The duodenal web is a thin, elongated, web-like structure that is one of the factors contributing to duodenal obstruction. Only 100 cases have been reported in the literature. We present a 2.5-year-old cachectic Afghan child who did not have any overt signs and symptoms of intestinal obstruction, like recurrent vomiting, abdominal distention, and weight loss. The web was discovered near the intersection of the third and fourth portions, which is an uncommon location for the duodenal web. The late presentation of congenital duodenal web with partial obstruction is rare but well-known and has been reported in this case.A 2.5-year-old cachectic Afghan child who had recurrent vomiting and experienced abdominal distention was brought to Maiwand Teaching Hospital from the Jabelsuraj region of Parwan province. The patient was suffering from unusual signs and symptoms like recurrent vomiting, abdominal distention, weight loss, and constipation. The diagnosis of these anomalies was established by a detailed history, clinical features, and abdominal CT scan. In the computerized tomography scanning (CT-Scan) image reported, there was a web with stenosis and partial obstruction in the distal aspect of the third-to-fourth portion of the duodenum. After preoperative stabilization, the child was taken for surgery. The abdomen was opened by a right upper abdominal transverse incision. After web resection and duodenoplasty, the patient was shifted to the recovery room in satisfactory condition. The child was allowed to feed after 8 days, which he tolerated well.Congenital duodenal web with partial obstruction is typically observed in the second and third years of life. It is suspected in patients with recurrent vomiting, abdominal distention, weight loss, and constipation. Partial obstruction may not have an overt presentation, making it a challenging diagnosis for general practitioners. Abdomen X-ray and CT scan usually confirm the diagnosis, and successful surgical intervention is recommended. A duodenal web, which is one of the causes of duodenal obstruction, is a thin, round, partially permeable, web-like structure. It is a rare condition; with approximately 1 in 10,000 to 1 in 40,000 cases reported in the literature , 8.CongeA 2.5-year-old cachectic Afghan child who was vomiting and experiencing abdominal distention was brought to Maiwand Teaching Hospital from the Jabulseraj region of Parwan province. The patient was suffering from fever, recurrent vomiting, abdominal distention, weight loss, constipation, and painful defecation. The patient's parents were consanguineous and had a normal prenatal history. The kid experienced food intolerance at this time, and he vomited four to five times daily, forcefully and non-biliously, often 30 min to an hour after feeds, according to his mother's explanation. The patient also experienced constipation, fever, and stomach distention at the age of one year. If he was fed primarily solids, his vomiting would worsen. Due to this, at the age of 2.5 years, his diet was primarily composed of milk and small portions of semisolid food.His parents took him frequently to their local general practitioners, who would give him medical treatment, but no remedy was achieved. As he did not have any overt signs and symptoms of intestinal obstruction, the local general practitioner did not think of the underlying organic cause of the vomiting. The parents were concerned as the vomiting was persistent and associated with weight loss. His parents added that they took the child once outside the country for better treatment at the age of 1.5 years. The reason for his referral to the capital and then to our pediatric service was the ongoing deterioration of his condition.He is the seventh child of his parents; he was born normally at term, and since birth, he has received on-demand breast milk. At 7 months old, the mother tried complementary feeding, but she was unsuccessful due to the infant's ongoing vomiting.In the family history, his two brothers suffered duodenal web and passed away during surgery.On physical examination, in the abdomen, bowel sounds were normal. There was no enlargement of the liver and spleen, but the abdomen was distended. The rest of the physical findings were normal.On the imaging study, the upright abdominal X-ray showed marked distension in the stomach and duodenum, and the ‘’double bubble’’ sign was observed. The gas detected within the distal intestinal loops pointed to a partial obstruction Fig. .Fig. 1ThThe abdominal CT scan with contrast noted severe distension in the stomach and duodenum; furthermore, there was stenosis in the distal aspect of the third-to-fourth portion of the duodenum, as well as distension in the first and second portions. The web between the third and fourth portions of the duodenum was present, but the distal intestinal loops still showed contrast, suggesting a partial obstruction there Fig. .Fig. 2A The patient was admitted to the surgical ward, the parents were counseled, and informed consent was taken for an exploratory laparotomy. The abdomen was opened by the right upper transverse incision. Between the third and fourth portions of the duodenum, a large duodenal bulb with distal collapse was discovered. There were small food particles like beans, nuts, and seeds of the date palm in the dilated part of the duodenum. The web was removed, duodenoplasty was performed, and the abdomen was then sutured layer by layer. Intravenous fluids, broad-spectrum antibiotics (ceftriaxone and metronidazole), and proton pump inhibitors were administered for 7 days. Adequate analgesia was given. An NG tube was used to decompress the stomach. NG drainage was high for 3 days postoperatively, which gradually reduced, and the NG tube was removed on the fifth postoperative day. Oral feeding was started gradually, and the patient tolerated full oral feeds on the 8th postoperative day. On the 12th day post-op, he was discharged from the hospital in good condition. The patient was scheduled for follow-up, and after 40 days of follow-up, he was doing well, accepting a normal diet, and gaining weight of about 1 kg. He had no episodes of vomiting or abdominal pain . Symptoms of complete DW are more significant, like vomiting, abdominal distention, weight loss, dehydration, water and electrolyte imbalance, and constipation, which are seen in the neonatal period , 2, 9–11The signs and symptoms of partially congenital DW increase after the intake of complementary foods , 8. In tThe majority of the duodenal web 85% to 90%) occurs in the first and second portions of the duodenum. However, the third and fourth parts of the duodenum represent 20% and 10% of WD cases, respectively. % to 90% . In this50% of congenital DW is associated with other anomalies like Down syndrome, CHD, gut malrotation, hypospadias, inguinal hernia, cleft palate, and microophthalmia with pthysis bulbi , 6, 7, bAccording to the medical literature, few cases of DW-related deaths have been reported, and most of them had other anomalies such as Down syndrome and CHD , but thiMost cases of DW can be easily diagnosed by a simple X-ray and abdominal CT scan with contrast , 5, 10, Congenital DW with partial obstructions in children occurred in both genders, male and female –5, 9; inThe final diagnosis of DW was made after the surgery procedure, the investigation of the area, and seeing the webs.In the differential diagnosis of DW, other diseases such as congenital pyloric stenosis, duodenal atresia, annular pancreas, superior mesenteric artery syndrome, periduodenal portal vein, duodenal duplication cysts, and midgut volvulus must be considered. , 4, 7. TIn conclusion, in this case, the patient did not have specific signs and symptoms of DW due to the partial obstruction, which occurred in a later stage of childhood, and the web being attached to the 3rd and 4th portions of the duodenum. In developing countries such as Afghanistan, due to a lack of access to equipped medical services, most such cases are put aside and not considered specifically.Our recommendation for local general practitioners is that when they encounter such cases of recurrent vomiting, abdominal distention, constipation, and weight loss, the diagnosis of DW should be kept in mind, and the patient must be referred to a well-equipped medical facility for further investigation and treatment.


Soft tissue filler injection is the second most common nonsurgical cosmetic procedure. Despite the safety of fillers, as use has grown, so has the number of patients affected by adverse events. Ophthalmoplegia following cosmetic filler injection is a rare complication, mostly occurring after injection to the glabella, nasolabial fold, periorbital, and lateral nasal site. In all cases where ophthalmoplegia has been reported following fillers, patients have simultaneously experienced vision loss and other ocular symptoms. We report a case of isolated acute ophthalmoplegia following hyaluronic acid injection solely in the temple region. A 40-year-old woman, 3 hours after the procedure, presented to our hospital with left eye ophthalmoplegia, ptosis, and hypotropia. Treatment started with hyaluronidase, steroids, and anticoagulants. After 4 weeks, left eye ophthalmoplegia remained unchanged, and through a 10-week follow-up, all left ocular movements improved, and only mild hypotropia and ptosis persisted. This case report shows that ophthalmoplegia may also happen with temple region filler injections. We also review available prevention techniques and treatments to avoid such complications when performing soft tissue fillers for gaunt appearance correction. Soft tissue filler injection is the second nonsurgical cosmetic procedure in the United States, following botulinum toxin injection for aesthetic purposes.Ocular complications of fillers are mainly attributed to vascular occlusion events.This article presents a case of isolated acute ophthalmoplegia following filler injection solely in the temple region.A 40-year-old woman with a history of lip filler injection underwent cosmetic hyaluronic acid filler injections to the temples by an experienced physician, through a 27-gauge cannula. First, the filler was injected into the right temporal region with no complications. Then, during injection to the left temple, the patient experienced a sudden frontal headache, severe eye pain, nausea, and vomiting. The procedure was stopped immediately, though the mentioned symptoms remained. Hyaluronidase treatment was given in less than 15 minutes. About 20 minutes after starting the injection, the patient complained of diplopia and was referred to the emergency department for further examination and treatment.We visited the patient 3 hours after the event. The patient had binocular diplopia, frontal headache, eye pain, severe nausea, and vomiting. No vision loss or blurred vision of the left eye was reported. On ocular examinations, pupillary light reflex, ocular tonometry, ophthalmoscopy, visual acuity, and right eye movements were normal. The left eye had mild hypotropia and ptosis. Abduction and supraduction of the left eye were severely impaired; in addition, infraduction, extorsion, and intorsion were mildly limited. Adduction was the only intact left ocular movement .The rest of neurological examinations, including cranial nerves, were normal. No skin lesions were seen at the injection site . The paSuperficial soft tissue ultrasonography of the injection site reported no mass effect or subcutaneous collection. Magnetic resonance imaging (MRI) of the head and orbit, MR angiography, and MR venography of the head were unremarkable. Notably, there were no infarction, hemorrhage, or mass effect findings.After admission, treatment started with intravenous dexamethasone 8 mg three times daily and subcutaneous injection of enoxaparin 40 mg twice daily empirically for possible ischemic events, which administered for 5 days. Brimonidine eye drops were also prescribed. Enoxaparin 40 mg daily, brimonidine eye drops twice daily plus intravenous ondansetron 4 mg, and acetaminophen 1000 mg if needed, resumed until discharge. Headache, eye pain, nausea, and vomiting resolved a few hours after steroid administration. The patient was discharged after 2 weeks with persistent diplopia.Through a 4-week follow-up, extensive ophthalmoplegia in the left eye remained unchanged, with involvement of all eye muscles except for the medial rectus.With a 10-week follow-up, all left ocular movements improved, and only mild hypotropia and ptosis persisted .Although hyaluronic acid injection is considered safe for cosmetic purposes, there are reports of ocular complications following injection into the facial skin. Lee et al, in their review on 50 cases of ocular complications after filler injection, found that most ocular complications include visual loss with or without ophthalmoplegia, usually in nasal, glabella, and periorbital injections.However, rare cases of blindness have been reported following filler injection into the temporal region, and there are only two cases of isolated ophthalmoplegia following temple region injection.According to recent studies, the most discussed reason for ocular complications is ischemic events due to the possible intra- or periarteriolar injection. The temporal region has complex anatomy due to multiple vessels running in the different tissue layers. The anatomical layers of the temporal region are the skin, subcutaneous tissue, superficial temporal fascia, deep temporal fascia, superficial temporal fat pad, and temporalis muscle. There are several critical vessels located on the margin of the temporal region, including the zygomaticofacial artery  deriving distal to the internal carotid artery, and all terminal branches of the external carotid artery, including the zygomatico-orbital , middle temporal, and the superficial temporal arteries .In this case, the patient had ocular symptoms immediately after the injection, which raises vascular events as the leading cause of the patient's situation. The accepted theory of vascular events proposed in previous studies is the retrograde arterial flow mechanism. During the injection, the needle tip enters the small arterioles, and with excessive pressure on the plunger or long injection time, the pressure inside the needle overcomes the arterial pressure, which causes the retrograde movement of the filler toward the proximal parts of the artery. Then by cutting off the pressure inside the needle, the vascular pressure behind the filler material can move these substances to distal vascular branches parallel to the primarily injected arteriol, also known as embolia cutis medicamentosa .When a large-bore needle and a large syringe are used, the pressure could be enough to drive the dense material into the internal carotid artery, resulting in embolization of the cerebral circulation and cerebrovascular accident.Our patient had a history of lips filler injections. In similar patients, another mechanism that has been suggested is hypersensitivity reactions.The immune system becomes sensitive to the filler material for any reason in the initial injection. In the subsequent injections, the immune system causes systemic or localized complications by provoking inflammation.A retrospective study of 21 patients on the prognosis of ophthalmoplegia following filler injection for cosmetic purposes showed that ocular movements recovered entirely in 77% of patients who had diplopia at initial presentation, with incomplete recovery in 23%.Tips have been noted to preclude complications caused by the mechanism of vascular occlusive events (retrograde followed by anterograde): awareness of anatomical danger zones and considering the most suitable technique,Regarding the complications of vascular occlusion accidents, all studies have pointed to starting treatment as soon as possible to prevent lifelong sequels. The sooner treatment is started, the better the final prognosis. The proposed golden time is currently less than 90 minutes.In conclusion, isolated ophthalmoplegia following cosmetic filler injection is a rare complication, mostly occurring after injection to the glabella, nasolabial fold, periorbital, and lateral nasal site. This case report shows that ophthalmoplegia can also happen with temple region filler injections. Therefore, to avoid such complications, awareness of prevention techniques and available treatments are necessary when performing soft tissue fillers for gaunt appearance correction.


Brain tumors rarely present during pregnancy; however, a life-threatening interaction may develop between maternal and disease factors. Moreover, awake surgery has been an infrequent treatment option during this life stage. We contribute to this knowledge gap by presenting the case of a 33-year-old woman who developed tonic-clonic seizures during the 18th week of pregnancy due to a neoplastic lesion near the left motor area. A multidisciplinary team performed an awake craniotomy for tumor resection and the histopathological examination revealed a diffuse astrocytoma. On the follow-up, radiotherapy was administered and the patient delivered a healthy newborn at week 37. A frequency of 3-6 per 10reported . MoreoveGliomas have been the most frequently reported primary brain tumors in this stage with few cases treated with awake surgery . Awake cA 33-year-old woman in the 18th week of pregnancy presented with a history of two episodes of tonic-clonic seizures and severe headaches that worsened progressively. The neurologic examination revealed right hemiparesis and a brain magnetic resonance imaging (MRI) showed a left frontal proliferative lesion causing deformation of the frontal gyrus which was hypointense in the T1-weighted sequence and hyperintense in the T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences (Figure A collaborative effort was established between the departments of Neurosurgery, Obstetrics and Gynecology, Psychology, and Anesthesiology. Because the patient remained symptomatic and with a high risk of further cerebral herniation, the best treatment decision was to perform an awake craniotomy with intraoperative fetal monitoring. The patient was educated about the risks and benefits of surgery and the maneuvers she would need to perform to map the language and sensorimotor functions intraoperatively.SurgeryThe patient was placed in a right lateral decubitus position for brain tumor excision surgery Figure . The aneFollow-upA two-month follow-up MRI showed an 8 mm residual tumor piece next to a cystic non-tumoral component Figure , which rBrain tumors are infrequent during pregnancy, with gliomas accounting for the majority of cases followed by meningiomas and acoustic neuromas , 3. A coBecause of these changes during pregnancy, the clinical presentation of tumors is usually exacerbated, which may precipitate obstetrical emergencies , 10. SigTreatment modalities during this life stage include medical management, radiotherapy, and surgery. Medical management refers to the use of chemotherapy and corticosteroids to alleviate cerebral edema, with the additional benefit of maturing the fetus’ lungs , 8. SurgAwake surgery allows optimization of the maximal extent of tumor resection in eloquent areas while the surgeon monitors the neurological function in real-time; thus, reducing postoperative deficits . UsuallyOverall, awake craniotomy during pregnancy is a reasonable treatment option for select patients with a stable gestational course and lesions compromising eloquent brain areas.This case study provides an example of the feasibility of performing an awake craniotomy on a patient in the second trimester of pregnancy with a brain tumor and deteriorating neurological function. In addition, we emphasized the importance of a multidisciplinary treatment approach to optimize maternal and fetal clinical outcomes. The main limitations are the fact that this is a single case and that some specific tests could not be performed due to the low-income patient's context. Given the paucity of literature regarding the safety and long-term outcomes of this approach in pregnant patients, further large-scale studies are necessary to elucidate its potential risks and benefits. Thus, such efforts could serve to refine clinical management strategies and improve the patient prognosis of the mother and fetus.


We present a distinctive case of solid pseudopapillary neoplasm as seen in a 44-year-old woman who presented with an abdominal mass but unremarkable labs with no elevation in any of the tumor markers. Her symptomatology ranged from typical symptoms suggestive of malignancy such as weight loss, lethargy, and anorexia to complaints like abdominal pain and jaundice. Prior to presenting at our center, she was given no hope or much in terms of treatment options. She was found to have a substantial mass over the body and tail of pancreas with characteristic and typical gross as well as histological features. Subsequently, she underwent a successful surgery and has found herself in remission since. Solid pseudopapillary tumor or solid pseudopapillary neoplasm of pancreas (SPN) is a rare entity. Its unique characteristics include occurrence in young females, uncertain histogenesis, borderline malignant potential, and excellent long-term prognosis, even when the initial presentation is aggressive. Its etiology and pathogenesis remain uncertain warranting discussion as more cases come to the fore due to the recent advancements in imaging and an increase in overall awareness and curiosity surrounding this tumor. Histopathological analysis remains the gold standard to reach a definitive diagnosis. Herein, we discuss the case of a 44-year-old woman who presented with an abdominal mass and was subsequently diagnosed with SPN.A 44-year-old obese woman, with no significant past medical history and otherwise in good health, presented with a left-sided abdominal mass that she had had for the past one year. She denied having experienced abdominal pain, nausea, vomiting, fullness, diarrhea, flushing, weight loss, anorexia, fatigue, jaundice, fever, or chills. She had undergone an exploratory laparotomy in Haiti a month earlier which was aborted. She was given the diagnosis of pancreatic cancer and told that there was little that could be done with respect to treatment. On physical examination, a mass was palpable in the left upper quadrant of her abdomen. Complete blood count and chemistry panels (including electrolytes and liver function tests) were in the normal range, except for a slightly elevated fasting blood glucose level of 103 mg/dl. The tumor markers were within the normal range with a carcinoembryonic antigen (CEA) level of 0.9 ng/ml and carbohydrate antigen 19-9 (CA 19-9) level of 5 U/ml. Computerized tomography Figures  demonstrThe mass impinged on the neighboring structures including the stomach and duodenum and abutted the left kidney and spleen. The mass measured approximately 13 by 13 cm in its greatest dimensions. There was neither any evidence of a disseminated disease nor of direct local invasion. Irregular heterogeneous enhancement of the irregularly thickened walls of multiple cysts and of the solid components of the mass was noted. No evidence of ascitic fluid or bowel obstruction was found, and no lymphadenopathy was observed. The case was discussed at a multidisciplinary tumor board, and the consensus was to proceed with resection without biopsy as further tissue sampling would not alter management but would risk tumor rupture and peritoneal dissemination.The patient was operated based on a presumptive diagnosis of SPN of the pancreas. She underwent an exploratory laparotomy with distal pancreatectomy and en-bloc splenectomy with extended left colectomy and primary colo-colonic anastomosis.Findings on gross examination  showed aOn microscopic examination , the tumβ-catenin  chromatin favor SPN. Also, the beta-catenin expression along with negativity for chromogranin goes in favor of SPN . Acinar One important question is predicting which patients may recur and what must be the appropriate follow-up after surgery. A study done by Serrano et al.  found thThe SPN remains a unique diagnosis with vague presenting symptoms and a poorly understood natural history of the disease. An enlarging abdominal mass is the most common presenting complaint, and surgery remains the mainstay treatment of choice. The etiology and the vulnerable predisposition of the female gender remain unclear warranting further research into understanding the mechanisms associated with the tumor. The prognosis is excellent with most patients showing no recurrence of the tumor postexcision. The rather perplexing nature of the neoplasm makes it a riveting case worthy of reporting.


An otherwise healthy 4-month-old girl presented to the community health service center because her abdomen was distended. Over the next 2 months, the girl's abdomen gradually became more distended. Her examination was notable for abdominal distention with a large, mobile, non-tender abdominal mass. Abdominal ultrasound images and subsequently obtained CT images showed a large, circumscribed cystic and solid mass. This led to the presumptive diagnosis of teratoma of the mesentery. The mass was completely resected during a laparotomy. The pathology, along with the surgical findings and imaging, led to the final diagnosis. Teratomas are common tumors in infancy, and they display a mixture of tissues from at least two of the three germ layers. The most common sites are the midline and gonads of the body, such as the sacrocaudal area 57%), gonadal area 29%), mediastinum (7%), and retroperitoneum (4%) . Mesente9%, media%, gonadaA 6-month-old girl was admitted to our hospital because of an abdominal mass. Approximately 2 months previously, the patient's parents had noticed that her abdomen had gradually become distended, but there were no other complaints. She was initially taken to the community health service center. On examination at the health service center, the patient appeared to be in normal condition. The patient's weight was 6.0 kg, the abdomen was mildly distended but soft, and there were normal bowel sounds and no apparent tenderness or mass on palpation. The examination concluded without any further diagnosis or prescribed treatment.The patient had been born by cesarean section because of preeclampsia after 38 weeks of gestation. She weighed 3.2 kg at birth and was fed breast milk. Supplements were added, and mixed feeding had begun when she was 5 months old. The patient's childhood immunizations were up to date. She had no known medical conditions, had not received any additional medications, and had no known allergies. She lived with her parents and grandparents. There was no family history of cancer.On examination at the hospital, the patient was placid. Her temperature was 36.6°C, pulse was 128 beats per minute, respiratory rate was 30 breaths per minute, and oxygen saturation was 98% while she was breathing ambient air. The patient's weight was 8.5 kg (89th percentile), her height was 69 cm (93rd percentile), and her head circumference was 42.0 cm (44th percentile). The abdomen was soft and distended with a circumference that measured 50 cm. On palpation, there was a large mobile non-tender abdominal mass that measured approximately 19 cm × 15 cm. There were no other abnormal physical findings. Blood levels of glucose, sodium, potassium, chloride, creatinine, total protein, albumin, globulin, and lactate dehydrogenase were normal, as were the results of coagulation and liver function tests. The other test results are given in An ultrasonography of the abdomen confirmed the presence of a mass in the patient's enterocoelia that measured 15.9 cm × 7.9 cm × 17.1 cm. The mass presented as a mixture of echoes and was well circumscribed; it mainly consisted of fat echo (multiple linear hyperechoic areas inside isoechoic areas) with many cysts of various sizes (the largest one measured 6.2 cm × 3.6 cm × 4.4 cm) and several hyperechoic areas with acoustic shadowing. The large retroperitoneal vessels were squeezed and shifted to the back of the patient, but they were not wrapped, and she did not exhibit any other abnormalities relating to any other organs.The ultrasonography indicated that the mass in the abdomen of the patient was significant in size and would be difficult to remove. Therefore, we needed to perform a computer tomography (CT) scan to clarify the origin of the mass and identify the surrounding vessels.The CT scan depicted a huge space-occupying lesion that appeared to be of mixed density, and the mass reached both the liver's lower edge and the pelvic cavity . The lesAbdominal masses found in children are associated with a broad differential diagnosis because of the various underlying causes; they may originate from one of several possible structures  , 16, andUsually, the common abdominal masses in this age group are neuroblastoma, mesenteric lymphangioma, retroperitoneal teratoma, and ovarian teratoma. Findings on the initial ultrasonography examination suggested that the mass was well circumscribed and had mixed echogenicity . The diagnosis of neuroblastoma was excluded because solid masses would have been present anywhere throughout the sympathetic nervous system, with the adrenal gland being the most common primary site. Using CT scans, the diagnosis was clarified because both cystic and solid components could be seen, including fat and calcified/ossific shadows. The diagnosis of mesenteric lymphangiomas was excluded because such tumors are cystic masses without calcification. The diagnosis of teratoma became evident. Therefore, the focus now turned to the origin of the teratoma. Although retroperitoneal teratomas are more common in children, a diagnosis of a retroperitoneal teratoma was excluded because the patient's ultrasonography (US) and CT indicated that the teratoma was located in the abdominal cavity. After the administration of intravenous contrast material, the supplying vessels were recognized as the branches of the superior mesenteric artery, which meant that the mass originated from the mesentery, and the diagnosis of an ovarian teratoma was excluded. Furthermore, the patient had normal ovaries and normal levels of sex hormones.Our initial impression was that this patient had a large, polycystic mass that seemed to be a mesenteric teratoma. Because mature teratomas are much more common than immature teratomas in infants, mature mesenteric teratomas were first considered in this patient.After an extensive discussion with her parents, a decision was made to proceed with a laparotomy. The patient had a preoperative diagnosis of a mesenteric teratoma that required complete resection.The abdomen was entered through a transverse upper abdominal incision, and a pineapple-sized multiple cystic mass was found . The supThe pathological diagnosis was a (Celiac) solid and cystic mature teratoma (WHO 0). The size of the mass was 14.5 cm × 12 cm × 11 cm. Most parts of the surface were covered with capsules, the nature of the surface ranged between dark purple and sallow, and the mass was smooth. In profile, it was solid with cysts of 0.5–5.5 cm in diameter. The capsule contained a bright viscous liquid, and the remaining solid area was sallow and felt greasy to the touch. Cartilaginous, adipose, glandular, and epithelial tissues were observed .The patient recovered well with no subsequent complications and was 9.5 kg (88th percentile) in weight and 73 cm (88th percentile) in height with an abdomen circumference of 49 cm. An ultrasonography revealed that there was no stenosis in the initial segment of the superior mesenteric artery and that blood flow was not restricted. The alpha-fetoprotein (AFP) level dropped to 4.65 ng/mL.The final diagnosis was a mature teratoma of the mesentery (WHO 0).Only 12 cases of mesenteric teratoma have been reported worldwide . Except Reviewing the history of this patient, we noted that she had asymptomatic abdominal distention when she was 4 months old. The patient's weight was 6 kg (29th percentile) at the first visit, and when compared with her birth weight of 3.2 kg (47th percentile), her weight gain was slower than that of infants of the same age. However, 2 months later, the patient's weight increased dramatically from 6 kg (29th percentile) to 8.5 kg (89th percentile), which meant that the weight gain rate significantly accelerated because of unknown factors. The patient's abdominal distention gradually became obvious at the same time, indicating that the mass was growing rapidly.In previous cases, all patients underwent ultrasonography, and the other imaging examinations were CT, x—ray, and magnetic resonance (MR). However, only three of these patients had been provisionally diagnosed with mesenteric teratoma before surgery. Two patients had been provisionally diagnosed with an ovarian mass and one other as having a tubercular mass. In the remaining patients, there was not sufficient information to determine the origin of the teratoma. While the diagnosis of teratoma can be relatively easy to establish through imaging findings such as ultrasound and CT , the origin of an abdominal teratoma is more difficult to determine. A mass located on the alimentary tract or related organs strongly suggests the diagnosis of gastrointestinal teratoma . The larThe treatment for mesenteric teratoma is surgery, and complete resection of the tumor is warranted. As teratomas always adhere to the root of the mesentery rather than to the distal mesentery, the supplying vessels are relatively independent. Thus, the tumor can be resected without interrupting the continuity of the intestine.Mesenteric teratomas are very rare teratomas that usually present initially with asymptomatic abdominal distension or digestive tract symptoms, and abdominal ultrasound is the first choice of examination.In infants, a circumscribed mass located on the alimentary tract or related organs with cystic and solid components, including fat, fluid, and calcified/ossific shadows, suggests mesenteric teratoma.All mesenteric teratomas should be surgically resected, and enhanced CT should be performed before surgery to clarify the vascular course and ensure that blood flow to the intestines is maintained.


As an invasive technique, selective venous sampling (SVS) is considered a useful method to identify a lesion’s location to increase the success rate of secondary surgery in patients with primary hyperparathyroidism (pHPT) caused by ectopic parathyroid adenomas.We present a case of post-surgical persistent hypercalcemia and elevated parathyroid hormone (PTH) levels in a 44-year-old woman with previously undetected parathyroid adenoma. An SVS was then performed for further localization of the adenoma, as other non-invasive methods showed negative results. After SVS, an ectopic adenoma was suspected in the sheath of the left carotid artery, previously considered as a schwannoma, and was pathologically confirmed after the second operation. Postoperatively, the patient’s symptoms disappeared and serum levels of PTH and calcium normalized.SVS can provide precise diagnosis and accurate positioning before re-operation in patients with pHPT. Primary hyperparathyroidism (pHPT) is a common endocrine disease caused by improper secretion of parathyroid hormone (PTH), leading to increased serum calcium levels . The cauHerein, we report a case of persistent hyperparathyroidism in a patient who continued to experience hypercalcemia after parathyroidectomy. After selective venous sampling (SVS), an ectopic parathyroid gland was finally identified. Subsequently, a second operation was performed and the mass was pathologically confirmed as an ectopic parathyroid adenoma.99mTc-Sestamibi scintigraphy (MIBI)  and a PTH level of 1396.5 pg/mL (reference range: 12.00–88.00 pg/mL). She was diagnosed with pHPT according to the guidelines for diagnosis and treatment by Marcella D Walker and Shonni J Silverberg [The patient was diagnosed with pHPT and was treated with intravenous hydration with 0.9% saline daily and diuretics. The mass and the tissue of the right lower pole of the thyroid gland were removed after operation. Intraoperative quick-frozen section showed the possibility of thyroid adenoma nodule. Meanwhile, intra-operative PTH level did not show significant decrease. Considering that frozen section analysis and intraoperative PTH measurement could not always provide the final definitive diagnosis and further neck exploration would cause unnecessarily extra damage and prolong the operation time, the operation was terminated after communicating with the family members by the surgeons at the local hospital. Finally, the paraffin-embedded tissue was showed to be a thyroid follicular adenoma with a small amount of parathyroid tissue on postoperative pathologic examination Fig. . After s2) of the lumbar spine (L1–L4), femoral neck, and hip were 0.731, 0.559, and 0.694, respectively. T-scores were -2.9, -2.6, and -2.0, respectively. US of the neck also suggested that the mass in the sheath of the left carotid artery might be a schwannoma ; a 4-cm–long, old, horizontal surgical scar could be seen on the neck, which was well healed. A mass was palpable behind the sternocleidomastoid muscle of the left neck, with smooth surface, clear borders, normal mobility and no tenderness. Laboratory investigations in our hospital showed hypercalcemia and elevated PTH level. In addition, the patient also had hypokalemia , increased urinary calcium excretion , and decreased urinary phosphorus excretion . Osteoporosis was defined by dual X-ray absorptiometry (DXA) (T score: -2.5 or below), and the bone mineral density (BMD) values  .In sum, US and MIBI are the most common localization methods for pHPT. It can increase the diagnostic rate up to 90% when neck US is combined with MIBI in patients with non-operative pHPT. However, in cases with negative or discordant diagnosis by non-invasive methods or in some persistent or recurrent cases after surgery when the sensitivity of the above methods decreases, further examination needs to be considered.Despite being an invasive technique, SVS has been considered a useful method . SVS is After SVS, the cystic solid mass in the sheath of the left carotid artery was highly suspected to be an ectopic parathyroid gland in our patient, which was negative by other non-invasive examination methods. The final pathological results revealed that it was an ectopic parathyroid adenoma after surgery. It is difficult to make a definitive diagnosis in some cases, and there may be false positives before surgery or insufficient exposure of the anterior cervical field during operation. These factors make it more necessary to perform an SVS examination before the operation. Intrathyroid parathyroid adenoma, a rather rare condition, is often difficult to be located non-invasively before surgery and is often missed as the parathyroid gland cannot be easily distinguished from thyroid nodules. Gofrit et al.  found thThe parathyroid glands are generally located near the thyroid gland, but there are exceptions; it is estimated that ectopic parathyroid glands may occur in 2% individuals . The ectIn our literature review, 60.98% (25/41) cases were in the mediastinum, 36.59% (15/41) were in the neck, and 2.44% (1/41) cases were in the right dome of the diaphragm. Of the 41 patients, only 39 showed postoperative pathology. Adenoma accounted for 87.18% (34/39), followed by 10.26% (4/39) hyperplasia and 2.56% (1/39) carcinoma. In all of these cases, the reasons for the failure of the previous operations were not described in detail or mentioned in the literature in 36 cases. The remaining five patients displayed negative US or MIBI results. In the population with persistent or recurrent pHPT, the possibility of ectopic parathyroid glands has been overlooked. Abnormally located glands often make it impossible for the surgeon to accurately locate the lesion, which accounts for 50% cases of unsuccessful parathyroid surgery.Moreover, taking into account the characteristics of cervical schwannoma, parathyroid adenoma in the neck should be differentiated from cervical schwannoma. The common non-specific symptom of schwannoma, which may also cause hypercalcemia, is an isolated, slow-growing mass in the neck , 58. PerImaging methods such as US, MRI, and CT can be used for identification. However, the diagnosis of this disease, to a certain degree, is usually non-specific. In this case, the P-pHPT after surgery and the mass in the sheath of the left carotid artery which should be differentiated from schwannoma and ectopic parathyroid adenoma significantly increase the difficulty of diagnosis and reoperation. The successful diagnosis and resection of the lesion in this patient strongly indicated that in patients with P-pHPT and suspected ectopic parathyroid adenoma or schwannoma, SVS should be conducted for precise diagnosis and lesion location before planning the second operation.Although reoperation is difficult and complex, choosing the right examination can improve the success rate of parathyroid reoperation. We report a patient with P-pHPT in whom SVS accurately identified the ectopic parathyroid gland, while conventional non-invasive imaging studies failed to determine the exact location of the lesion. In pHPT patients accompanied with possible thyroid follicular lesions, lesion location according to noninvasive methods should be done cautiously before surgery. Patients with P-pHPT who require a reoperation, especially when the lesion needs differentiation from ectopic parathyroid adenoma and schwannoma, should undergo SVS as it can provide precise diagnosis and accurate positioning before the second operation.


A 26-month-old boy presented with recurrent hematochezia. Abdominal examination was normal. Laboratory findings showed hemoglobin of 7.5 g/dL. Abdominal ultrasound (US) for intussusception revealed a short (0.3 cm) segment of wall thickening in the distal ileum and Meckel’s scan was negative. The patient underwent two additional Meckel’s scans, tagged red blood cell scan, CT-angiography, upper endoscopy and colonoscopy, and video capsule endoscopy, but had normal results. Superior mesenteric angiography was performed revealing an area of abnormal vasculature in the terminal ileum Fig. . SubsequMD is the most common congenital malformation of the gastrointestinal tract, occurring in approximately 2% of the population . The golA.P. was involved in conception and design, acquisition of data, drafting the work, final approval of the version to be published. M.R.A., P.M., T.R.B., and C.M.S. were involved in conception and design, critical revision of work, final approval of version to be published. L.A. was involved in conception and design, acquisition of data, critical revision of work, final approval of version to be published.


A Bochdalek hernia is a common congenital diaphragmatic defect in infants. A late presentation during adulthood is rare with misleading signs and symptoms, resulting in misdiagnosis and errors in treatment. We describe a 30-year-old man who presented with abdominal pain and chronic choking sensation, which was previously treated as peptic ulcer disease. During the present admission, radiological imaging performed revealed loops of bowel and a gastric volvulus in the left hemithorax. The patient underwent a successful emergency surgery and repair of a Bochdalek hernia. Due to its rarity and ambiguous presentation, a symptomatic Bochdalek hernia in an adult is commonly misdiagnosed. A comprehensive evaluation is pertinent for early diagnosis and treatment, to prevent complications arising from obstruction and strangulation of herniated intraabdominal contents.  A Bochdalek hernia is a transposition of abdominal organs into the thoracic cavity through a congenital defect in the closure of the pleuroperitoneal canal, located between the lateral  and posterior  components of the diaphragm. Most cases occur in the neonatal and postnatal period. Although it can present late during adulthood, this is extremely rare and is commonly misdiagnosed . Adult BA 30-year-old male patient presented with a 1-week history of intermittent fever, generalized abdominal pain, vomiting, and coughing. He had been complaining of multiple episodes of choking sensation for several years which was treated as peptic ulcer disease. His past medical and surgical history were unremarkable with no previous history of trauma. On examination, he was tachycardic with a low grade fever. There was reduced left sided chest expansion with reduced breath sounds on auscultation. Interestingly, bowel sounds were heard over the left chest. On palpation of the abdomen, the left hypochondrium was tender and guarded. A Computed Tomography (CT) scan of the thorax and abdomen was done and showed a large left posterolateral diaphragmatic defect with herniation of the stomach, mesenteric fats, spleen, pancreatic tail, and bowel into the left hemithorax with mesenteroaxial gastric volvulus . An explBochdalek hernia was first described in 1848 by the Czechoslovakian anatomist, Vincent Alexander Bochdalek . The majAs most Bochdalek hernia occurs on the left side, a large number presents with gastric herniation. Other organs involvement include colon, spleen, small, and large intestines. Increase in abdominal pressure such as chronic cough and choking can further aggravate the protrusion of the abdominal contents into the thoracic cavity with the occurrence of a gastric volvulus mainly due to laxity or absence of gastrohepatic, gastrocolic, gastrosplenic, and gastrophrenic ligaments .Radiological imaging, particularly a CT scan of the thorax and abdomen, is important in the diagnosis and exclusion of other differentials, enabling the identification of a diaphragmatic defect and a continuous density over and under the diaphragm's discontinuity to suggest a diaphragmatic hernia .The surgical approach depends on the presence of visceral complications. Most authors recommend the thoracic approach for elective cases; on the other hand, when there are complications, the abdominal approach is preferred. Minimally invasive techniques such as laparoscopy and thoracoscopy are gaining traction. In our case, an abdominal approach was employed because of the presence of a gastric volvulus and suspicion of peritonitis. A large diaphragmatic defect was visualized and in the absence of contamination, a decision was made for a mesh repair. Most defects can be closed primarily using non-absorbable sutures, however when a defect is large, a tension-free mesh is chosen to ensure a successful repair. As such, a primary closure would not have been possible in our case due to the size of the defect, necessitating the use of a mesh to provide a tension-free repair. Most patients with late-onset congenital diaphragmatic hernia (CDH) do well after surgical repair with apparently normal ipsilateral lung volume .CDH is an uncommon diagnosis among the adult population due to its misleading and non-specific signs and symptoms. A thorough clinical examination and radiological imaging are important for an accurate diagnosis and exclude other differentials. The findings of a gastric or intestinal gas shadow in the chest cavity on a radiograph warrants a CT scan for better delineation as the knowledge of an anatomic defect and ensuing complications are crucial to dictate the correct surgical approach and planning. This case demonstrates a rare presentation of Bochdalek hernia in an adult patient complicated with a gastric volvulus. Clinicians need to be aware of this rare but life-threatening clinical entity in order to secure a timely diagnosis and institute appropriate management.


Amyand's hernia is a rare type of inguinal hernia with an appendix inside an inguinal hernia sac. Most cases are diagnosed intraoperatively during hernia repair. A 66-year-old male was received at the Emergency Department with complaints of acute onset abdominal pain, vomiting, and groin swelling. The patient was diagnosed with obstructed left inguinoscrotal hernia with suspected bowel perforation. Following the emergency laparotomy, the intraoperative picture depicted a leftsided Amyand's hernia with a perforated caecum in the hernia sac. Mobile caecum, malrotation, situs inversus, and excessively long appendix denoted it to be the prime factors for the left-sided Amyand's hernia. A diverse range of pathological features and presentations might complicate the diagnosis and management of Amyand's hernia and all in all treatment has to be individualized according to the intraoperative finding. Most cases of Amyand's hernia are right-sided pertaining to the normal position of the appendix.2 Out of the handful of literature on left Amyand's hernia that is seldom encountered most of them are diagnosed intraoperatively during the management of incarcerated inguinal hernia and management of the case is usually determined by the pathology encountered. Preoperative diagnosis requires a high degree of clinical suspicion and imaging is the key. We, herein report a rare case of left-sided Amyand's hernia containing perforated caecum which is among a few scarce events ever encountered intraoperatively.Hernia containing vermiform appendix clarifies the diagnosis of Amyand's hernia.A 66-year-old male with a long-standing left-sided inguinal hernia was referred to our emergency with chief complaints of irreducible inguinoscrotal swelling for one day and increased pain abdomen since three hours. The pain was acute in onset, present in the left inguinoscrotal area, and dull aching associated with a few episodes of vomiting. On clinical examination he was vitally stable except blood pressure was 90/50 mm Hg in supine position on both arm and signs of dehydration was positive. Abdominal examination revealed a rigid abdomen with no bowel sound on auscultation. Local examination suggested a large irreducible left inguinoscrotal swelling reaching up to the base of the scrotum. Right-sided inguinoscrotal region seemed intact. Routine lab parameter was within normal limit except for raised serum urea and creatinine value. Lab investigation revealed the value of serum urea and serum creatinine as 95 mg/ dl and 4.5 mg/dl respectively. Abdominal X-ray was done and revealed gas under the diaphragm. Scrotal ultrasound depicted obstructed left inguinoscrotal hernia with dilated and aperistaltic bowel loop with minimum vascularity. At this stage, a provisional diagnosis of obstructed left inguinoscrotal hernia with perforated viscus and acute kidney injury was made. The patient was resuscitated with intravenous fluids, broad-spectrum antibiotics covering both anaerobic and aerobic organisms. The patient was then prepared for emergency laparotomy and proceeded with general anesthesia. Midline laparotomy incision was performed which was followed by the opening of the hernia sac. Appendix, omentum, and caecum were found during the surgery. Approximately, 4x4 cm irregular perforated margin was noted in the caecum along with an inguinal defect of 2 cm was visualized .th postoperative day. Vitals were within normal limits with no fresh complaints and laparotomy wound was healthy at the time of discharge. Patient was discharged on antibiotics and advised to follow up after 6 months for closure of stoma. After 6-month stoma was closed and subsequently normal bowel function was regained with no significant complications.Fecal peritonitis was also observed which was then followed by aseptic peritoneal lavage and drainage. Contents were reduced and then the primary closure of caecal perforation and appendectomy along with herniorrhaphy was done. Temporary diversion ileostomy was created for the better healing of sutured perforated caecum. The postoperative period was uncertain with gradual decline in serum creatinine and urea level. Patient's condition symptomatically improved over subsequent days of hospital stay and patient was discharged on the 14st appendectomy for an inflamed appendix during a herniotomy of an 11-year child.1 Amyand's hernia is mostly diagnosed on the right side with reference to the normal anatomical position of appendix. Only 0.1% cases of right-sided Amyand's hernia have appendiceal inflammation present.4 There is a handful of literature describing the leftsided Amyand's hernia.5 Our patient had left-sided Amyand's hernia with a perforated caecum which is an extremely rare encounter.Hernia by definition is a protrusion of the viscus or part of the viscus through an abnormal opening in the wall of its containing cavity. Appendix, when present within hernia sac meets the criteria for Amyand's hernia. Claudius Amyand Surgeon at St Georges hospital performed the 15 Mobile caecum was the most common clinical reason left behind Amyand's hernia in our patient. Appendix when found to be inflamed is usually due to adhesion causing compression of the appendix in the external ring. Obstruction caused by fecolith and lymphoid hyperplasia can also cause appendiceal inflammation in Amyand's hernia.Due to the relative paucity of cases and clinical presentation which is synonymous with incarcerated inguinal hernia and lack of adequate available literature exact pathophysiology, diagnostic criteria and management of Amyand's hernia is still lacking. Situs inversus, mobile caecum, mal-rotation and excessively long appendix are some of the theories purpose biased to the left sided Amyand's hernia.6 A great number of cases of Amyand's hernia present in the form of incarcerated inguinal hernia and for such cases the usual standard treatment modality is emergent surgical repair so most of them are diagnosed intraoperatively with only a few asymptomatic cases diagnosed preoperatively via diagnostic radio imaging such as CT scan.8Presentation of Amyand's hernia varies with some cases being asymptomatic while most presenting clinically as an obstructed or strangulated inguinal hernia.4 Most surgeons wouldn't recommend removing appendix however some authors suggest removing appendix as it has a high chance of herniation and subsequent appendicitis.3 Losanoff and Basson have proposed a widely accepted initial tool for grading the management of Amyand's hernia into four categories.9 Losanoff and Basson grading proves to be a very useful guidance tool for the management of wide range of anomalies and pathology encountered in Amyand's hernia. However, in our patient, the intra-operative finding was caecal perforation with peritonitis and a non-inflamed appendix. So it categorically did not fall into any of the Losanoff types. Although the amount of literature pertaining to Amyand's hernia is found on increasing trend but to the author's knowledge this is among few rare cases of left Amyand's hernia with caecal perforation which was followed by caecal repair, appendectomy, and herniorrhaphy.Our patient presented with clinical features similar to strangulated inguinal hernia and rigidity of abdomen led to suspicion of perforated bowel content, henceforth management of the case proceeded accordingly. As in most cases, the diagnosis of Amyand's hernia containing perforated caecum was made intraoperatively. Usually, the management of Amyand's hernia is based on the contents, presence of appendix, and peritoneal inflammation. The appendix, when found to be inflamed, is to be removed and the mesh is avoided in case of peritonitis.This case signifies how diverse variation is there in amyand's hernia and sometimes widely accepted tool for management of amyand's hernia might not be completely helpful in the proper treatment of individual variation encountered intraoperatively and the treatment has to be individualized for each patient. It also raises the awareness for surgeons to have high degree of suspicion for potential involvement of caecum. Limitations associated with this case was that the management protocol followed in our patient might not be same in other cases and the treatment guideline followed here could not be generalized for other individuals.


The vitello-intestinal duct normally regresses with the development. But, in certain cases, it could persist and present as Meckel's diverticulum. Here we report a case of an eight-year-old boy presenting with peri-umbilical abdominal pain, vomiting and loose stool. He was initially diagnosed and managed as acute appendicitis but did not improve, rather developed features of intestinal obstruction. Exploratory laparotomy was done which revealed persistent vitello-intestinal duct and was managed surgically. This case report highlights that in any case of suspected acute appendicitis, the complications of persistent vitello-intestinal duct should be considered as one of the differentials. This communication is supposed to be obliterated in between fifth to the seventh week of gestation;In this case, an eight-year-old boy presented to our pediatric emergency with complaints of abdominal pain and multiple episodes of vomiting and passage of loose stools for one day. The pain was periumbilical and spasmodic in nature at presentation. The pain was sudden in onset, non-radiating, aggravated on attempting to eat anything, was not relieved by non-steroidal anti-inflammatory drugs, and was severe enough to bring the boy crying and wriggling in pain. The pain gradually migrated to the right iliac fossa. Along with severe abdominal pain, he also had multiple episodes of vomiting, which was non-projectile and contained food particles, bilious, but not mixed with blood. He also suffered from multiple episodes of watery loose stool, not mixed with blood, for the same duration and pain was not relieved with passage of stool.On examination, the boy was ill-looking, well oriented to time, place and person. The general examination of the boy was normal. His heart rate was 88 beats per minute, blood pressure was 110/80 mm of Hg, respiratory rate was 22 breaths per minute, temperature was 36.1°Celsius. On abdominal examination, guarding, right iliac fossa tenderness, and rebound tenderness was present.3; and ultrasonography (USG) was not suggestive of anything except mild probe tenderness in right iliac fossa. Abdominal X-ray was not laden with any findings either during initial set of investigations. His 'MANTRELS'  was calculated to be eight. With this clinical presentation in the emergency, the boy was rushed for an emergency open appendectomy.Baseline investigations were sent and his initial management was done in the emergency. His total leukocyte count was found to be 19,200/mmIntraoperatively, the tip of the appendix, which was retrocecal in this patient, was found to be inflamed; but the base was healthy. There was around 20 ml of serous peri-appendiceal collection. Appendectomy was done but the patient did not get better after the procedure. Instead, his condition started worsening. Meanwhile, conservative management was being done but showed no signs of improvement. He had not passed either stool or flatus even till the second postoperative day. Further, his pulse rate was climbing over 110 per minute, blood pressure was 110/80 mm of Hg, respiratory rate was 25 breaths per minute, and his temperature was 37.1° Celsius. This is when an abdominal X-ray (supine and erect views) was done which revealed signs of intestinal obstruction .He was rushed in for emergency exploratory laparotomy as his condition worsened clinically.Incision was given through the same incision made for appendectomy. The skin at the site of incision was healthy. The incision was extended. The appendectomy stump appeared healthy. Further exploration revealed a closed loop obstruction of a segment of ileum .The obstruction had taken place due to a patent vitello-intestinal duct that was attached to the abdominal wall and the loop of ileum took a twist around this persistent anomaly. Resection of the vitello-intestinal duct along with the adjoining ileal segment (wedge resection), 20 cm proximal to the ileocecal junction was done .An end-to-end ileal anastomosis was fashioned and the patency of anastomosis was ensured .A 16F drain was kept, thorough lavage was done, and the abdomen was closed in layers in half an hour.The postoperative period was uneventful and he recovered quite well from two surgeries without any postoperative complications. Upon follow-up after two months, he had recovered without any new issues.4 Bowel obstruction manifests itself with classic features of abdominal pain, nausea, vomiting, obstipation and distension, with variations in its presentation depending on the site of obstruction. Among the multitude of patients presenting every day to different emergencies across the globe, closed loop obstructions forms a small minority of cases and is a result of internal hernias, congenital bands, postoperative adhesions and malrotation. A closed loop obstruction is a type of intestinal obstruction in which the bowel is nipped off both distally and proximally, such that a segment of either the small or the large bowel is closed from both ends leading to the obstruction. It should be treated as a of surgical emergency.5One of the presentations of the persistent vitello-intestinal duct is bowel obstruction.Our patient had developed a closed loop obstruction of a segment of ileum secondary to its twisting around the persistent vitello-intestinal duct, leading to dilated bowel loops proximal to the obstruction. The interesting thing about this case is that despite being a bowel obstruction, it initially presented with clinical manifestations pointing towards acute appendicitis.6 When a patient presents with these symptoms, and the demographic is also compelling, it becomes a knee-jerk reaction to think of acute appendicitis, and this bias can lead to blunders, which occurred in the case. And intraoperatively, as we saw the inflamed tip of appendix and peri-appendiceal fluid we went along with the surgery, never suspecting that it could be something else as well, and looked on for other causes only after the patient did not improve with given management.The pain in acute appendicitis, in its classic form would begin in the periumbilical area to later become localized to the right lower quadrant.7 However, the diagnosis is not always straightforward, especially in case of a virgin abdomen . There can be diagnostic difficulties based on the clinical presentation alone, which happened in our case where we suspected acute appendicitis and operated for the same. Further, plain x-ray of abdomen and USG also do not confer specific signs for small bowel obstruction, especially early on, which happened in our case too.5Early and prompt diagnosis of this condition is essential as the risk of bowel ischemia is particularly high in closed loop obstructions wherein the bowel is being pinched both proximally and distally.8 Complete or high-grade obstructions, closed loop obstructions, call for urgent or emergent surgical interventions as the risk of ischemia increases. There is high morbidity and mortality if the diagnosis is delayed.9 Hence, emergency laparotomy should be done promptly. Also, some centers mandate laparotomy in cases of virgin abdomen.10 Treatment is guided by the patient's clinical condition, and in our case, the boy had started worsening despite us misinterpreting that we had treated the patient, and he was rushed in for exploratory laparotomy wherein we found the obstruction due to the vitello-intestinal duct, and it was managed via wedge resection of duct and end to end ileal anastomosis.The management of bowel obstruction is aimed at correction of physiological derangements, proper bowel rest and decompression, and eliminating the source of obstruction. Non-operative management should be tried in case of adhesive bowel obstruction without the signs of peritonitis, strangulation or hernia.We should always keep our mind and eyes open, despite the presentation and management being seemingly obvious as it is not always what it appears to be in the first glance. A persistent vitello-intestinal duct should be kept as a differential in patient's presenting with closed loop obstruction. The management of these cases should be prompt with wedge resection and anastomosis.


Chondrosarcoma is a rare form of carcinoma that originates in the cells of cartilage, the flexible tissue that cushions the joints and gives structure to various parts of the body. This malignant tumour primarily affects adults and is most commonly found in the bones of the arms, legs, pelvis, and ribs. The severity and prognosis of chondrosarcoma can vary widely depending on factors such as tumour size, location, and grade. We are reporting a case of an 83-year-old male patient who presented with swelling over the left hip joint. A mass was detected on radiograph and ultrasound that was further characterized using contrast-enhanced CT and MRI. Imaging findings suggested chondrosarcoma. The patient was diagnosed on histopathological examination. Chondrosarcomas comprise around 10-15% of malignant bone tumours, exhibiting slow growth and a higher occurrence in regions like the femur, humerus, pelvis, scapula, and ribs [An 83-year-old male patient presented with swelling over the left hip and restricted joint movement in the last six months. The swelling was associated with pain. There was no history of trauma. On local examination, the swelling was soft, non-tender, and firm in consistency measuring 10 x 14 cm. No local rise in temperature or discolouration was noted.On plain radiograph, an ill-defined, large lobulated soft tissue density lesion with permeative bone destruction was seen. Calcific foci were noted within it giving a heterogenous appearance suggestive of matrix mineralisation Figure .On high-frequency ultrasound, a large heterogeneous lesion with flex of multiple calcific foci was noted in the left iliac region. Some cystic changes were noted within it. The lesion showed no vascularity on colour Doppler Figure .On CT, an ill-defined soft tissue mass measuring 18 x 13.2 x 11.5 cm encased the iliac blade on the left side, extending from the left hip to the left paraspinal muscles. There was a mixture of hyperdense and hypodense areas noted reflecting the heterogeneous nature of the tumour. Central and peripheral calcifications were noted within the tumour , which revealed clusters of monomorphic cells and a few scattered large cells. The cells were bland with abundant eosinophilic and vacuolated cytoplasm. Few cells showed mild anisonucleiosis and binucleation. The background showed myxoid material; however, mitosis and osteoclastic giant cells were not seen. It depicted low-grade chondrosarcoma Figure .Chondrosarcoma, a malignancy generating cartilage matrix, presents as primary (de novo) or secondary (superimposed on benign cartilaginous neoplasms). Categorized by osseous location, it encompasses central and peripheral types, the latter further divided into those arising from a preexisting osteochondroma and those developing on the bone surface  . In descConventional intramedullary chondrosarcoma, commonly termed central chondrosarcoma, constitutes the predominant primary subtype. Clinical manifestations are vague, with pain as the prevailing symptom in over 95% of cases. This pain, often gradual and worsening at night, persists for months to years prior to diagnosis. A palpable soft-tissue mass or swelling has been noted in 28-82% of individuals [Plain films serve to depict and locate lesions, revealing their cartilaginous nature and aggressiveness. Central chondrosarcoma, the prevalent type, originates in the metaphysis and extends to the diaphysis. The lytic lesion displays well-defined characteristics, often with mixed lytic and sclerotic features, including characteristic ring-and-arc calcification patterns, endosteal scalloping, cortical changes, and irregular margins in high-grade cases. Calcifications within the tumour matrix vary in pattern, presence, and density. When invading soft tissue, the mass becomes palpable . UltrasoCT imaging offers excellent matrix mineralization detection, crucial for intricate anatomy or subtle cases. Distinct matrix mineralization patterns are widespread within the lesion. Three-dimensional CT imaging assists in assessing endosteal scalloping depth, showing long bone chondrosarcoma, and exhibiting significant scalloping. Lobulated endosteal scalloping and cortical response are typical in chondrosarcomas. More the soft tissue involvement, higher will be the grade on histopathology. CT with contrast showcases mild enhancement, while higher-grade lesions exhibit increased attenuation and pronounced contrast enhancement due to cellularity. MRI is highly effective for assessing marrow involvement by conventional intramedullary chondrosarcoma. T1-weighted images depict marrow replacement as low-intermediate signal intensity, revealing entrapped yellow marrow. The lobular architecture is evident, especially at the margin, and non-mineralized chondrosarcoma components exhibit high signals on T2-weighted images. Matrix mineralization, seen on MRI, appears as a low signal. Peritumoral oedema, detected through water-sensitive sequences, aids in distinguishing intramedullary chondrosarcoma from enchondroma .Unlike the prevalence of chondrosarcoma in long bones, the pelvis is a common site for chondrosarcoma. Pelvic chondrosarcomas frequently affect the ileum, with a notable inclination toward the triradiate cartilage region. Due to delayed symptom onset, these lesions are often large at initial assessment. Paradoxically, though sizable, radiographs may show subtle abnormalities due to pelvic anatomy complexity .There are various types of primary chondrosarcoma, including conventional intramedullary, clear cell, juxtacortical, myxoid, mesenchymal, extraskeletal, and dedifferentiated. These are differentiated by histopathologic appearance . HoweverChondrosarcoma, a rare malignancy arising from cartilage cells, exhibits diverse presentations and complex imaging characteristics. Diagnostic modalities, including radiography, CT, and MRI, play pivotal roles in identifying its features and extent. Comprehensive understanding aids in accurate diagnosis and effective management.


Palisaded encapsulated neuroma (PEN) is generally seen in the head and neck area as an asymptomatic nodule with the same color as the surrounding skin and rarely occurs in the oral cavity. The exact etiology of PEN is not known, but there is evidence supporting the role of trauma as its etiological factor.Palisaded encapsulated neuroma (PEN) is one of the benign nerve sheath tumors of Schwann cell origin, which is commonly found in the skin of the head and neck area, and rarely occurs in the oral cavity. Its exact etiology is unknown, but there is evidence that supports the role of trauma as an etiological factor. Here we present a case of PEN in the hard palate of a 30‐year‐old patient and review the differential diagnoses of these nerve sheath tumors of the oral cavity. PEN is a benign tumor of neural origin, a diagnosis seldom suspected clinically. In the clinical examination, an exophytic mass with a diameter of about 1 cm with a smooth and telangiectatic surface was observed  was first introduced as a benign neuroma in 1972 by Reed et al.Soft tissue oral cavity masses are often a diagnostic challenge because a diverse group of pathological processes can cause such lesions. Oral nerve tumors may be neoplastic or reactive in nature. These tumors consist of specific parts of peripheral nerves including endoneurium perineurium and epineurium.PEN is of the least common nerve tumors of the oral cavity and is often mistaken for schwannoma in microscopic view. Its histological variants include classic, lobular (the most common form), plexiform, and multilobular, which are clinically and biologically insignificant.This lesion can be classified as classic or lobular, which is a more common histological variation, or plexiform, (fast growth rate like a mushroom), fungating, and multilobular. The cells are spindle‐shaped and arranged together in sheets, which indicates a palisading tendency. These sheets may bend immediately below the epithelium, together with the connective tissue. A specific finding is excessive artificial clefting as a result of tissue shrinkage. The stroma contains collagen and sometimes mucoid, and mast cells may be present in some lesions. Cells are positive for s100 and are always negative for GFAP. EMA in peritumor tissues non‐consistently stains not more than one cell layer. Some tumoral cells are also positive for CD34.The most common oral nerve tumors are neurofibroma (NF), traumatic neuroma (TN), schwannoma (SCH) or neurilemoma, and palisaded encapsulated neuroma (PEN), which are included in the differential diagnosis of PEN, and differentiating these lesions is a diagnostic challenge.Neurofibroma is less common than schwannoma in the head and neck area, but the opposite is true inside the mouth. Neurofibroma is usually observed in the palate, gums, and tongue, and in the clinical view, is seen as isolated small sessile nodules or, with less prevalence, as multiple asymptomatic nodules that are covered with normal‐appearing mucosa.In the histopathological view, nerve axons within the tumor stroma can be observed with silver staining. Cells are S100 positive in neurofibroma (Schwann cells), but not as much as in schwannoma. Some of them are also positive for epithelial membrane antigen (EMA) , CD34 , Collagen IV , and CD68 (putative resident macrophages).The most common site of schwannoma is the head and neck region, but it is not so common in the oral cavity. Schwannoma is the third or fourth most common oral nerve tumor. It usually appears as a peripheral lesion, but sometimes it can occur inside the maxillary bone. The most common site of schwannomas manifestation includes the lips, especially the lower lip, as well as the buccal mucosa. Most cases are asymptomatic, but pain is reported in up to one‐third of cases. In rare cases, difficulty in swallowing, speaking, breathing, bleeding, macroglossia, or loss of sensation and taste may be reported by patients.Microscopically, conventional schwannomas are usually encapsulated and show cell proliferation in two different histological patterns and the most common one is Anthony A, which is characterized by spindle‐shaped cells surrounded by acellular eosinophilic areas, also known as Verocay bodies, and Antony B with cells and less organization, where the spindle cells are randomly placed in a loose myxomatous substrate.Traumatic neuroma (TN) is a reactive lesion and indicates a high growth response in response to nerve damage. Most TNs are peripheral, and only a small number of intraosseous traumatic neuromas have been reported in the English literature. TN usually manifests as an ulcerated nodule with a smooth surface on the tongue, mental foramen, and lip mucosa. Oral TNs are painful in 25%–30% of cases.4Although neural neoplasms are uncommon in the oral cavity, these lesions should be considered in the differential diagnosis of oral masses, and a definitive diagnosis requires confirmation by histopathological and immunohistochemical examinations.Sara Pourshahidi: Conceptualization; project administration; supervision; writing – review and editing. Pouyan Aminishakib: Data curation; formal analysis; investigation. Negin Aliyari: Data curation; writing – original draft. Kimia HafeziMotlagh: Conceptualization; data curation; writing – original draft; writing – review and editing.The study was not funded.The authors declare that the research was conducted without any commercial or financial relationships construed as a potential conflict of interest.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


Upstream infection with vaginal flora can develop into tubal endothelial damage and tubal edema, which can lead to tubal obstruction and fallopian tube abscess if left untreated. Fallopian tube abscess in adolescent virgins is very rare, it may lead to long-term or even lifelong complications once it occurred.A 12-year-old adolescent virgin with no history of sexual intercourse and previous physical fitness who presented with lower abdominal pain with nausea and vomiting for 22 h, body temperature up to 39.2 °C. Laparoscopic surgery revealed an abscess in the left fallopian tube, the left fallopian tube was surgically removed, successfully treated, and the pus was cultured for escherichia coli.It is important to consider possibility of tubal infection in young. Fallopian tube abscess is a serious consequence of pelvic inflammatory disease, often due to upstream infection with vaginal flora cause tubal infection which can lead to tubal obstruction and fallopian tube abscess if left untreated . FallopiNeisseria gonorrhea, and Chlamydia trachomatis and a variety of obligate anaerobic bacteria as commonly associated microorganisms. [Adolescence is the transitional period of development from the childhood stage to the adult stage, usually between the ages of 10 and 18 for girls, and the occurrence of pelvic inflammatory disease and fallopian tube abscess is very rare during this period if there is no history of sexual intercourse and is less frequently reported in the literature. Once they occur they may lead to long-term or even lifelong complications such as pelvic adhesions, chronic pelvic pain, infertility and ectopic pregnancy . It is rganisms. .9/L, neutrophil percentage of 87.4%, and C-reactive protein (CRP) of 28.73 mg/L, all were above normal. The body temperature was 36.7 °C, pulse 128/min, respiration 25/min, blood pressure125/79mmHg, abdomen was flat, drum sound on percussion of the abdomen, lower abdomen was tense, there was tenderness on palpation, rebound tenderness was.A 12-year-old adolescent virgin with no sexual intercourse had lower abdominal pain with nausea and vomiting for 22 h, with persistent pain. Vomit is digestive fluid and food residue, and the lower abdominal pain was not significantly relieved after vomiting, body temperature is normal. Visit pediatric department, the transabdominal pelvic ultrasound was performed :A 9.7 × 3.8 cm cystic anechoic area with multiple band-like separations and a grid-like distribution was seen in the posterior left side of the uterus  was 4.88 ng/ml, erythrocyte sedimentation rate (ESR) was 60 mm/h, all were above normal. In combination with gynecologic ultrasound, pelvic abscess and ovarian cyst torsion combined with infection were not excluded. She was treated with intravenous cefoperazone sodium and metronidazole.Body temperature gradually increased to 38 °C during the patient’s transfer to the gynecology department and the repeated transabdominal pelvic ultrasound was performed: The tortuous tubular anechoic area, about 7.2 × 4.8 cm in extent, with a thick and rough wall and poor internal translucency was seen in the posterior left side of the uterus Fig. -b. AfterGeneral surgery consultation was requested and pelvic enhancement computed tomography (CT) was recommended. Since pelvic enhancement CT could not be performed during nighttime, the patient was considered to have significant acute abdomen and fever, and laparoscopic exploration was performed urgently. Laparoscopic examination showed that the anterior uterus and intestinal space were covered with pus and blood fluid, amounting to about 100 ml, and the intestines and large omentum were densely adherent to the posterior wall of the uterus and bilateral adnexa, and the Douglas fossa was completely closedFig. -a, and pAfter the operation, the body temperature dropped rapidly to normal, and the patient was treated with two intravenous antibiotics: cefoperazone sodium for 12 days at a dose of 1 g/dose every 8 h, and metronidazole for 5 days at a dose of 100 ml/dose every 8 h. The postoperative course was stable, and WBC count, neutrophil percentage, CRP and PCT all returned to within the normal range of fallopian tube abscesses in adolescent virgins included 14 cases (Table 022 of faIn conclusion, It is important to consider possibility of tubal infection in young women with acute abdomen and fever, even if they have never had sexual relations, although there are no high-risk factors. E. coli may be the causative agent, and early and timely diagnosis and treatment are essential to prevent future sequelae.


A few cases of small omphalocele with umbilical evagination of the bladder have been reported. However, its embryology is yet to be elucidated. Only a few reports have indicated the existence of urachal anomalies and umbilical cysts related to bladder evagination. The incidence of urachal anomalies at birth is reported to be 1 in 5000–8000 live birth, and urachal aplasia is rare. Herein, we report a rare, novel case of urachal aplasia.We encountered a small omphalocele with bladder evagination associated with urachal aplasia for which the neonate underwent surgery one day after birth. The patient was a one-day-old boy with a prenatally diagnosed omphalocele. A fetal magnetic resonance image (MRI) scan (25 weeks of gestation) revealed a 30 × 33 mm (approximately 1.3 in.) cystic lesion which was suspected to be an umbilical cyst. The baby was born vaginally at 38 weeks, weighing 2956 g. An omphalocele  with bladder prolapse was recognized. After sac excision, the prolapsed bladder was resected and closed with two-layer sutures. In order to secure sufficient bladder capacity, we estimated the minimum residual volume as 21 ml after bladder plasty. The remaining bladder capacity was confirmed to be 30 ml by injecting a contrast dye and saline into the bladder. The neonate had no associated cardiac urogenital or skeletal anomalies. Postoperative course was uneventful. The patient was regularly followed up for two years after surgery and underwent umbilicoplasty. He had no trouble with urinary function.In this case, we experienced extremely rare condition of a small omphalocele with bladder evagination associated with urachal aplasia and reviewed 7 case reports of anomalies similar to those in the present case. Umbilical cord cysts may be an informative indicator of these symptoms in utero. Therefore, ultrasonography scans should be conducted until delivery, despite the spontaneous disappearance of cord cysts. The urachal anomalies occurrence at birth is reported to be 1 in 5000–8000 live birth, and urachal aplasia is extremely rare. Only a few reports have indicated the existence of urachal anomalies and umbilical cysts related to bladder evagination [The patient was a one-day-old boy with a prenatally diagnosed omphalocele. A 34-year-old pregnant mother was referred to our hospital at 17 weeks (approximately 4 months) of gestation with the chief concern of a singleton fetus with a 30 mm (approximately 1.18 in.) cystic lesion near the umbilical cord base. A fetal magnetic resonance image (MRI) scan (25 weeks of gestation) revealed a 30 × 33 mm (approximately 1.3 in.) cystic lesion as shown in Fig. Under general anesthesia, the patient was placed in supine position. After hernia sac excision, the intestine was naturally reducible and there were no other structural anomalies. In order to secure sufficient bladder volume, we estimated the minimum residual volume as 21 ml after bladder plasty. For bladder capacity, the simplified formula: Capacity (mL) = 7 × weight (kg) was a reliable estimate of expected bladder capacity in infants independent of age [Postoperative course was uneventful. He was regularly followed up for 2 years after surgery Fig. a, and unHere, we experienced and defined present case a small omphalocele with bladder evagination as a prolapsed bladder at the umbilicus with no significant abdominal wall defects and coexisting anomalies; the pelvic ring is intact and the genitalia are normal, which makes it distinct from bladder exstrophy. We reviewed seven cases of small omphaloceles with bladder evagination as shown in Table The urachus is a structure lying between the peritoneum and the transversalis fascia, stretching from the anterior dome of the bladder to the umbilicus, located between the umbilical ligaments. A patent urachus is a complete connection between the bladder and umbilicus with a fully open ureteral duct which sometimes causes bladder prolapse. However, in the present case, not only the bladder but also the small intestine prolapsed from the umbilical cord. Furthermore, all the tissue of resected specimen submitted for pathological diagnosis revealed the bladder wall. Results of pathological examination indicated that the bladder did not prolapse through the urachus remnant, but through the umbilical cord . From thRegarding the treatment after birth, umbilical cord hernia is the primary surgical indication, and surgical repair would be required in all cases. In our present case, the patient underwent surgical procedures of umbilical cord hernia repair, vesicourethral fistula resection, and bladder repair one day after birth. To determine the resection line of vesicourethral fistula resection, the sufficient bladder capacity should be left intact. However, the bladder should be resected because of the risk of carcinogenesis transformation from the residual ureteral duct tissue. Urachal masses are rare, but have the potential characteristics for malignant diseases. Thus, minimally invasive diagnostic and treatment strategy are strongly recommended to prevent the malignant transformation of large cysts in the early stages . In our Several authors suggest that systematic excision of urachal lesions detected during childhood should be performed to prevent infection or other problems in adulthood, such as the development of malignancy in residual remnants . Of adulIn the present case, the specimen did not show urachal histology. However, if an urachal anomaly is not resected in childhood, the pediatric surgeon and patient should keep in mind regarding the potential future risk of malignancy and the need for lifelong screening. If bladder capacity cannot be secured at the time of initial surgery, the augmentation cystoplasty using stomach and small intestine should be considered in the future. Augmentation cystoplasty is a surgical intervention that can significantly and continuously improve the quality of life in children. However, careful patient selection and appropriate preoperative counseling are essential, given the potential complications of urinary leakage and urinary tract stone disease and the fact that augmentation cystoplasty is an independent risk factor for bladder malignancy .Regarding the design of the umbilical ring, a new ring was formed on the caudal side, with the umbilical artery and cord remaining in the center, which was expected to be retracted and recessed in the future . Here, tWe experienced extremely rare case of a small omphalocele with umbilical evagination of the bladder caused by an umbilical cord cyst combined with urachal aplasia. Umbilical cord cysts may be an informative indicator of these symptoms before delivery. Therefore, ultrasonography scans should be conducted until delivery, despite the spontaneous disappearance of cord cysts. Furthermore, umbilical cord hernia is the primary surgical indication and we recommend to determine the resection line after confirming a bladder capacity of over 7 × weight (kg) in infants.


Splanchnic vein thrombosis due to co-existing metastatic pancreatic neuroendocrine tumour (pNET) and JAK2V617F mutation is a rare condition.Here we present a case of a young woman with complete remission of a non-functioning grade 2 pNET with unresectable liver metastases, coexisting with JAK2V617F mutation. Splenectomy and distal pancreatectomy were performed. Neither surgical removal, nor radiofrequency ablation of the liver metastases was possible. Therefore, somatostatin analogue (SSA) and enoxaparine were started. Peptide receptor radionuclide therapy (PRRT) was given in 3 cycles 6-8 weeks apart. Genetic testing revealed no multiple endocrine neoplasia type 1 (MEN-1) gene mutations. After shared decision making with the patient, she gave birth to two healthy children, currently 2 and 4 years old. On pregnancy confirmation, SSA treatment was interrupted and resumed after each delivery. Ten years after the diagnosis of pNET, no tumour is detectable by MRI or somatostatin receptor scintigraphy. PRRT followed by continuous SSA therapy, interrupted only during pregnancies, resulted in complete remission and enabled the patient to complete two successful pregnancies. Splanchnic vein thrombosis (SVT), including portal vein, splenic vein, mesenteric vein thrombosis and the Budd-Chiari syndrome, is a manifestation of unusual-site venous thromboembolism. All may present with uncharacteristic epigastrial pain. Predisposing factors are liver cirrhosis, myeloproliferative neoplasms, liver and pancreatic malignancies, factor V Leiden mutation, protein C or protein S deficiency, microparticles, JAK2V617F mutation, and methylenetetrahydrofolate reductase (MTHFR) C677T and A1298C polymorphisms. The association between solid cancers and SVT can be explained by cancer-related haemostatic system alterations. In addition, there are published data on the association of MEN1 syndrome with portal or splenic vein thrombosis  is characterized by thrombocytosis and thromboembolic complications. More than half of ET patients are JAK2V617F mutation-positive. According to our present knowledge, JAK2 mutation has been an independent factor for thromboembolic events; however, the precise mechanism remains unknown . Most ETUp to 2% of all pancreatic tumours are neuroendocrine tumours  which caIn Europe and in the USA the incidence of gastroenteropancreatic (GEP) tumours has increased over the last four decades from 0.27 to 1.00 per 100,000 individuals . UnfortuWritten informed consent for publication of the clinical data and images was obtained from the patient.In January 2011, a 25-year-old woman was admitted to our gastroenterology unit due to recurrent epigastric pain. Gastroscopy confirmed reflux esophagitis, gastric varices and gastritis. Contrast-enhanced abdominal computed tomography (CT) demonstrated splenomegaly, splenic infarction, portal and splenic vein thrombosis, and an 85x50 mm inhomogeneous necrotic lesion located between the stomach, spleen and left kidney, adjacent to the pancreas. Color Doppler ultrasonography of the abdominal vessels showed massive portal and splenic vein thrombosis. Anticoagulation treatment was immediately started. Low-molecular-weight heparin was followed by warfarin, later switched to rivaroxaban. In April 2011 bone marrow immunophenotyping by flow cytometry did not find any abnormality and chromosome analysis did not detect clonal chromosome aberration. The enlarged spleen was considered a consequence of splenic vein thrombosis. The inhomogeneous necrotic lesion in the left abdomen was assumed to be caused by thrombus in the splenic vein therefore biopsy was not performed. During regular control visits white blood cell count and hemoglobin levels were in the normal range.While JAK2 V617F mutation was detected, the presence of FV Leiden mutation, prothrombin 20210A allele, lupus anticoagulant, antiphospholipid syndrome and the deficiency of antithrombin-III, protein C or protein S were excluded. The patient neither used oral contraceptives nor was a smoker.Three years later, in 2014, while on rivaroxaban, the patient was admitted to the Emergency Department for upper left abdominal pain. Physical examination showed extreme splenomegaly, without hepatomegaly. Abdominal ultrasound found splenomegaly of 7.5 x 23 cm in addition to the previously diagnosed portal vein thrombosis and dilatation of splenic vein. Platelet count was 121 G/L Figure 1Splenectomy was performed due to the increased risk of splenic rupture. Histopathology of the spleen revealed numerous necrotic neuroendocrine tumour foci with vascular and splenic vein invasion. By immunohistochemistry, tumour cells were neuron-specific enolase (NSE), chromogranin, cytokeratin 7 (CK7) and pan-CK positive. The MIB-1 proliferation index was 5%. There were no symptoms of hormone overproduction.After splenectomy, the platelet count increased to 2107 G/L, mainly due to reactive thrombocytosis but the possibility of ET could not be clearly ruled out. Interferon therapy was not covered by insurance in Hungary, therefore, hydroxycarbamide was applied for rapid cytoreduction. At that time, the patient was not yet married and did not plan pregnancy. Enoxaparine and allopurinol were added. Platelet count gradually decreased to the normal range within a year.18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) demonstrated a 13 cm, irregular soft tissue mass with intensive metabolism in the pancreas. Serum chromogranine-A (CgA) level was >1150 µg/L (reference range: 20-100 µg/L). Somatostatin receptor scintigraphy (SRS) single-photon emission computed tomography/computed tomography (SPECT/CT) using 400MBq 99mTc-EDDA/HYNIC-Tyr3-Octreotide  showed a 10 cm, irregular, multifocal, highly SRS positive lesion in the splenic bed which could not been separated from the tail of pancreas and stomach wall. Moreover, two focal accumulations with diameters 15 mm and 12 mm were observed in segment VII of the liver. For tumour staging, abdominal CT was performed; the 11 cm diameter lesion in the left hypochondrium spread to nearby organs, and several lesions with diameters between 8-17 mm in segments VII and VIII of the liver were detected. The abdominal lesion was surgically removed by distal pancreatectomy (90Y-DOTATOC followed by 200 mCi of 177Lu-DOTATOC and 200 mCi of 177Lu-DOTATOC). Treatments were well tolerated. The liver and kidney function remained normal. The elevated platelet count normalized after the first treatment; hydroxycarbamide treatment was stopped. One year after PRRT, in December 2016, abdominal CT, liver magnetic resonance imaging (MRI) and octreoscan SPECT/CT showed significant regression of the liver metastases without tumour recurrence in the pancreatic region. Over the next three years, platelet counts were in the 217 to 679 G/L range without any specific treatment, followed by a rise to the current 736 G/L . After one year, control SRS showed 4.4 cm and 2.3 cm focal lesions in segment VII of the liver with marked radiopharmaceutical accumulation. In 2015, peptide radioreceptor therapy (PRRT) was performed: DOTA-d-Phe  was performed on the Illumina NextSeq 500 sequencer system in 2 × 150 cycle paired-end mode. A Twist Library Preparation EF kit (Twist Bioscience) was used for library preparation. Raw data were aligned to the hg38 reference genome using the NextGene software (SoftGenetics). The test targets all protein coding exons and exon-intron boundaries (± 20 bps). The analysis was restricted to the following genes: MEN1, VHL, NF1, TSC1, TSC2, MUTYH, BRCA2, CHEK2. No causative variant was identified.The presented case is instructive in several ways. First, paraneoplasia has to be considered in case of splanchnic vein thrombosis and detailed tumour search is warranted especially in young patients. Second, diagnosis of non-functioning neuroendocrine tumours may delay by several years due to the lack of specific clinical symptoms. Third, systemic therapies such as SSAs and chemotherapy, and targeted therapy using PRRT are therapeutic options for patients with advanced metastatic or inoperable pNET –17.SSAs exert antiproliferative and antisecretory effects. In addition, activation of somatostatin receptor-2 and -3 also have proapoptotic effects , 17. In 90Yttrium or 177Lutetium is bound to SSA via a chelator and SSA directs the complex to NET cells expressing somatostatin receptors in their surface. In the NETTER-1 trial the results of the interim analysis suggested longer progression-free survival and a higher response rate with 177Lu-DOTATATE than with high-dose octreotide LAR  in the 177Lu-DOTATATE arm vs.11.0 months  in the sunitinib arm  NET after failure of medical therapy including SSA, chemotherapy, or novel targeted drugs (sunitinib or everolimus). It remains unclear if, and how long SSA should be continued after PRRT as a maintenance therapy. Opinions favoring the early consideration of PRRT are accumulating  repository, accession number PRJNA902518 , Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants provided their written informed consent to participate in this study. Written informed consent for publication of the clinical data and images was obtained from the patient.LS, NZ and KR cared for the patient. LS, ZB and EVN participated in the conceptualisation and manuscript preparation. LB made histopathological examination. SB performed somatostatin receptor scintigraphy. ZK performed the surgical resections. EBN evaluated the CT and MR images. NG and DW performed PRRT. SA and IB made the genetic testing. ZB and EVN finalized the manuscript. All authors contributed to the article and approved the submitted version.