Segmental dilatation of the intestine

Purpose

The aim of this work is to discuss the pathogenesis of the segmental dilatation of the intestine (SDI) and to review its clinical presentation and the ways to confirm the diagnosis.

Methods

Eight cases of pathologically proven SDI from 1987 to 2003 were reviewed and discussed. There were 7 newborns and a 1-year-old boy.

Results

Our patients are 5 boys and 3 girls. In all cases, the diagnosis was not suspected before surgery. Two patients presented with a low neonatal bowel obstruction. Six patients were operated for omphalocele, which was the most frequent associated malformation. The SDI involved the ileum in all patients. The treatment consisted on a resection of the dilated segment with an end-to-end anastomosis. Histological examination demonstrated the presence of ganglion cells in all cases. The muscular layer was hypertrophied in two cases and very thin in one case. A heterotopic gastric mucosa was observed in one case. No anomalies were observed in 5 cases. The postoperative course was uneventful in 6 cases with a mean follow-up of 5 years.

Conclusions

Segmental intestinal dilatation is an exceptional pathology with an unknown etiology and a misleading clinical presentation. Several theories were proposed to explain this malformation; however, most authors are rather inclined to an embryological theory incriminating an extrinsic intrauterine intestinal compression. Most cases are neonatal discoveries. The clinical polymorphism and the lack of specificity of radiological investigations explain the difficulties to have a preoperative diagnosis. However, this difficulty is compensated by the favorable evolution after the resection of the dilated segment.     

Cystic Duplication of the Cecum with Segmental Dilatation of the Ileum: Report of a Case

Duplication cyst and segmental dilatation are rare congenital anomalies of the gastrointestinal tract, both of which are known to result in intestinal obstruction. We describe herein a case of intestinal obstruction in a neonate, caused by a duplication cyst in the cecum. A small dilated segment of ileum was also present at the site where Meckel's diverticulum would be expected, which was not causing obstruction to the luminal contents. Although it is well known that either of these conditions may coexist with a number of congenital malformations, their concurrent occurrence has never been reported before. Received: September 1, 1999 / Accepted: July 25, 2000     

Congenital mesenteric defect: Description of a rare cause of distal intestinal obstruction in a neonate

INTRODUCTIONInternal hernias are a rare cause of bowel obstruction in the neonate and present with bilious vomiting. Newborns may be at risk of loss of significant length of bowel if this rare condition is not considered in the differential diagnosis of bilious emesis.PRESENTATION OF CASEWe report a case of a twin with an internal hernia through a defect in the ileal mesentery who presented with neonatal bowel obstruction. The patient had a microcolon on the contrast enema suggesting that the likely etiology was an intra-uterine event most likely a vascular accident that prevented satisfactory meconium passage into the colon.discussionAn internal hernia is rarely considered in the differential diagnosis of distal bowel obstruction in a neonate with a microcolon. Congenital trans-mesenteric hernias constitute only 5–10% of internal hernias. True diagnosis of trans-mesenteric hernias is difficult due to lack of specific radiology or laboratory findings to confirm the suspicion.conclusionWhen clinical and radiological findings are not classical, rare possibilities such as an internal hernia must be considered in the differential diagnosis, to avoid catastrophic bowel loss.     

Segmental multicystic dysplasia and ureteropelvic junction obstruction in a nonduplicated kidney

A 2-month-old child presented for evaluation of prenatal hydronephrosis. Imaging studies were consistent with a right duplex system with a dysplastic, nonfunctioning upper pole and lower pole ureteropelvic junction obstruction. We proceeded with removal of the upper pole and pyeloplasty and were surprised to find a single collecting system with a cystic, dysplastic upper pole segment and the absence of an upper pole pelvis or ureter. The rare diagnosis of a segmental multicystic dysplastic kidney with ipsilateral ureteropelvic junction obstruction was made. We present a review of the case and of previous literature on this topic.     

Congenital nasal pyriform aperture stenosis: a rare cause of neonatal nasal obstruction

Congenital nasal pyriform aperture stenosis has been described as an unusual cause of neonatal nasal obstruction. Clinical suspicion is based on respiratory distress, cyclic cyanosis, apneas, and feeding difficulties. A bony overgrowth of the maxillary nasal processes is thought to be responsible for this deformity. This anomaly has been reported as an isolated feature or can be associated with craniofacial or central nervous system anomalies. Surgery is indicated in cases of severe respiratory distress, feeding difficulties, and when conservative methods fail.     

A rare cause of small bowel obstruction due to bezoar in a virgin abdomen

IntroductionBezoar is an unusual cause of small bowel obstruction accounting for 0.4–4% of all mechanical bowel obstruction. The common site of obstruction is terminal ileum.Case reportA 28-year-old male with no past surgical history, known to have severe mental retardation presented with anorexia. CT scan demonstrated dilated small bowel loops and intraluminal ileal mass with mottled appearance. At exploratory laparotomy, a bezoar was found impacted in the terminal ileum 5–6 inches away from the ileocecal valve and was removed through an enterotomy.DiscussionBezoars are concretions of fibers or foreign bodies in the alimentary tract. Small bowel obstruction is one of common clinical symptoms. The typical finding of well-defined intraluminal mass with mottled gas pattern in CT scan is suggestive of an intestinal bezoar. The treatment option of bezoar is surgery including manual fragmentation of bezoar and pushing it toward cecum, enterotomy or segmental bowel resection. Thorough exploration of abdominal cavity should be done to exclude the presence of concomitant bezoars. Recurrence is common unless underlying predisposing condition is corrected.ConclusionsBezoar-induced small bowel obstruction remains an uncommon diagnosis. It should be suspected in patients with an increased risk of bezoar formation, such as in the presence of previous gastric surgery, a history suggestive of increased fiber intake, or patient with psychiatric disorders. CT scan is helpful for preoperative diagnosis.     

Intestinal duplication in adulthood: A rare entity, difficult to diagnose

Duplications of the alimentary tract (ATD) are rare congenital anomalies often found early in life. They may occur anywhere in the intestinal tract but the ileum is the most frequently affected site. Clinical presentation of ATD in adults is variable and because these lesions occur so infrequently they are rarely suspected. In the present report we describe a case of ileal duplication in a 61-year-old patient with Crohn’s disease. Despite various radiological investigations and medical consultations, the diagnosis was only made on the surgical specimen.     

少见病因肠梗阻的诊治

目的 探讨少见肠梗阻的诊断及治疗.方法 回顾性分析5年收治的20例少见肠梗阻的临床资料.结果 20例病人中,3例胆石性肠梗阻,4例柿石性肠梗阻,4例放射性肠炎致肠梗阻,小肠扭转2例,小肠内疝3例,乙状结肠子宫内膜异位症3例,肠系膜根部挛缩致血运性肠梗阻1例,均经手术治愈.结论 正确认识少见病因引起的肠梗阻的临床特点,对指导诊断和治疗有着重要的意义.     

Segmental dilatation of sigmoid colon in a neonate: atypical presentation and histology

Segmental dilatation of the colon is a rare disorder of colonic motility in children, often presenting with severe constipation in older infants, children, and occasionally adults. It may mimic the commoner Hirschsprung disease clinicoradiologically but differs in that the ganglion cell morphology and distribution are typically normal in the colon. We report a neonate with segmental dilatation of the sigmoid colon who had an atypical clinical presentation and describe certain abnormalities in bowel histology (hypertrophied muscularis propria, nerve plexus, and ganglion cells located within the circular layer rather than the normal myenteric location), for the first time in the English literature.     

Infantile visceral myofibromatosis--a rare cause of neonatal intestinal obstruction

Infantile myofibromatosis (IM) is a rare cause of intestinal obstruction in the newborn. A neonate with generalized IM having multifocal intestinal lesions presenting with intestinal obstruction is reported here. Unique intraoperative pictures are provided. The presentation, management, and the prognosis of IM are discussed.     

Segmental dilatation of the ileum accompanying hypoproteinemia

Purpose

Segmental intestinal dilatation (SID) is a rare pathologic finding, which causes intestinal obstruction in newborn period and gastrointestinal bleeding, anemia, abdominal pain, malabsorption, and growth failure in older children. We present a case of SID causing hypoproteinemia.

Patient

A 10-year-old girl presented with recurrent abdominal distension since she was 8.5 months old. She was diagnosed to have protein-losing intestinal lymphangiectasia. Result of physical examination was normal except moderate growth retardation. Her blood workup showed depletion in total protein, albumin, and globulin levels. Gastrointestinal series revealed a dilated segment of small intestine, and Tc^99m-pertechnetate scintigraphy detected ectopic activity in abdomen. The patient was taken to operation with the presumptive diagnosis of intestinal duplication. A dilated segment of ileum was encountered, and segmental resection and anastomosis were performed.

Results

Patient was discharged on the postoperative fourth day and remains well. Histopathologic examination of the specimen revealed SID.

Discussion

Segmental intestinal dilatation is an uncommon congenital anomaly. Most of the cases were operated on in newborn period because of intestinal obstruction, and their diagnoses were made perioperatively; the others were diagnosed in older ages during the investigation of nonspecific symptoms. The index patient is the second case of SID having hypoproteinemia in the literature. Gastrointestinal series suggested the diagnosis, and segmental resection and anastomosis were the adequate treatments.     

Superior mesenteric artery syndrome: a rare cause of complete intestinal obstruction in neonates

Superior mesenteric artery syndrome (SMAS) is an uncommon cause of high intestinal obstruction in neonates; it is owing to incomplete obstruction in the third part of the duodenum caused by compression between the SMA and abdominal aorta. In neonates, complete intestinal obstruction owing to SMAS has been very rarely reported in the literature .We present a 7-day-old previously healthy male infant with a short history of gastroenteritis and sepsis followed by progressive abdominal distension and persistent bilious vomiting that resulted in hypovolemic shock. The patient was aggressively resuscitated, and a gastrografin study showed a hugely distended stomach with an abrupt narrowing at the third part of the duodenum. Exploratory laparotomy unexpectedly revealed a high insertion of the duodenum at the ligament of Treitz, with upward displacement and SMA compression leading to duodenal obstruction. The ligament of Treitz was divided, and the duodenum, mobilized. The postoperative course was uneventful. Although extremely rare, SMAS should be considered as one of the differential diagnoses in newborns presenting with complete intestinal obstruction, especially if preceded by gastroenteritis or sepsis-like symptoms.     

A rare cause of small bowel obstruction: Abdominal cocoon

INTRODUCTIONThe clinical manifestations of abdominal ‘cocoon’ are non-specific and hence its diagnosis is rarely made preoperatively and the management is often delayed. Surgery remains the main stay of treatment with satisfactory outcome and comprises excision of the fibrous membrane, meticulous adhesionolysis and release of the entrapped small bowel.PRESENTATION OF CASEA 45-year-old male patient presented with 6-month history of progressive subacute small bowel obstruction. After initial radiological investigations, he underwent diagnostic laparoscopy and was misdiagnosed as abdominal tuberculosis. He was started on anti-tuberculous therapy, but exploratory laparotomy was carried out after failure to respond to anti-tuberculous therapy. At laparotomy, the abdominal ‘cocoon’ which was encapsulating the entire small bowel was excised, and the adhesions were carefully lysed. The patient remained well and without recurrence at 1-year follow-up.DISCUSSIONAbdominal ‘cocoon’ is a rare cause of subacute, acute and chronic small bowel obstruction. Its diagnosis is rarely made preoperatively.CONCLUSIONAbdominal ‘cocoon’ should be thought of as a rare cause of small bowel obstruction. It may be mistaken with abdominal tuberculosis. Surgery remains the mainstay of curative treatment.     

Antral mucosal valve: a rare cause of gastric outlet obstruction in newborn

A 15-day male neonate presented with postprandial nonbilious vomiting. Barium meal suggested pyloric obstruction. Ultrasound of the stomach after saline loading revealed an echogenic antral valve. Subsequent laparotomy confirmed the ultrasound findings. Excision of the valve resulted in excellent recovery. To our knowledge, gastric outlet obstruction because of an antral mucosal valve in a neonate has not previously been described.     

Neonatal ovarian torsion complicated by intestinal obstruction and perforation, and review of the literature

We present a case of neonatal ovarian torsion complicated by bowel obstruction and perforation and review the literature regarding the incidence of bowel obstruction in neonatal ovarian cysts, the presentation, and treatment. A term neonate was prenatally diagnosed with a cystic abdominal mass palpable on physical examination. A postnatal abdominal x-ray showed paucity of gas in the left hemiabdomen with rightward displacement of bowel loops. Exploratory laparotomy on day 2 of life revealed a large cystic mass in the left lower quadrant consistent with a torsed left ovary, an omental band causing strangulation of the bowel mesentery, and a perforation of the distal ileum. Our literature search revealed 19 reported cases of neonatal ovarian cysts resulting in bowel obstruction. Infants may present with a palpable abdominal mass, respiratory distress, as well as signs and symptoms of intestinal obstruction. Two mechanisms exist for bowel obstruction: adhesions caused by a torsed necrotic ovary and mass effect of a large ovarian cyst, often measuring 9 to 10 cm in diameter. Options to treat ovarian cysts include antenatal or postnatal aspiration, laparoscopy, and laparotomy. Cysts less than 4 to 5 cm can be observed, whereas operative intervention is indicated in symptomatic cases and in persistent or enlarging ovarian cysts.     

Distended urinary bladder and diverticulum—a rare cause of large-bowel obstruction

A 76-year-old man presented to the emergency department with diffuse abdominal pain and constipation. In the few months before this admission the patient had complained of strenuous micturition. The diagnostic work-up included a plain abdominal radiograph and an abdominal computed tomography scan that revealed large-bowel obstruction with a rare cause. The colonic obstruction was secondary to external compression of the rectosigmoid colon against the sacrum by a distended bladder and diverticulum. The immediate management was insertion of an indwelling urinary catheter that resulted in quick relief of the obstruction. Later the patient underwent surgery to remove an enlarged benign prostatic adenoma, which was the underlying cause of the bladder distention.     

Intestinal obstruction from appendiceal abscess in a newborn infant

Perforated appendix in the newborn period is rare, its symptoms are occult and its outcome is disastrous. Associated mechanical small bowel obstruction as part of the clinical picture is rare indeed. We have treated a premature infant who presented with prolonged intermittent vomiting. Factors contributing to difficulty in diagnosis and to eventual succussful management are discussed.     

Hemiresective reconstruction of a redundant ileal conduit with severe bilateral ileal conduit-ureteral reflux

A 58-year-old man was referred to our hospital with high fever and anuria. Since undergoing a total pelvic exenteration due to bladder-invasive sigmoid colon cancer, urinary tract infections had frequently occurred. We treated with the construction of a bilateral percutaneous nephrostomy (PCN), and chemotherapy. Although we replaced the PCN with a single J ureteral catheter after an improvement of infection, urinary infection recurred because of an obstruction of the catheter. Urological examinations showed that an ileal conduit-ureteral reflux caused by kinking of the ileal loop was the reason why frequent pyelonephritis occurred. We decided to resect the proximal segment to improve conduit-ureteral reflux for the resistant pyelonephritis. After the surgery, the excretory urogram showed improvement and the urinary retention at the ileal conduit disappeared. Three years after the operation, renal function has been stable without episodes of pyelonephritis. Here we report a case of open repair surgery of an ileal conduit in a patient with severe urinary infection.