excision was done for four cases. In one case, the patient operated with complete resection of the scrotal skin because of monstrous scrotal lymphedema. And radical orchiectomy was done in the rest of four cases.Of these 36 cases, three patients were lost to follow-up and one case showed no interval for NED (no evidence of disease). The median follow-up time for the remaining 32 cases was 24.5 months (1 to 84 months). Recurrence occurred in three cases (9.09%), indicated by re-check of imaging examination. And the recurrence in all three patients was found at the primary sites and there were no cases of distant metastasis.AAM or deep angiomyxoma is a rare and special type of soft tissue tumor that is recognized as a mesenchymal malignant tumor by the World Health Organization and was first described by Steeper and Rosai in 1983 . AggressFor the etiology of AAM, Kenny et al. found that the formation of new organisms in the lesion site of AAM may be associated with the loss of the X chromosome . Some auMost patients with AAM present with a long history of asymptomatic masses or swelling in the scrotum. In our 36 cases, the size of the masses ranged from 1.6 to 25 cm and the history ranged from 1 month to 20 years. The private location and painless symptoms led to delayed medical treatment, resulting in a gradually increased tumor size and high clinical misdiagnosis rate. Clear preoperative diagnosis is difficult, and differential diagnosis includes non-neoplastic causes, such as inguinal hernia, edema, testicular, paratesticular tumors . It alsoIn terms of the preoperative diagnosis of AAM in the scrotum, the currently used auxiliary examinations mainly include US, CT, and MRI . AAM lesOn gross evaluation, AAM is usually a partially limited, uneven, lobulated, soft, and grayish-white tumor with a smooth contour and colloid appearance. Histologically, nearly all AAMs are identical, and characterized by prominent vascular components and invasive growth. They are composed of thick- or thin-walled vessels of varying sizes, and the tumor cells are evenly distributed in the stroma-containing mucus, showing as uniform spindled, star shaped, or oval shaped. Further, bland nuclei and sparse mitoses are its cytological characteristics, and nuclei with morphological changes such as abnormal shape and nuclear division are rare . So therImmunohistochemically, AAM is positive for Desmin, Vimentin, SMA (smooth muscle actin) and CD34, and negative for S-100, CK and CD68 . Among tThe differential diagnosis of AAM with other tumors mainly contains angiomyofibroblastoma , myxoid neurofibroma (commonly occurs in extremities and is strongly positive for S-100), myxoma , myxofibrosarcoma , myxoid liposarcoma  . Other dThe mainstay treatment of AAM in the scrotum is wide local excision with tumor-free surgical margins . HoweverThe risk of local recurrence of AAM is high (35 to 72%), especially within 2–3 years after the first operation . ApproxiAs for those AAM patients of local recurrence, radiotherapy has been proposed as a control method for multiple recurrence after surgical resection, but the effect is not satisfactory; and chemotherapy is often ineffective because of low mitotic activity of AAM . VasculaIn conclusion, AAM of the scrotum can occur in middle-aged and elderly men. The clinical manifestation generally involves a long history of asymptomatic masses or swelling in the scrotum. US is the most commonly used diagnostic technique preoperatively but MRI may be more effective. The mainly treatment is surgical excision and postoperative histopathological examination is still the gold standard for its diagnosis. Although it is locally aggressive, metastasis is extremely rare in males.The original contributions presented in the study are included in the article, further inquiries can be directed to the corresponding author/s.JS and PL were responsible for collecting, sorting out data and writing articles, were co-first authors. ZY, DD, and ZJ were responsible for collecting data. JW and HL were responsible for putting forward ideas and reviewing articles, were co-corresponding authors of this paper.Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences (2019XK320027) and Project Management Fund for Foreign Cultural and Educational experts (G20190001645).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


The visual pathway and its defects have been thoroughly studied in clinical correlation to temporal lobe lesions related to epilepsy and traumatic lesions. Nevertheless, its clinical correlation and other decision-making have not been addressed regarding neoplastic lesions. We present a case report of a 28-year-old man with a one-year history of generalized seizures and left superior homonymous quadrantanopia, with no other neurological disturbance on physical examination. According to diffusion tensor imaging tractography, MRI demonstrated a non-enhancing, right temporal lesion disrupting the visual pathway. An awake surgery with direct cortical electrostimulation of visual pathways was performed with subtotal resection of the tumor to preserve visual function, confirmed with postoperative MRI. Histopathological studies revealed a fibrillary astrocytoma.Surgical technique aided with intraoperative cortical and subcortical stimulation involving low-grade gliomas in eloquent areas is an exceptionally suitable procedure for complex cases where the visual pathway is compromised. Our objective is to describe how intraoperative mapping of visual function is performed in our institution and to comment on the relevant technical nuances, which can serve as a practical guideline for young neurosurgeons, as no previous cases have been reported in our country. In 1907, Dr. Adolf Meyer, a Swiss psychiatrist, first described “the peculiar detour of the ventral portion of the geniculocalcarine pathway.” He concluded that a portion of the optic radiation, after leaving the geniculate body, plunged anteriorly into the temporal lobe through the tip of the horn of the ventricle before turning back towards the calcarine cortex . Since tGliomas are the most common primary malignant brain tumor among adults, with an incidence reported from 4.67 to 5.73 per 100,000 people [A 28-year-old man presented with a one-year history of generalized seizures to our institution; at admission, the general and neurological evaluation revealed no disturbances. Contrast-enhanced MRI demonstrated a non-enhancing, right temporal lesion, measuring 52 mm x 50 mm x 56 mm, and diffusion tensor imaging (DTI) tractography showed tumoral visual pathway invasion, affecting the right parietal portion, which carries information from the inferior quadrant of both retinas Figure -D. NeuroThe situation was thoroughly discussed with the patient and his relatives, as complete resection of the tumor represented a high risk of an added visual field defect, in this case, a complete homonymous right hemianopia. Due to the situation's complexity and the consequences, it could bring to the patient's quality of life, the decision to respect functional areas was taken. We decided to perform an awake surgery with direct cortical electrostimulation of visual pathways. The patient was instructed to inform us whenever he detected an alteration in his visual field using an evaluation chart Figure .A conventional pterional approach and a three-phase asleep-awake-asleep anesthetic technique for intraoperative mapping were performed. Total intravenous general anesthesia (TIVA) with fentanyl, propofol, dexmedetomidine, and a single dose of rocuronium, was used for induction. A laryngeal mask was placed for airway management. Subsequently, anesthetic maintenance was performed with propofol, fentanyl, and dexmedetomidine in the first asleep phase. An intrinsic lesion affecting the middle and inferior temporal gyri was found, with a rostral displacement of the superior temporal gyrus. The vein of Labbé was used as the caudal limit for the resection, which was medially directed, and the patient was awakened. The patient's emersion was initiated through metabolic lysis of the drugs (fentanyl and propofol), spontaneous ventilation, aspiration of secretions, removal of the laryngeal mask, and support ventilation with nasal cannulas. During visual tests, anesthesia maintenance was carried out with low-dose dexmedetomidine in the second awake phase. We mapped the optic radiations all the way to the lateral wall of the temporal horn of the lateral ventricle, preserving these functional locations Figure ,B. We usThe patient´s postoperative course was uneventful. Postoperative MRI was performed, and it evidenced a subtotal resection with minimal residual tumor, which was left behind to spare visual function Figure . AlthougEarly surgery is currently the treatment of choice when facing a patient with low-grade glioma. The extent of resection (EOR) has been reported as one of the most significant predictors of overall survival . Smith eConsequently, in the past two decades, awake patient mapping surgery has shown to be a valuable tool to optimize resection rates in eloquent areas and minimize neurological deficit while improving postoperative functionality and quality of life . As in oVisual field defects account for necessary functionality and quality of life outcomes in the postoperative course. Defects such as quadrantanopia may be acceptable, as it is usually asymptomatic, and in most countries, it is not a contraindication for driving . On the Functional neuro-oncology, preserving functions and quality of life, has become an essential aspect of glioma surgery. Although the EOR is well known to be one of the most important factors for overall survival, as a surgeon, one must outweigh a reduction in progression-free and overall survival versus a completely functional patient. Functional neuro-oncology proves the essential role of using awake surgery techniques aided with intraoperative cortical and subcortical stimulation in low-grade gliomas involving eloquent areas.


When performing pancreaticoduodenectomy with resection of the confluence of the superior mesenteric vein and portal vein, division of the splenic vein may cause sinistral portal hypertension resulting in gastrointestinal bleeding, splenic congestion, and hypersplenism. To prevent these adverse events, it is important to intentionally decompress the splenic vein. This report is of a 68-year-old woman with stage IA carcinoma of the head of the pancreas who survived for more than six years following tumor resection and pancreaticoduodenectomy and distal splenorenal shunt. A 68-year-old woman was diagnosed with carcinoma of the head of the pancreas that involved the confluence of the superior mesenteric vein, portal vein, and splenic vein. No unresectable cancer sites or distant metastases were detected. Pancreaticoduodenectomy with resection of the confluence of the superior mesenteric vein and portal vein was performed. The superior mesenteric vein and portal vein were anastomosed in the end-to-end fashion, and the remnant splenic vein was anastomosed to the superior aspect of the left renal vein in the end-to-side fashion. At 22 months after the initial surgery, the patient underwent partial lung resection for a metachronous lung metastasis. For 6 years after the initial surgery, the venous reconstructions have maintained their patency without any obstruction of splenic venous flow, and the patient has remained in good health without further metastases or recurrences. This case has shown the importance of early diagnosis of carcinoma of the head of the pancreas, as appropriate and timely surgical management can result in good outcome. This patient responded well and remains alive six years following pancreaticoduodenectomy and preservation of the spleen with the use of a distal splenorenal shunt. Pancreas cancer is the fourth leading cause of cancer-related death in developed countries , and thePancreatic cancer located in the pancreatic head and/or uncus easily invades the superior mesenteric vein and/or portal vein –11. AlthW. Dean Warren (1924-1989) was a pioneer in surgery for portal hypertension , and he Division and ligation of the splenic vein during pancreaticoduodenectomy is often associated with the development of sinistral portal hypertension, which results in gastrointestinal bleeding, splenic congestion, and hypersplenism over the long term , 13. TheThis report describes a 68-year-old woman with stage IA carcinoma of the head of the pancreas who survived for more than six years following tumor resection and pancreaticoduodenectomy with the use of a distal splenorenal shunt to preserve the spleen.A 68-year-old woman was referred to our hospital for the investigation of elevated serum levels of amylase and lipase. Enhanced computed tomography (CT) detected a hypovascular mass (18 mm diameter) in the pancreatic head Figures . The maien bloc resection of the confluence of these veins was performed, with simultaneous venous reconstructions  was graphically detected in the right lung. The patient underwent partial lung resection for a right lung tumor that was pathologically diagnosed as a lung metastasis originated from the pancreatic cancer , although graphical and surgical curability were obtained. Chemotherapy with S-1 was resumed for 1 year after the lung resection. The patient has been in good health, with no other oncologic metastases detected during the 6 years since the initial surgery.μL and 235,000/μL, respectively. The estimated splenic volumes before surgery and at 5 years and 6 months after the initial surgery were 38 mL and 35 mL, respectively.Dynamic CT and upper esophagogastric endoscopy performed 5 years and 6 months after the initial surgery revealed that all venous reconstructions had maintained good patency , and theIntentional decompression of the splenic vein after division of this vein is still controversial –20, and Although venous reconstruction of the superior mesenteric vein and portal vein during pancreaticoduodenectomy for pancreatic cancer is widely accepted, intentional decompression of the splenic vein in case when division of the splenic vein is needed is still controversial –20. It hAs pancreatic cancer generally has a very poor prognosis –6, many A native vein may be the optimal candidate as a drainage route of the splenic vein. The inferior mesenteric vein is useful as a drainage route from the splenic vein , 34, 35,Most surgeons currently believe that the splenic venous flow must be reconstructed in cases where the splenic vein is divided –20. AlthWarren was a pioneer in surgery for portal hypertension and variceal bleeding and originally published the distal splenorenal shunt in 1967 as an operative procedure for bleeding esophageal varices . Warren To our knowledge, there are only few reported cases of splenic venous reconstruction via a distal splenorenal shunt , 37–39. In conclusion, this case has shown the importance of early diagnosis of carcinoma of the head of the pancreas, as appropriate and timely surgical management can result in good outcome. This patient responded well and remains alive six years following pancreaticoduodenectomy and preservation of the spleen with the use of a distal splenorenal shunt.


Primary soft tissue sarcomas contribute to only 2% of all malignancies arising from the male genitourinary tract. Leiomyosarcoma (LMS) is a malignant soft tissue neoplasm which originates from the mesenchyme and has a characteristic smooth muscle differentiation. Usually, it presents as a painless, firm, slow-growing unilateral scrotal mass. Investigations include imaging, tumor markers, and histopathology.A 65-year-old gentleman known diabetic and beta-thalassemic trait was referred to the Urology OPD at Letterkenny University Hospital. His presenting complaint was a left groin lump that appeared 1 year ago and was growing larger in size gradually. According to the patient, his lump was slightly painful  initially that later became painless. He did not report any testicular trauma/infection or UTI. There was no significant history of malignancies running through his family. Clinical examination revealed a soft and lax abdomen, normal testes. There was a non-tender 2cm x 2cm well-circumscribed, mobile, firm to cystic irreducible left inguinoscrotal mass and appeared to be attached to the spermatic cord. Cough impulse was indiscernible. Ultrasound left groin showed 1.8 cm transverse x 1.4 cm AP x 1.9 cm sagittal) well-circumscribed ovoid nodular subcutaneous lesion present in the upper left inguinal area just lateral to the left pubic tubercle that appeared solid with heterogeneous internal echotexture and no internal calcification. Some internal vascularity is demonstrated with color Doppler assessment.Because of its rareness, LMS represents a management conundrum. There is no standard protocol for treatment. We present a case and discuss the available evidence from the literature to date to help identify LMS of the spermatic cord that is highly unusual. Masses in the scrotal sac in adults can be either testicular or paratesticular. Paratesticular tumors are uncommon and account for < 5% of scrotal masses. The paratesticular area can give rise to a vast range of neoplasms with a wide behavioral spectrum due to its complex anatomy which houses a number of structures, including the epididymis, tunica vaginalis, and spermatic cord .Scrotal leiomyosarcomas (LMS) are rare. Approximately 30% are malignant and of those almost 90% are sarcomas . PrimaryThe pre-operative diagnosis of LMS is difficult. Since most arise from the distal spermatic cord, distinguishing them clinically from a scrotal mass is challenging. LMS can be mistaken as epididymal cyst, cord lipoma, incarcerated hernia, inguinal hernia, and epididymo-orchitis. The clinical presentation is usually vague and non-typical. It can range from painless to mildly painful paratesticular intrascrotal mass with or without a feeling of heaviness . The peaA 65-year-old man with type-2 diabetes and a beta-thalassaemic trait was referred to urology presenting with a left groin lump. This had appeared about 1 year ago and gradually increased in size. Initially, the lump was slightly painful but later became painless.There was no history of any testicular trauma, sexually transmitted diseases, UTI, undescended testis, or groin/scrotal surgery of any kind. There was no family history of malignancies.Clinical examination revealed a soft abdomen and normal testes. There was a non-tender, 2x2cm well-circumscribed, mobile, firm left inguinoscrotal mass which appeared to be attached to the spermatic cord.Ultrasound of the left groin Fig.  showed aThree-phasic contrast CT Fig.  showed tLaboratory parameters were all within normal limits. Testicular tumor markers such as beta-HCG, alpha-fetoprotein (AFP), and lactate dehydrogenase (LDH) were in the normal range.The patient underwent a biopsy excision of the lesion. Histopathology showed spindle-shaped, elongated, moderately atypical cells in fascicular arrangement Fig. a with ovTumor differentiation: 2Mitotic count: 2Tumor necrosis: 0The French Federation of Cancer Centres Grading System for sarcomas was employed to further classify the specimen [5]. Each of its domains is mentioned below, along with their respective scores:With an overall score of 4, our specimen was suggestive of a Grade 2 LMS of the spermatic cord.Consequently, the patient underwent left orchiectomy with excision of the spermatic cord up to the deep inguinal ring via an inguinal open approach.The postoperative course was uneventful. The final histopathology of the orchiectomy specimen showed that the LMS had earlier been resected completely. Follow-up surveillance with the CT chest, abdomen, and pelvis did not reveal any recurrence, lymph nodes, or distant metastasis. Twelve months of follow-up did not reveal any recurrence. The patient was satisfied with the treatment given to him. Written informed consent was obtained from the patient for publication of this case report.The first paratesticular sarcoma was reported in 1996 . LMS is LMS exhibits a wide-ranging clinical presentation. Clinically, any painless slow-growing lump should be considered suspicious for LMS amongst other differential diagnoses . It is iAs for any unclear suspicious testicular or para-testicular lesion, investigations for LMS should include initial ultrasonography, CT, testicular tumor markers, histopathology, and immunohistochemical staining.Ultrasonography has excellent sensitivity and specificity to assess any lesion in the scrotum. It is easy accessibility, and safe application makes it the initial exam of choice . UltrasoThe definite diagnosis relies on histopathology and immunohistochemistry . The graBecause of its rarity, there is no consensus on the treatment for spermatic cord LMS . OrchideThere is no guideline on radiotherapy for LMS of the spermatic cord. Some authors advocate the use of adjuvant radiotherapy after orchidectomy to reduce local disease recurrence .Chemotherapy has no clearly defined role in the LMS of the spermatic cord. It is often employed in metastatic disease . A long In conclusion, LMS must be considered in the differential diagnosis of a slow-growing, unilateral, para-testicular mass in middle-aged men.Radical orchidectomy and high spermatic cord excision along with resection of surrounding soft tissues are required to achieve a negative margin. Lymph node dissection is only recommended in cases of enlarged lymph nodes. Chemotherapy and radiotherapy have a limited and as yet poorly defined role in specific cases only. And finally, a long follow-up is mandatory as late recurrences are possible.


Juvenile xanthogranuloma is a proliferative cutaneous manifestation encountered in the paediatric population. Adult cases are uncommon, but have been reported. Lesions are prevalent in the head and neck region, but rarely observed in the external auditory canal. We present the case of a 39-year-old female with a rapidly progressing obstructive soft tissue lesion of the external auditory canal. Surgical excision diagnosed the lesion as a rarely observed otological manifestation of juvenile xanthogranuloma. Surgical excision was curative with no locoregional recurrence. Otolaryngologists should consider juvenile xanthogranuloma as a differential for atypical soft tissue cutaneous lesions of the head and neck, including in divergent populations. Juvenile xanthogranuloma (JXG) is a proliferative cutaneous disorder of infancy most commonly encountered in the first year of life, although adult cases have been previously reported . LesionsA 39-year-old female was referred to our department with a red fleshy lesion occupying her left external auditory canal. The lesion developed insidiously and grew rapidly over the course of months. On clinical evaluation, a well circumscribed, epithelialized lesion was identified deep to the conchal bowl within the external auditory meatus. It was broadly pedicled to the antero-superior canal wall. The lesion was 8 mm in diameter with near complete occlusion of the canal . The texGiven the brisk progression and uncertain aetiology, surgical excision was undertaken. Local infiltration with xylocaine was used for anaesthesia and to aid hydro dissection. Sharp dissection of the broad base with a combination of scalpel and dissecting scissors provided en bloc resection of the lesion. When dividing the tanned, fatty contents from the underlying meatal cartilage, no clearly demarcated tissue plane or capsule could be identified. Haemostasis was achieved with bipolar diathermy and the defect was primarily closed with absorbable sutures. Histological assessment of the 8 × 9 × 6 mm lesion identified diffuse sheets of mononuclear and multinucleated histiocytes within the dermis. Eosinophilia, foamy cytoplasms and Touton cells were described. These findings were characteristic and diagnostic of JXG . There wJXG are an uncommon manifestation of non-Langerhans cell histiocytosis. The characteristic microscopic findings are abundant histiocytes, foam cells and Touton cells , 5. The Classic clinical morphology is the insidious development of red papules that can evolve into yellow, scaly nodules in a solitary or clustered configuration. Lesions typically plateau at 5 mm in size, but can progress beyond 20 mm. The natural history in children is to regress completely over months to years. JXG are overwhelmingly more common in the paediatric population with a greater incidence in Caucasians and males . The incJXG can occur at any cutaneous site, but the primary distribution is the skin of the head, neck and trunk. Atypical non-cutaneous lesions have been described in the oral cavity, central nervous system, gastrointestinal tract, lungs, bones and kidneys. Lesions occurring in the external auditory canal are incredibly rare. Previous case reports in the ear describe otorrhoea, bleeding and hearing loss , 3, 7, 8The swift growth and variable morphological features can mimic other benign and malignant pathologies of the external ear. Exostoses, osteoma, adenoma and ceruminoma share common physical traits with JXG . The sofThe majority of cases are treated expectantly; however, in an atypical site like the external ear, the potential for local complications coupled with diagnostic uncertainty justifies a more invasive approach. Surgical excision provides accurate diagnosis and symptom resolution. Following surgical resection, JXG rarely recur, irrespective of margins .JXG are a rarely encountered proliferative pathology of the external auditory canal. Although they are invariably benign, the unique anatomical location and spectrum of morphology evokes diagnostic ambiguity. Primary surgical excision provides definitive diagnosis, symptom alleviation and carries a low risk of recurrence. Otolaryngologists should include JXG in their cache of differentials when presented with a rapidly evolving soft tissue lesion of the external ear.


Pseudomyxoma peritonei (PMP) syndrome is a disease process that typically occurs from ruptured appendiceal mucocele neoplasms. PMP syndrome arising from malignant transformation of an ovarian primary mature cystic teratoma (MCT) is a pathogenesis rarely encountered.Herein, we report a 28-year-old patient evaluated and treated for a right ovarian mass and large volume symptomatic abdominopelvic mucinous ascites. Molecular profiling and genetic analysis revealed mutations in ATM, GNAS, and KRAS proteins while IHC demonstrated gastrointestinal-specific staining for CK20, CDX2, CK7, and SATB2. Peritoneal cytology showed paucicellular mucin. Diffuse peritoneal adenomucinosis (DPAM) variant of PMP arising from a ruptured ovarian primary MCT after malignant transformation to a low-grade appendiceal-like mucinous neoplasm was ultimately confirmed. Treatment included staged therapeutic tumor debulking and right salpingo-oophorectomy followed by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC).Our report builds upon the existing literature supporting this aggressive treatment option reserved for advanced abdominal malignancies utilized in this patient with a rare clinical entity. Pseudomyxoma peritonei (PMP) is a rare condition associated with ruptured mucinous neoplasms that typically originate from the appendix. PMP arising from malignant transformation of an ovarian teratoma is exceedingly rare with a reported incidence of 1-2/1,000,000 per year . If leftDefinitive treatment for PMP is complete gross resection and/or cytoreductive surgical (CRS) removal of all visible macroscopic tumor burden followed by hyperthermic intraperitoneal chemotherapy (HIPEC) , 6. CRS In patients with ovaries, determining the origin of PMP is challenging when a non-appendiceal source is suspected, implicating the possibility of a rare ovarian mucinous tumor etiology. Historically, Ronnett et al. classified PMP as being ovarian in origin if the appendix cannot be identified and/or if a rupture of a mucinous tumor of the appendix goes undetected .Herein, we describe a very rare occurrence of PMP syndrome arising from a ruptured low-grade appendiceal-like mucinous neoplasm that evolved from a transformed ovarian primary mature cystic teratoma (MCT) in a young patient that was definitively treated by cytoreductive surgery and regional intraperitoneal perfusion with hyperthermic chemotherapy.A 28-year-old female presented to the emergency department for a gynecological consultation after imaging performed at an outside hospital showed a large right ovarian mass concerning for malignancy. The patient’s presentation followed two months of upper abdominal pain aggravated by movement, coincident with weight loss, shortness of breath, abdominal distention and decreased appetite. Her ultrasound and CT scan showed an 18cm abdominopelvic mass originating from the right ovary which was cystic in appearance containing soft tissue, fatty and calcified components. Importantly, there was a large volume symptomatic mucinous appearing ascites  was employed. The peritoneal cancer index (PCI) was scored and calculated as described by Jacquet and Sugarbaker . CalculaPathology after complete macroscopic cytoreduction similarly demonstrated abundant pools of paucicellular mucin on the resected abdominopelvic disease-bearing visceral and parietal peritoneal surfaces. Importantly, the appendix was diagnosed as benign on permanent histology. Abundant pools of mucin demonstrated no additional or synchronous malignancy and were confirmatory for the DPAM-variant of PMP. Outpatient genetic counseling revealed no familial and/or genetic predisposition.The patient’s tumor was sent for molecular profiling and next-generation sequencing. The genetic report showed mutations in ATM at splice site 8268+1G>A, GNAS R201C, and KRAS G12D. The molecular profile was also consistent with tumor-associated microsatellite stability with a tumor mutational burden of 3Muts/Mb. Due to the low-grade nature of the tumor, systemic therapy was not recommended. The patient will undergo a biannual routine examination and cross-sectional imaging. Currently, there is no evidence of disease recurrence 1 year after the procedure.Mature cystic teratomas (MCTs) otherwise known as dermoid cysts are benign and frequently present as ovarian neoplasms. However, in 0.2–2% of cases, they can undergo malignant transformation, as described in this patient . The majPMP syndrome typically arises from perforation and/or rupture of an appendiceal mucocele or low-grade appendiceal mucocele neoplasms (LAMN) , 19. ExtThe decision to perform cytoreductive surgery is contingent upon disease biology, disease burden, and performance status of the patient. In general, limited abdominopelvic peritoneal metastasis or low peritoneal cancer index (PCI) with a low-grade tumor biology is a clinical presentation most amenable to this treatment option in healthy patients . Expert The HIPEC procedure is routinely carried out using a closed abdomen technique. After cytoreduction of tumor, inflow, and outflow drainage catheters connected to a perfusion pump are placed within the pelvis and upper abdomen, respectively. The patient’s skin is closed around the catheters with accompanying temperature probes. Regional perfusion of crystalloid containing heated chemotherapy at moderate hyperthermia temperatures (40–44 °C) is circulated and mechanically distributed throughout the abdomen and pelvis for a predetermined period of time in accord with the antineoplastic and chemotherapeutic properties of the agent(s) employed. At the conclusion, drainage catheters and chemotherapeutic perfusate are removed.Historically, the first patient treated with hyperthermic intraperitoneal chemotherapy was in 1979 for recurrent PMP, having previously undergone CRS. Subsequent phase I and phase II clinical trials in the 1980s demonstrated the effectiveness of intraperitoneal chemotherapy with improved survival in patients largely suffering from ovarian and appendiceal cancer. Currently, CRS and HIPEC are regarded as the standard of care for the treatment of PMP . Recentlrare “diagnosis of exclusion.” As demonstrated in this current case, it initially masqueraded as an advanced gynecologic malignancy but on subsequent pathologic review resembled the DPAM-variant of PMP arising from a ruptured appendiceal-like neoplasm originating from a MCT. Importantly from a therapeutic standpoint, complete cytoreduction and HIPEC for the DPAM-variant of PMP has a 10-year survival rate of 68% [Advanced abdominal malignancies, as in our patient, frequently present with significant tumor burden and upper abdominal, hepatobiliary involvement. There is little debate that suboptimal CRS offers no benefit to the patient. At our institution, cytoreductive surgery frequently includes extensive upper abdominal procedures involving consultant hepatobiliary, gastrointestinal  along with gynecologic surgical oncologists  to optimize the chance for complete macroscopic removal of tumor . As our e of 68% .Regardless of origin, PMP has a propensity to recur despite optimal therapy. Similar to traditional appendix-derived PMP, our patient’s tumor harbored non-targetable mutant KRAS-GNAS co-mutations with ATM mutant positivity . PresencThe present case represents an unusual occurrence and presentation of a rare clinical entity: a PMP syndrome arising in appendiceal-like tissue from a mature cystic teratoma. Discovery of a mature cystic teratoma with malignant transformations coincident with a PMP syndrome should promptly lead to an investigation into the malignancy status of the appendix. During surgery for removal of the mass and/or cytoreduction, the appendix should be removed and submitted for pathologic analysis. Notably, CRS and HIPEC are recommended treatments for appendiceal-derived PMP. However, the question will remain whether “proof of concept” performance of CRS and HIPEC will ultimately contribute to similar durable disease control with PMP-derived from a transformed MCT. Our case provides a clinical clue and suggests that it does. This report adds to the existing evidence supporting this aggressive treatment option in these unique patients. Limitations of this case report include the retrospective nature of this review, duration of follow-up, and requirement for more patients to validate this treatment strategy.


Echinococcus species, especially Echinococcus granulosus. This parasite can develop cysts in different organs of the human body.Echinococcosis is a zoonotic parasitic infection, caused by the larval stage of An osseous hydatid cyst is an uncommon and rare phenomenon. Here, we report a rare case of an osseous cyst in a 49-year-old woman with pain in the sacral region and a history of hydatidosis. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed for the patient. The chest CT scan and MRI results indicated masses in the pelvis. According to the patient's clinical signs, CT and MRI findings, and clinical history, osseous hydatidosis was established as the final diagnosis. Accordingly, surgical removal of cysts and chemotherapy were applied for treatment. The removed cysts were also sent for pathological examinations, which confirmed the diagnosis.Surgical removal is essential for the treatment of hydatidosis, and adjuvant chemotherapy is crucial for a better prognosis. Echinococcus granulosus, Osseous hydatid cyst, Pelvis, Surgery. Among species belonging to the genus Echinococcus, two species, namely, E. granulosus, as the causative agent of cystic echinococcosis (CE), and E. multilocularis, as the causative agent of alveolar echinococcosis, are of greater importance clinically [Hydatidosis is a zoonotic parasitic infection, caused by the larval stage of inically .E. granulosus can infect humans as accidental intermediate hosts. The infection is transmitted to humans by helminth eggs through close contact with infected dogs or contaminated fresh food products. After egg hatching, the larvae penetrate into the intestines and distribute in various organs through the blood or lymphatic system [The disease has a worldwide distribution. In the life-cycle of these heteroxenous parasites, dogs (and other canines) and ruminates are definitive and intermediate hosts, respectively. Besides, c system ; eventuaAlthough CE may develop in different organs, it mostly affects the liver and the lungs, respectively. In addition to mentioned organs, other can be affected, for example retroperitoneal cyst such as pancreatic cysts, and parietal cysts were reported , 3, 4. O2In February 2021, a 49-year-old woman was admitted to Shohada Hospital in Tabriz, Iran, with a complaint of pain in the sacral region. The patient had a history of surgery due to pulmonary hydatidosis and osseous hydatidosis in the left hip four years ago. She was from a rural area in Hashtrood County, East Azerbaijan Province, Iran, and had close contact with dogs and sheep. She had experienced progressive swelling in the sacral region for one year. Written consent was obtained from the patient for using the images and publishing this case report.3On admission, the analysis of blood samples revealed a normal range for hematocrit (38.1%), hemoglobin level (12.6 g/dL), erythrocyte sedimentation rate (ESR: 17 mm/h), platelet count , and WBC count ; however, neutrophilia (71.2%) and lymphocytopenia (23.3%) were detected. Also, the C-reactive protein was measured to be 1+.Computed tomography (CT) and magnetic resonance imaging (MRI) were carried out for the patient . The CT According to the patient's clinical signs, CT and MRI findings, and medical history, the final diagnosis of osseous hydatidosis was established. Surgery was the treatment of choice. Radical excision of the soft tissue and curettage of the iliac wing were performed, and the bone was filled with cement. Under the central operating room conditions and general anesthesia, the skin and subcutaneous tissue were cut using a posterior approach. After making an incision, the larger cyst of the soft tissue and cysts of the left wing of the ilium, which also affected the sacroiliac joint, were carefully removed.After removing the cysts, the area was curetted; a hypertonic solution was injected; the area was rinsed extensively with normal saline and ethanol; and bone defects were filled with cement. Moreover, chemotherapy was initiated with oral albendazole (400 mg twice a day) prior to surgery and continued for four weeks after surgery. The removed cysts were sent for pathological examinations, which also confirmed the diagnosis of hydatidosis. Chemotherapy was continued for one month after surgery.4Hydatidosis is a worldwide zoonotic disease and a global public health problem. Hydatid cysts grow gradually and can be usually found in visceral organs. They are endemic in developing countries, such as Iran, where sheep breeding is common. The highest prevalence of CE has been reported in temperate-zone countries, including Central Asian countries, China, Australia, the Mediterranean region, and parts of South America .As mentioned earlier, osseous hydatidosis is a rare phenomenon. This disease may remain asymptomatic for years, and its clinical symptoms are often insignificant. It results in the replacement of the trabecular bone by various slow-growing vesicles that proliferate along the medullary canal, without creating encapsulated cysts similar to other organs; therefore, diagnosis can be delayed . OsseousThe differential diagnoses for osseous hydatidosis are broad and include osteosarcoma, chronic osteomyelitis, chondrosarcoma, osteoclastoma, some tumors, such as aneurysmal cysts, metastases, and tuberculosis . BesidesThe surgical removal of osseous cysts is the primary treatment method; however, due to the high recurrence rate, chemotherapy is also recommended along with surgery, and replacing damaged bone with artificial bone , 14, 15.5Although osseous hydatidosis is not a malignancy, it is a severe disease that should be differentiated from diseases with a poor prognosis.All authors listed have significantly contributed to the investigation, development and writing of this article.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Data included in article/supplementary material/referenced in article.The authors declare no conflict of interest.No additional information is available for this paper.


Abdominal cocoon syndrome is a rare condition characterized by small bowel encapsulation by a fibrous membrane or a cocoon-like sac. It is an uncommon cause of intestinal obstruction. Less than 300 cases have been reported from all over the world. This is the first case of such a disease entity to be reported from Sudan.A young female patient, presented with features of intestinal obstruction that was managed conservatively. Failure of the conservative management has warranted a laparotomy.Her features were suggestive of intestinal obstruction that was confirmed radiologically.Laparotomy revealed a membrane-like fibrous material and extensive multiple loops adhesions, findings consistent with primary sclerosing encapsulating peritonitis (PSEP), also known as abdominal cocoon's disease. The membrane was excised and adhesiolysis was done. Intestinal obstruction was relieved after surgery and the patient showed good outcome.Abdominal cocoon syndrome is a rare cause of intestinal obstruction.The takeaway lesson from this case would be that the PSEP should be sought in any patient with no clear cause for obstruction can be identified. A contrast-enhanced CT scan is the diagnostic modality of choice. Finally, we think that the disease is underreported from Africa and more efforts should be carried out to increase patients' access to healthcare especially in rural areas with no access to hospitals in order to bring more cases to light. This case report has been reported in line with the SCARE Criteria . This condition can be classified into primary or secondary, in terms of etiology 2A 16-year-old female student, presented to our emergency department by her family with complaints of persistent vomiting, nausea, constipation, and epigastric abdominal pain along with weight loss. She has a history of on-and-off constipation, but no previous history of hospitalization or surgical operations. She has no history of allergies or chronic medications consumption. She lives with her parents and does not smoke or drink alcohol. Regarding her family history, there was no history similar presentation.3Her physical examination showed a distended abdomen with full flanks, and it was dull on percussion. There was tenderness over the epigastric area but no grading. There was mild hepatomegaly, and she was slightly jaundiced. Her vital signs were as follows: PR = 78, BP = 80/70, T = 36,9 and SPO2 = 98% on room air. Her respiratory and cardiovascular systems were normal.4Our patient has presented first to the emergency department with constipation and vomiting and was admitted to the internal medicine department. Three days after admission and conservative treatment with no response, we have been called in the surgical department for consultation. Based on her symptoms, a diagnosis of bowel obstruction was made and emergency surgery was arranged which revealed the abovementioned findings. She was discharged one week later in a good condition. A two-week later follow-up visit was scheduled which was satisfactory with a good outcome. A one-month later, phone interview was scheduled given the fact that the patient has returned to her home country (South Sudan).55.1Lab investigations revealed a normal WBCs count with low Hb at level of 8.5. Regarding her electrolytes, serum calcium and potassium were low  . Renal function tests and bleeding profile were normal, while her liver function tests including total protein, albumin and T bilirubin were low  ), but direct bilirubin was slightly high (0.560 mg/dl) . Viral screening was negative, urinalysis showed uncountable pus cells. CRP (5.38 mg/l)  and ESR (50.0)  were high. Blood grouping is A+.Abdominal ultrasound showed a mildly enlarged liver with excessive bowel gases, but otherwise, it was unremarkable. CT scan of abdomen and pelvis  shows a 6Based on the presence of abdominal pain, constipation, vomiting, and abdominal distension along with the radiological findings, a diagnosis of bowel obstruction was made, and the patient was managed conservatively . Three days later, a laparotomy was conducted due to the failure of the conservative management. Intraoperatively a shiny grey-whitish cocoon-like sac encapsulating the small bowel was observed . The sacOperator: Mr. Nadir Salih, a senior consultant of general surgery. He attained his fellowship at the Royal College of Surgeons in Ireland in 2002. He progressed from surgical SHO to surgical registrar then to a senior surgical registrar, he did covers as a locum consultant in Ireland before he returned to Sudan in 2010. He joined the Faculty of Medicine, Alzaiem Alazhari University as an assistant professor, and as a consultant surgeon in Khartoum North Teaching Hospital in the same year. The operation was done in a private hospital in Khartoum state.7The patient was discharged seven days post-operatively with no in-hospital complications. Two weeks after discharge, and at the clinic for follow-up, there were no complications nor evidence of relapse. The histopathology report revealed mild chronic inflammatory infiltrate in the submucosa on the appendix. The serosa and peri-appendiceal adipose tissue showed congestion, edema, and infiltration by mixed inflammatory cellular infiltrate. There was no evidence of neoplasm or specific granuloma in the sample examined, which excluded abdominal tuberculosis. The features are consistent with those of peri-appendicitis. Microscopy of mesenteric lymph nodes showed normal architecture with marked follicular hyperplasia with expanded parafollicular T cell region. There was no evidence of granuloma or malignancy in the biopsy. The features are consistent with those of reactive follicular hyperplasia. Four months later, a second follow-up phone call revealed no recurrence of her symptoms, and she was leading a normal life.8Primary sclerosing encapsulating peritonitis (PSEP) is a rare disease entity in which there is a thick, grayish-white membrane made of fibrous tissues and collagen enclosing the abdominal structures partially or totally, a cocoon-like sac There is no identifiable cause for Primary sclerosing encapsulating peritonitis (PSEP), although a role of cytokines, fibroblasts, and angiogenic factors has been suggested More than half of patients have been diagnosed during the surgical operation and 16.7–48.7% of patients have their diagnosis preoperatively according to large case series conducted in China While there is no absolute agreement on the best diagnostic and therapeutic approach as most of the studies reported on this disease are case reports with only one systematic review available, dated to 2015 Additionally, A clue to the diagnosis may be apparent on barium studies of the small bowel as a cauliflower sign That is being mentioned, most cases, especially in young population, are diagnosed during laparotomies after the failure of conservative management of the bowel obstruction with a combination of clinical, radiological, surgical, and histopathological findings being the best way to have an ultimate and sure diagnosis given the rarity of this disease entity. Excision of the membrane and adhesiolysis, whether through an open approach or laparoscopically is the usual management.There is a limited number of cases reported from the African continent when compared to other regions of the world, including 5 reported cases from Nigeria and one reported case from Senegal Written informed consent was obtained from the patient's parent for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer-reviewed.Ethical approval was obtained from Ethical committee at Alzaiem Alazhari University.Authors received no funding from any individual or institution and this work is completely a voluntary work.Farah Mohammed.Not applicable.1.Farah Mohammed: Involved in study design, data acquisition, drafting the article, revising it critically and finally approved the manuscript.2.Mohamed Abdulkarim: Involved in study design, data acquisition, drafting the article, revising it critically and finally approved the manuscript.3.Ammar Ibn Yasir: Involved in conception of the study design, drafting the article and finally approved the manuscript.4.Osman Taleballah: Involved in conception of the study design, drafting the article and finally approved the manuscript.5.Dafalla Shani: Involved in the design of the study, revising it critically and finally approved the manuscript.6.Nadir Salih: Involved in the design of the study, revising it critically and finally approved the manuscript.Authors report no conflict of interest of any sort.


Teratoma in the medulla oblongata is extremely infrequent and has been rarely reported. Severe and sustained brain stem compression resulting from these granitic tumors may cause potentially fatal impairment. Here, we reported a novel case of teratoma that occurred in the medulla oblongata. This 15 year-old boy suffered from a progressive gait disturbance and weakness of limbs for nearly 13 years. Magnetic Resonance Imaging (MRI) revealed an unusual mixed mass in the medulla oblongata and C1-2 spine, which was confirmed as mature teratoma by histopathological examination. Then, surgical resection was performed, followed by postoperative continuous rehabilitation. After a period of rehabilitation, this patient is currently able to mobilize with sticks. No signs of local recurrence occurred. Conclusively, surgical removal is still the preferred treatment for teratoma. Intracranial teratomas, one of Central Nervous System (CNS) non-germinomatous germ cell tumors (NGGCTs), according to the 2016 CNS WHO classifications, account for 0.5% of all intracranial tumors and 2–4% of intracranial tumors in children –5. SurgeA 15 year-old boy was admitted to our hospital with a 13 year history of progressive gait disturbance and weakness of limbs. Magnetic resonance imaging (MRI) performed in 2008 showed a mass lesion with mixed signal compressing the medulla oblongata (the primary image was lost because of floods that year). However, his parents refused surgery due to high risks of neurological impairment and chose the palliative treatment of achilles tendon lengthening instead. Symptoms were slightly alleviated in the following years. One year ago, the patient received a Computed Tomography (CT) scan revealing a predominantly calcified lesion in the same area of the medulla oblongata. Subsequently, MRI examination  indicateThe patient underwent a craniotomy via a midline sub-occipital approach in prone position. A vertical skin incision was made from the external occipital protuberance to the C2 spinous process. The foramen magnum was opened and then C1-2 laminectomy was performed. Intraoperatively, a slightly swollen medulla oblongata was exposed. An ossified crust surrounded by soft material and partial calcified portion were visible. We carefully isolated the tumor border from the brain stem and performed a complete removal of the intramedullary lesion. The patient tolerated the procedure well without any neurological deterioration.Pathological examination  found wePostoperatively, the strength of the right limbs recovered to 4+/5 with normal muscle tone, while the left remained as it was. Then, the boy received hyperbaric oxygen therapy and exercise-based rehabilitation, involving skin care, muscle massage, and passive mobility training of left limbs. During the follow-up period of 1 year, the strength of the left limbs had improved progressively. Currently, he is able to walk independently without any evidence of tumor recurrence.Intracranial teratomas are comprised of mature cells that originate from three embryonic layers: ectoderm, mesoderm, and endoderm. During the 3rd to 4th week of embryonic development, neural tube closes and these tissue implantation leads to cyst formation. Some misplaced cells of the embryonic disc, may be involved in this movement and migrate with the moving cells of the mesoderm, especially the lateral mesoderm, to the future cranial area . Our casThe World Health Organization has classified teratoma into the following groups : mature As midline lesions, these tumors usually occupy the pineal and suprasellar region . Rarely,Teratoma in the medulla oblongata is extremely infrequent. According to our extensive literature review, only four cases of primary teratoma in medulla oblongata had been reported so far  3–5). C–5. C3–5)Until now, the aim of treatment always be radical resection of tumors, and postoperative adjuvant treatment for intracranial teratomas has remained controversial. Lee et al. and Bi et al. both hold the opinion that mature teratomas may be resistant to chemoradiation and could eventually grow during adjuvant therapy , 19. As Our study has some limitations. Although, there was no positive biomarkers in serum that could make sure of mature teratoma specifically, we failed to detect the relevant markers in patient's CSF, which may have missed some meaningful results. However, the gold standard still depends on the postoperative pathological findings. Furthermore, his parents had been delayed for many years to decide the operation, and the symptoms developed to a more serious situation, otherwise he may got a better outcome. Currently, this study was a retrospective study performed in a single case, and the follow-up duration was not long enough for benign tumors. As well as the extremely low incidence of this disease, there was ineluctable statistical deviation in the literature review. It is necessary to conduct related cases in the future for further summary.This study retrospected an extremely rare case of mature teratoma in the medulla oblongata. Bsed on our experience, early surgical resection with the capsule maintained intact is a preferable option before irreversible neurological dysfunction occurs. The surgeon needs to maintain the balance between the degree of tumor resection and the preservation of neurological function. Also, a long-term follow-up and further summary of related cases in the future is required to obtain a definite prognosis. Finally, further studies focusing on biological characteristics are needed, which can help the management of this tumor in the medulla oblongata.The datasets presented in this study can be found in online repositories. The names of the repository/repositories and accession number (s) can be found in the article/Written informed consent was obtained from the individual (s), and minor (s)' legal guardian/next of kin, for the publication of any potentially identifiable images or data included in this article.XZ, TX, and JC: conception or design of the work. XZ, HW, and FH: drafting the paper. TX and HW: revising it critically for important intellectual content. TX and JC: provide approval for publication of the content. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Acute necrotizing encephalopathy is a devastating clinico-radiological syndrome characterized by fulminant neurological deterioration after an antecedent febrile illness, as well as the imaging hallmark of bilateral thalamic involvement. Herein, we describe a 4-year-old boy with typical clinical and neuroimaging features of acute necrotizing encephalopathy. The bithalamic swelling led to a block of cerebrospinal fluid circulation at the foramen of Monro, thereby causing the mild dilatation of lateral ventricles. The periventricular areas could, therefore, have been potentially affected by the acute necrotizing encephalopathy per se and/or transependymal edema secondary to obstructive hydrocephalus. The information from diffusion imaging allows for differentiation between these two pathophysiological processes. He was admitted for supportive care. However, consciousness disturbance and seizure developed on the day of hospitalization. A diagnostic lumbar puncture was performed, revealing elevated leukocyte count (30 cells per µL), erythrocyte count (190 cells per µL), and protein level (212 mg/dL) in cerebrospinal fluid. Magnetic resonance imaging of the brain on the next day revealed the mild dilatation of lateral ventricles a,b, as wANE is a distinct clinico-radiological syndrome characterized by fulminant neurological deterioration following a febrile illness, as well as bilateral thalamic involvement on neuroimaging. The radiological picture of bilateral diffuse thalamic lesions with mass effect, as illustrated in this case, may occasionally mimic bithalamic glioma , Leigh sANE has been reported across different age groups and geographic regions, while it is most often seen in young children in East Asia . Both ge


We present a case of tubular esophageal duplication in a 3-day-old female newborn  without concomitant abnormal development. Esophageal duplication was diagnosed based on the clinical picture, direct laryngoscopy, esophagography and computed tomography. The duplicated esophagus was resected by thoracoscopy leaving the orthotopic esophagus in place. Isolation from the pharynx was performed via a separate cervical incision. After a follow-up period of 20 months, the child returned to normal growth and development. Congenital tubular esophageal duplication is a rare congenital anomaly in which the second esophagus with mucous, submucous, and muscular membranes, corresponding to the esophagus, is adjacent to the true esophagus without a common wall. This condition causes dysphagia, nausea, vomiting, retrosternal pain or respiratory problems (stridor and recurrent pneumonia). Presentation typically occurs during the newborn period. Tubular esophageal duplication represents approximately 10% of all foregut duplications.We report the case of a 3-day-old female  presented to our surgical clinic from the maternity hospital with a history of respiratory distress, salivation, and dysphagia. The newborn was delivered via caesarean section to a primigravida mother. There was no visible additional malformation. On a physical examination in maternity hospital, she had tachycardia and tachypnea, maintaining oxygen saturation of 88% at room air. The patient aspirated and was intubated and ventilated, a nasogastric tube was placed. The child was consulted by a surgeon and transferred to a surgical clinic for further examination. During laryngoscopy, two esophageal lumen were discovered. Two nasogastric tubes were placed. First into the blind-ending esophagus and second into the normal esophagus under fluoroscopic guidance. The nasogastric tube was used for enteral feeding while the tube in the long and blind-ending duplicated esophageal pouch was attached to low pressure suction pump. A contrast esophagram confirmed a tubular esophageal duplication extending from the cervical region to the diaphragm without any gastric communication. The duplicated segment was found on the left side in the neck and on the right posterior side of the normal esophagus in the thorax . ComputPediatric tubular duplication of the esophagus is a rare congenital anomaly. The incidence of congenital esophageal duplication is estimated to be 1:8,200, with male sex predominance .


Primary malignant tumors of the heart are rare. Although preoperative histological diagnosis is difficult, it has paramount value in therapeutic strategy development and prognostic estimation. Herein, we reported 2 cases of intracardiac tumors.Both patients presented to the hospital with heart-related symptoms. Echocardiography showed massive masses in the atrium and positron emission tomography–computed tomography (PET/CT) revealed hypermetabolism and invasiveness. One patient cannot take surgery due to extensive metastasis and poor condition. The other patient was primarily diagnosed with lymphoma, and surgery was not recommended. They successfully underwent intravenous atrial biopsy, and histological samples confirmed intimal sarcoma and diffuse large B cell lymphoma. Based on immunohistochemical and molecular assessments, targeted chemotherapy was administered, resulting in clinical and imaging remission at discharge.Percutaneous intravenous catheter biopsy as a safe invasive test provides an accurate pathological diagnosis after imaging evaluation, and offers a therapeutic direction. Nonmalignant masses and some chemo-radiosensitive malignant tumors in the atrium could have good prognosis after targeted therapy. Primary cardiac tumors (PCTs) are extremely rare, with an autopsy incidence ranging from 0.001 to 0.030% . A meta-Case 1: A 48-year-old male was presented with palpitation and chest distress after activity for 3 months. Echocardiography revealed a large mass (maximum size: 58 mm × 40 mm) attached to the lateral and posterior atrial walls, invading the inferior vena cava. Cardiac magnetic resonance (CMR) imaging showed right atrial soft tissue mass close to the tricuspid orifice with the valve leaflet Fig. a–d.Fig. To confirm the nature of the mass, PAMB was performed through the femoral vein, wherein three tissues were removed. The pathological analysis revealed thrombus which was consistent with imaging features. We speculated that blood flow changes caused blood turbulence. This could increase the risk of thrombus adhesion on the mass surface and influence biopsy results. After 3 weeks, the patient underwent second PAMB through the internal jugular vein. With the bedside echocardiography auxiliary positioning, three tumor tissues were removed from the atrial mass. The pathological report concluded intraepithelial metaplasia; the abnormal cell pattern showed high-grade spindle cell neoplasm with moderate atypia and focal necrosis, consistent with intimal sarcoma histologically  attached to the left atrium that invaded the right atrial walls and tricuspid annulus. The atrial mass protruding into the pericardial cavity caused moderate pericardial effusion and early signs of tamponade. CMR confirmed the presence of an invasive intracardiac mass , and had a dismal long-term survival rate despite various modes of treatment . The oveAlthough intracardiac mass is considered as a dangerous signal of PCM, it should be noticed that a neoplasm located in the atrium usually causes less systolic dysfunction and ventricular arrhythmia. Moreover, benign lesions such as thrombus and myxoma, accounting for a large proportion of atrial masses, but seldom appear in ventricles. Therefore, the criticality of atrial mass is different from that of ventricular mass. For atrial mass, more effort is needed to enable precise diagnosis, especially histological diagnosis. Echocardiography, CT, CMR, and PET/CT imaging could help in the preliminary differentiation between malignant and benign tumors based on tumor shape, aggressiveness, and radioactive material intake , 12. HowThe endomyocardial biopsy technology has been widely used in cardiomyopathy, and has been extended to ventricle mass biopsy. By contrast, atrial biopsy has not been promoted for some reason. Atrial biopsy means thinner cardiac muscle walls, closer intervention to the relatively fragile vein, and higher risk of thrombus detachment. In 1989, Gosalakkal and Sugrue  firstly Among 37 cases with reported malignant tumors, lymphomas accounted for the largest proportion (15/37), followed by soft tissue sarcomas (13/37), and 9/37 reported metastatic tumors. Table For various spatial atrial mass locations, different vessel approaches and ultrasonic guidance methods could be selected in PAMB. Biopsy has been performed via the internal jugular, femoral and subclavian veins. Transesophageal echocardiography (TEE) is highly effective in mass locating and bioptome guidance. Hence, about 50% of physicians use TEE as a supplementary tool to digital subtraction angiography (DSA). Transthoracic echocardiography (TTE) is more convenient but provides less location information compared with TEE, thereby necessitating highly experienced doctors. Intracardiac echocardiography (ICE) can provide the most precise intra-atrial imaging and structures, but requires an extra-intravenous ultrasound catheter, which limits its application; only about 33% of physicians utilize this method. Table Percutaneous intravenous catheter biopsy provided accurate pathological diagnosis after imaging evaluation, avoiding unnecessary surgical excision and reducing medical resource waste. Benign tumors, thrombus, bacterial vegetation, and lymphoma detected by PAMB could be cured. Nevertheless, when pathological biopsy results suggest thrombus but do not correspond to clinical symptoms, doctors should consider the possibility of false negatives. Multiple biopsies from different vessel approaches and varied mass locations might reduce the probability of misleading results. However, whether biopsies are beneficial to PCM patients is currently controversial. Ryo et al.  speculat


Taenia solium. Cysticercosis is acquired via the fecal-oral route and is prevalent in low- and middle-income countries (LMICs). Patients typically manifest with skeletal muscle, subcutaneous, or central nervous system involvement. Though there are reports of oral mucosa involvement, solitary involvement of the parotid gland is rare. This is a rare case of a 57-year-old man diagnosed with parotid cysticercosis by imaging and FNA. He was successfully treated by anthelminthic therapy and needle aspiration. The patient has been seen back several times. The cyst is not palpable, and he is satisfied. Parotid cysticercosis should be considered in the differential of a parotid mass in patients who have traveled to endemic regions. Though prior reports have indicated the importance of surgical excision, this patient was treated medically.Cysticercosis is a systemic parasitic infection caused by the establishment of the larval form of the parasitic cestode,  T. solium. Cysticercosis is endemic to many LMICs that lack proper sanitation, resulting in environmental fecal contamination of both food products and water [Cysticercosis is a parasitic disease caused by the larval stage of the cestode, nd water . RegionaSymptom presentation of cysticercosis is variable and is dependent on the location and number of cysts (cysticerci) involved . TissuesTraditionally, cases of cysticercosis are diagnosed by imaging and serological methods. Histopathological examination of excised lesions can be performed after the time of surgery –5. The mA 57-year-old man presented with a chief complaint of right facial swelling after traveling to South Korea. Physical exam demonstrated a palpable right parotid mass. The patient had no other symptoms and denied dysphagia, pain, swelling, or difficulty opening their jaw.CT scan of the neck with contrast demonstrated a 2.1 × 1.0 × 2.0 cm ovoid low-density rim-enhancing lesion in the superficial lobe of the right parotid gland .FNA of the right parotid and right lymph node demonstrated benign cyst contents from the parotid, from the right lymph node, and a polymorphous lymphoid population consistent with lymph node sampling. There was no cytologic evidence of malignancy. However, a single degenerated structure suspicious for the neck, scolex, and partial strobila of the cestode was identified . The sliThe patient returned and noted some residual right facial swelling. The mass was nontender, and the patient denied dysphagia or pain with chewing. Of note, the patient had not traveled and had no further infectious exposures. An ultrasound was performed and revealed a residual 1.9 × 0.9 × 0.8 cm hypoechoic lesion within the right parotid gland . A repeaThe patient returned 6 months following the initial diagnosis, and his swelling and symptoms were much improved. At the time of publication, he continues to do well without any further treatment.T. solium is a cestode parasite (tapeworm) that consists of a head (scolex) with suckers and hooklets, a neck, and numerous flat segments that contain both male and female reproductive structures [T. solium causes cysticercosis infections secondary to the development of larvae after the ingestion of tapeworm eggs. Cestodes have a complex life cycle that requires two mammalian hosts: a pig intermediate host, which supports the nonreproductive form of the parasite, and a human definitive host, which supports the reproductive form [T. solium cysticerci establish in tissues where they degenerate after a certain period and cause a local inflammatory response. When intermediate hosts (pigs or humans) ingest eggs in food or water contaminated with feces, the egg hatches and the oncosphere penetrates the gut wall [ructures . T. soliive form , 2. T. sgut wall , 2. The gut wall , 2, 4, 8gut wall , 2, 4, 8Cysticercosis of the oral mucosa is rare, and according to a case report by Sharma and Kaur, only 38 cases of oral cysticercosis have been published in the literature as of 2017 . This caThe diagnosis of parotid cysticercosis should be suspected in patients with a solitary, well-circumscribed, mobile, nontender parotid mass. The diagnosis is established based on clinical manifestations, imaging findings, cytopathological analysis, and epidemiologic exposure –8. The mTreatment of cysticercosis was successful in all previously documented cases and has become less invasive in recent reports –7. EarliThis is a rare case of a 57-year-old man diagnosed with parotid cysticercosis by imaging and FNA. He was successfully treated by needle aspiration and anthelminthic therapy. Parotid cysticercosis should be considered in the differential of a parotid mass in patients who have traveled to endemic regions. Though prior reports have indicated the importance of surgical excision, this patient was treated medically.


Gallbladder agenesis (GA) is a rare embryological anomaly that presents acute cholecystitis like-symptoms. It is often an incidental finding diagnosed during surgery. We reported a case of GA in a patient who presented with dyspepsia and acute right upper abdomen pain with ultrasonographic signs of acute lithiasic cholecystitis. The preoperative assessment, according to first-level exams, is oriented to the diagnosis of acute lithiasic cholecystitis with atrophy and sclerosis. During laparoscopy, the proximal transverse colon was found strictly adherent to gallbladder fossa. The gallbladder was found to be absent. The surgical procedure consisted of lysis of multiple colo-hepatic adhesions. The diagnosis of congenital GA was made laparoscopically. The postoperative radiological images, based on CT and MR examinations, documented the diagnosis of GA with a biliary duct anatomical variant. The recovery was uneventful and the patient remained symptom-free for more than four years. GA is a clinical challenge that still poses diagnostic and therapeutic dilemmas. Although no diagnostic and therapeutic algorithm is accepted worldwide, due to heterogeneity of clinical scenarios and the variability in hospital facilities, surgeons have to be familiar with this rare entity, and conversion in laparotomy or unnecessary operative procedures should be avoided in the same operative setting. Gallbladder agenesis (GA) is a rare embryological anomaly with an incidence of 10-65 per 100,000 ,4,6-8. G3/µl) and a total serum bilirubin level of 30.95 µmol/L (conjugated bilirubin level of 6.84 µmol/L). Liver function tests were within normal limits . The troponin test showed normal values (9.10 pg/ml). Laparoscopic cholecystectomy is indicated due to clinical and AU signs of acute lithiasic cholecystitis. During exploration, the proximal transverse colonic flexure was found strongly adherent to the gallbladder fossa but the gallbladder was found to be absent. The surgical minimally invasive manoeuvres consisted of lysis of multiple peritoneal adhesions and dissection of the porta hepatis (Figure An 84-year-old-man presented acute abdominal pain localized in the right upper quadrant associated with dyspepsia and vomiting. He had no previous abdominal surgery and he was taking oral hypoglycemic agents. He had myocardial revascularization, more than 10 years ago, with aorto-coronary bypass. In his history, there were recurrent biliary colics as documented by personal examinations. Preoperative AU showed the signs of acute lithiasic cholecystitis with contracted and shrunken aspects Figure /µl and aFirst described in 1701, the pathogenesis of GA still remains unclear although an embryological malformation has been hypothesized -6. GA miIn conclusion, hepato-biliary surgeons have to be familiar with this rare entity because GA therapy requires prudence. More and more frequently, GA might be a laparoscopic surprise, and the conversion to open surgery should be kept as low as possible by offering to patients all the advantages of minimally invasive procedures. A modern and accurate imaging investigations have to be indicated, whenever possible, during laparoscopy by using intraoperative ultrasounds or after the intraoperative GA finding, with the aim of avoiding biliary complications.


This report presents a case of a one-month three-day-old full-term female infant with hydrometrocolpos (HMC) and post-axial polydactyly whose first clinical sign was acute intestinal obstruction and hydroureteronephrosis, caused by compression of the structures due to the increasing size of the cystic-like pelvic mass. This is the first report of HMC with post-axial polydactyly complicated with acute intestinal obstruction in Peru. It raises importance on prenatal diagnosis, management and complications of HMC. Although it is rare, clinicians should have it as an option when discussing abdominal cystic masses in neonates to perform early management and avoid complications. Continuous follow-up should be carried out on patients presenting with HMC and post-axial polydactyly to assess for Bardet-Biedl syndrome, which could affect different systems in those patients long-term. Hydrometrocolpos (HMC) is an uncommon pathology worldwide that consists of the accumulation of fluid in the uterus and vagina due to some malformation that blocks the drainage of cervical secretions. HMC, when is accompanied by post-axial polydactyly, may be related to certain syndromes such as McKusick-Kaufman syndrome (MKKS), which more often presents in children between 5 and 10 years old. The difference between them varies according to other clinical presentations.  while hyA one-month and three-day-old full-term female infant was admitted to the emergency room due to five days of progressive abdominal distension. One day before being admitted to the hospital, she presented alimentary vomiting and obstipation. Physical examination revealed marked abdominal distension with collateral circulation and a palpable mass in the lower hemiabdomen of soft consistency with regular margins of 8 cm approximately.In addition, the urethral orifice is evidenced in the inferior middle third of the vulvar vestibule and vaginal introitus is not distinguished. Also, post-axial polydactyly is evidenced in all extremities. After admission, an abdominal ultrasound Figure  and comp Findings suggested hydro-hematocolpos which extended into the vaginal canal with no distinguishable communication or fistula between the bladder and vagina. Additionally, a congenital bilateral grade IV hydroureteronephrosis was observed. Pediatric surgery was consulted, who performed a colpostomy in which clear fluid is drained from the uterine cavity; drainage was placed. Two weeks later, a brownish foul-smelling draining material was evident. Immediately, an emergency exploratory laparotomy was performed, in which purulent material was obtained after performing a hysterotomy. The diagnosis of infected HMC was confirmed. Consequently, a permanent drain was placed, which requires monthly replacement. One week after surgery, a pelvic MRI was performed with suggestive findings of hydro-hematometra, which compresses the rectum, bowel loops and ovaries. The patient is still to be programmed for definitive surgery by specialists.HMC is a malformation caused by vaginal atresia, imperforate hymen or a transverse vaginal membrane . AlthougAntenatal diagnosis of HMC is usually challenging as it is ultrasound dependent. Usually made during gynecological visits since the third trimester of pregnancy and can be misdiagnosed with other abdominal fetal cysts . SimilarManagement should be performed with early drainage of the HMC, either by hymenectomy or laparotomy for patients with a high vaginal obstruction or resolution of associated abdominal complications. A quick and temporary decompression with an aspiration of the cystic mass or vaginostomy, which reduces the chances of sepsis, is a complication that has been reported before ,9. PreviThis is the first report of HMC with post-axial polydactyly complicated with hydroureteronephrosis in Peru, where its prevalence is unknown. This case highlights the importance of prenatal diagnosis, management and complications of HMC. Although it is a rare disease, clinicians should consider this pathology as a differential diagnosis when discussing abdominal cystic masses in neonates in order to perform early management and avoid complications, as contemplated in this case. We further recommend the periodic follow-up on patients presenting with HMC and post-axial polydactyly as it could be part of the BBS syndrome which could affect different systems in those patients long-term. The association of HMC and post-axial polydactyly represents a challenging diagnosis. Although clinical manifestations can be observed at birth, a definitive diagnosis can only be made several years later. Surgical management remains the mainstay of treatment.