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1/17. Agenesis of the vermiform appendix.

    Agenesis of the vermiform appendix is very rare. The incidence is estimated to be one in 100,000 laparotomies for suspected appendicitis. Several criteria have to be met before the investigator can conclude that the appendix is congenitally absent. This case is reported to bring this entity to the attention of surgeons who may encounter a similar situation during celioscopy. A 29-year-old patient was admitted through the emergency room with the chief complaint of abdominal pain. Acute appendicitis was suspected, and he was accordingly prepared for celioscopy. This report presents a patient with vermiform appendix agenesis diagnosed at celioscopy with concomitant mesenteric lymphadenitis. Agenesis of the vermiform appendix is very rare, and the diagnosis should not be made unless the ileocecal and retrocecal area are thoroughly explored.
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ranking = 1
keywords = congenita
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2/17. Cecal duplications: a rare cause for secondary intussusception.

    Duplications of the alimentary tract are rare congenital anomalies that may occur at any level from mouth to anus. While the oesophagus and the ileum are the most common sites, duplications of the colon are rare. Two cases of ileocolic intussusceptions in 8-month-old girl and 6-month-old boy who were admitted to our hospital with acute abdomen findings are presented. Intraoperatively, cecal cystic duplications leading intussusception were revealed. intussusception is one of the most important surgical emergence in infancy and typically, it does not involve a lead point in childhood. Although duplication cyst may act as lead point, the review of literature reveals its rarity.
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ranking = 1
keywords = congenita
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3/17. Idiopathic segmental infarction of the greater omentum as a cause of acute abdomen report of two cases and review of the literature.

    The segmental infarction of the greater omentum is a rare cause of acute abdomen. Its etiology is uncertain although several predisposing factors have been underlined such as congenital venous anomalies, sudden change of position and substantial meal. The clinical picture simulates an appendicitis or cholecystitis, thus being difficult to make a preoperative diagnosis. However, ultrasonography or computed tomography scan can help us make this diagnosis and then we alternatively perform a conservative treatment, laparoscopic approach or resection by laparotomy. We present two cases, preoperatively diagnosed by ultrasonography and computed tomography scan that were treated by laparotomy resection. We also review the published cases in the medical literature.
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ranking = 1
keywords = congenita
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4/17. An unusual case of herniation of small bowel through an iatrogenic defect of the falciform ligament.

    Internal herniation through a congenital defect in the falciform ligament is extremely rare. We report an unusual observation of small bowel obstruction through an iatrogenic defect of the falciform ligament. Prompt diagnosis was made by helical CT, permitting a rapid surgical procedure to preserve the viability of the obstructed segment.
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ranking = 1
keywords = congenita
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5/17. Acute abdomen due to torsion of a pelvic wandering spleen.

    wandering spleen is a rare entity characterized by incomplete fixation of the spleen by lienorenal and gastrosplenic ligaments. wandering spleen can migrate to the lower abdomen or pelvis, and can be either congenital or acquired. It is most commonly found in women of reproductive age, and may be misdiagnosed as an abdominal or adnexal mass. It is usually asymptomatic, but may present with acute, chronic, or intermittent abdominal pain. Here, we report a case of torsion of a huge congenital pelvic wandering spleen and microscopic isolated pancreatic tissue (disconnected from the pancreas) with impending splenic rupture in a 23-year-old female patient. Progressively severe chronic or intermittent torsion of the vascular pedicle of the wandering spleen caused progressive intermittent lower abdominal pain. The patient underwent splenectomy with resection of the long pedicle and the postoperative course was uneventful. The pathognomonic computerized tomography features of this case, including absence of the spleen in the left upper quadrant and the presence of a whirl-like structure running down to the central portion of the distally located large soft-tissue mass and with a notched- (hilar-) like contour, are also described.
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ranking = 2
keywords = congenita
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6/17. Intrathoracic gastric volvulus mimicking pyloric stenosis.

    A 5-week-old-infant presented to hospital following the acute onset of non-bilious vomiting with clinical and acid-base features suggestive of pyloric stenosis. A chest radiograph obtained because of intercurrent infection unexpectedly revealed a left-sided congenital diaphragmatic hernia. A barium meal demonstrated the presence of an intrathoracic gastric volvulus, requiring urgent surgical management. We discuss the presentation and management of this rare surgical cause of non-bilious vomiting in infancy.
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ranking = 1
keywords = congenita
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7/17. Acute abdomen due to wandering spleen infarction: a case report.

    We report a rare clinical case of acute abdomen due to partial infarction of a wandering spleen in the pelvis in a 60-year-old woman. The patient was suffering from stabbing pain in the external lower quadrant of the abdomen, irradiating back to the lumbosacral area, together with an unremitting feverish state (38 degrees C), sickness and constipation. After carrying out serological examinations, which revealed an increase in CPK and leukocytosis, ultrasonography and CT examinations were performed, revealing a mass in the left iliac cavity, which in all probability was a wandering spleen with an abnormally long pedicle and a dyshomogeneous lower area bearing witness to a splenic infarction. The patient was therefore submitted to surgery consisting in splenectomy after lysis of the adherences, which were plainly inflammatory. A wandering spleen, especially when infarcted, is a very rare clinical condition that may be congenital or acquired. Its presence can be confirmed by serological, ultrasonographical and CT examinations and must be suspected when there is no clearly defined acute abdomen.
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ranking = 1
keywords = congenita
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8/17. A rare cause of intestinal obstruction in the adult: Morgagni's hernia.

    Morgagni's hernia is a rare congenital diaphragmatic herniation and is usually diagnosed in childhood. It is quite rare in adults, and intestinal obstruction as a complication due to intrathoracic intestinal herniation rarely occurs. We present the plain radiography and computed tomography findings of an adult patient with acute abdomen symptoms due to Morgagni's hernia.
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ranking = 1
keywords = congenita
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9/17. Antiphospholipid antibodies and splenic thrombosis in a patient with idiopathic myelofibrosis (antiphospholipid antibodies and thrombosis).

    A case of idiopathic myelofibrosis and hepatosplenic myeloid metaplasia associated with antiphospholipids antibodies is described. The patient developed a lethal complete splenic vein thrombosis in spite of an intravenously heparin treatment had been started soon after a clinical pattern of "acute abdomen".
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ranking = 0.19627805025531
keywords = aplasia
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10/17. Acute loss of the small bowel in a school-age boy. Difficult choices: to sustain life or to stop treatment?

    A 9-year-old boy lost almost all his small bowel after an acute volvulus due to a congenital, but previously unsuspected malrotation. survival using total parenteral nutrition is possible in these cases, but the medical burden is heavy. Small intestinal transplantation was performed for the first time in the netherlands in 2001 and this patient was treated 3 years earlier. The results of bowel transplantation are not as good as in kidney or liver transplantation. A method of Ethical Case Deliberation helped to elucidate the importance of each contribution in the discussion and provided space and a broad basis for decision-making. The parents refused to allow parenteral nutrition to be started because of the bad prospects for quality of life in the future and the medical team, after thorough deliberation with specialists throughout the country, and consultation of the literature, agreed. CONCLUSION: Despite the many different opinions, the parents felt accepted in their refusal of treatment for their son and the team accepted the decision.
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ranking = 1
keywords = congenita
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