Congenital segmental dilatation of the jejunum in an African child: a case report.

Congenital segmental dilatation of the intestine is a rare disease. It is rarely located in the jejunum and its etiology is still unknown despite many theories suggesting its mechanism. We report a case of a 17 months girl who experienced nonspecific symptoms (abdominal pain, constipation and loss of appetite) since early her infancy. She had no growth retardation and had moderate abdominal distension on physical examination. Investigations undertaken could not increase suspicion of congenital segmental dilatation of the intestine (CSDI). The diagnosis was made peroperatively and a resection was done, followed by end-to-end jejunal anastomosis. There were no postoperative complications and the patient is doing well after four months. One should think of CSDI in children with chronic subocclusion or digestive hemorrhage.

Perinatal hemorrhage from ulceration of the umbilical cord: A potentially catastrophic association with duodenal and jejunal obstruction.

PURPOSE: The purpose of this study is to review published reports and contribute new cases of umbilical cord ulceration (UCU) with perinatal hemorrhage into the amniotic cavity in the setting of duodenal or jejunal obstruction because knowledge of this sequence is poorly disseminated and could be lifesaving. METHODS: Published reports of UCU with hemorrhage associated with congenital duodenal or jejunal obstruction were reviewed. Chart review was conducted for the cases encountered at our institutions between January 2008 and March 2017. We noted perinatal complications, method of delivery, gestational age, birth weight, gender, number, location, and pathologic description of umbilical cord ulcers, and outcome. RESULTS: Thirty-one reports and 7 new cases were studied. Perinatal complications included: preterm labor or preterm premature rupture of membranes: 63%; fetal distress: 95%; mean gestational age: 33weeks; premature gestation: 95%; bloody amniotic fluid: 90%. Pathological analysis of UCUs revealed solitary, multifocal, helical and punched-out lesions. There were 12 neonatal deaths (32%), and 12 intrauterine deaths (32%). Survival rate was 37%. CONCLUSIONS: UCU with perinatal hemorrhage is associated with duodenal and jejunal obstruction. Knowing the typical clinical signs of this potential catastrophic complication could prompt lifesaving delivery. TYPE OF STUDY: Prognostic LEVEL OF EVIDENCE: IV.

Gastroschisis with gastric perforation and jejunal stenosis A rare association of anomalies.

AIM: Gastroschisis with prenatal gastric perforation and intestinal stenosis is a rare and serious anomaly. although there are several case reports, no case series exists to suggest the prognosis for these infants. MATERIAL AND METHODS: In this report a case of gastroschisis with gastric perforation and jejunal stenosis in male newborn is presented with literature review. The stomach, small bowel and the part of the colon were herniated through the abdominal wall defect. A large perforation site at the anterior wall of fundus and a thin fibrous strip that causing stenosis of jejunum was found. RESULTS: Gastrorraphy was performed. Stenosis of jejunum was resected and t-t anastomosis was performed, followed by primary fascial closure. DISCUSSION: The prenatal sonographic finding of bowel or gastric perforation are variable. Antenatal bowel dilatation and in particular intraabdominal bowel dilatation is prognostically useful for detection of patients with worse outcome. The absence of bowel dilatation cannot fully exclude complex patients. Early restoration of bowel continuity using primary anastomosis and primary abdominal wall closure are not associated with prolonged time for full enteral feeding and length of hospital stay. CONCLUSIONS: We have presented the first detailed report of surgical intervention and outcomes in case of gastroschisis with prenatal gastric perforation and congenital jejunal stenosis. Early restoration of bowel continuity using primary anastomosis and primary abdominal wall closure is recommended here. More research should be focused to predict complex gastroschisis and to improve prenatal diagnosis and postnatal management, without a significant increase in morbidity and mortality. KEY WORDS: Gastroschisis, Gastric perforation, Stenosis of jejunum.

Ultrasonographic diagnosis of congenital membranous jejunal stenosis and gastric duplication cyst in a newborn: a case report.

INTRODUCTION: Congenital jejunal stenosis and gastric duplication cysts are very rare congenital anomalies of the gastrointestinal tract in the newborn. We present a case of congenital membranous jejunal stenosis associated with gastric duplication cysts, which was diagnosed by ultrasonography. To the best of our knowledge, this is the first report of ultrasonographic diagnosis of congenital membranous jejunal stenosis associated with a gastric duplication cyst in a newborn. CASE PRESENTATION: A 1-month-old Chinese baby girl presented with projectile vomiting and hyperpyrexia for 3 days. An upper gastrointestinal contrast study showed incomplete duodenal obstruction; however, ultrasonography revealed congenital membranous jejunal stenosis associated with a gastric duplication cyst. After surgical excision of the jejunal membrane and gastric duplication cyst, she recovered well with no bilious vomiting at the 1-year follow-up. CONCLUSION: Ultrasonography is a useful tool for the evaluation of membranous jejunal stenosis and the identification of small, asymptomatic gastrointestinal duplication cysts.

Jejunal diverticulum with ectopic pancreatic mucosa: was it really a Meckel's diverticulum?

We report the case of a 72-year-old man who underwent surgery for a jejunal intussusception caused by an infarcted mass on the antimesenteric surface of the small bowel approximately 90 cm from the duodenojejunal flexure. The specimen was reported as an infarcted diverticulum containing heterotopic pancreatic mucosa in keeping with infarcted Meckel's diverticulum. We initially wished to establish when the term "Meckel's diverticulum" should be used. The anatomical site in the present case argues against this being a Meckel's diverticulum, a remnant from the mid-gut loop. Non-Meckelian diverticula are usually acquired and often multiple and generally occur on the mesenteric border of the bowel. It is rare for them to contain ectopic mucosa. The diverticulum in our patient thus has features which suggest it may have been congenital. We believe our patient had a rare occurrence of a congenital, but non-Meckelian, diverticulum with associated ectopic epithelium.

Apple-peel atresia presenting as foetal intestinal obstruction.

Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA) is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed signs of intestinal obstruction on day one after birth, for which an exploratory laparotomy was performed. Type 3 JIA was found for which resection of atretic segments with jejuno-ascending colon anastomosis was preformed.

Should we be concerned about jejunoileal atresia during repair of duodenal atresia?

INTRODUCTION: During repair for duodenal atresia, it has been emphasized that inspection of the small bowel to identify a second atresia is required. The laparoscopic approach for repair of duodenal atresia has been criticized for its limitation to perform this step. Given that duodenal atresia and jejunoileal atresias do not share common embryologic origins, we question the validity of this concern. Therefore, we conducted a multicenter retrospective review of duodenal atresia patients to quantify the incidence of jejunoileal atresia in this population. METHODS: After institutional review board approval (IRB #07-12-187X), a retrospective review was conducted on all patients who have undergone duodenal atresia repair at seven institutions over the past 7-12 years. Demographics and the presence or absence of a jejunoileal atresia were recorded. RESULTS: Four hundred eight patients with duodenal atresia were identified. The mean gestaational age was 36.3 ± 2.9 weeks, and the mean weight was 2.5 ± 0.8 kg. Mean age at operation was 19 days (range, 1-1314). There was a 28% incidence of trisomy 21. Two patients (0.5%) were identified as having a second intestinal atresia, and both were type IIIb. One patient was diagnosed at the time of duodenal atresia repair; the other was a delayed diagnosis. Both patients did well after repair. CONCLUSIONS: In this, the largest series of duodenal atresia patients compiled to date, the rate of a concomitant jejunoileal atresia is less than 1%. This low incidence is not high enough to mandate extensive inspection of the entire bowel in these patients, and a second atresia should not be a concern during laparoscopic repair of duodenal atresia.

Disorders of interstitial cells of Cajal in a neonate with segmental dilatation of the intestine.

Localized myopathy of the muscular layers may be an important factor contributing to segmental dilatation of the intestine (SDI). Only one report has described SDI of the jejunum in a neonate showing no abnormality of the interstitial cells of Cajal (ICC). The present report describes the very rare case of a neonatal girl with segmental dilatation of the distal duodenum and proximal jejunum with irregular arrangements of Auerbach's plexus and ICC and the successful surgical treatment of SDI. We review the literature on this type of relationship between abnormality of ICC and SDI and discuss the clinical features of this complication. Furthermore, the possible neuropathic cause of SDI complicated with disorders of ICC was explored in this report.

Different types of intestinal atresia in identical twins.

The authors present a previously unreported association of different types of intestinal atresia in identical low-birth-weight twins. Both babies were affected by duodenal atresia, associated in the first case with a complete mucosal duodenal membrane and in the second one with an "apple-peel" jejunal atresia. These occurrences may suggest that they were either the consequence of linkage of 2 genes or a pleiotropic expression of a single gene responsible for such rare conditions.

Volvulus of a jejunal lymphatic vascular malformation presenting with bilious vomiting and the radiological appearances of malrotation.

Bile-stained vomiting in infancy is abnormal. Malrotation with small bowel volvulus is probably the most worrying of possible causes because if untreated for too long, it can result in massive bowel necrosis. As a consequence, if examination or plain radiography does not suggest another cause, an upper gastrointestinal (GI) series is often performed to exclude malrotation. If the examination is normal however and symptoms settle, should investigation stop, or how far should it be pursued? Small bowel lymphatic vascular malformations in children are extremely rare but carry a risk of obstruction, volvulus, and loss of small bowel. We present a case of a 5-year-old boy referred to our unit with bilious vomiting and the radiological findings of malrotation on upper GI series. Interestingly, a previous upper GI series undertaken during the first year of life for intermittent bilious vomiting was normal. Operative findings were of a 16-cm cystic lymphatic vascular malformation of the jejunal wall causing a 360 degrees volvulus. The mesentery was derotated, and the cyst removed with the attached section of jejunum. This case highlights the need to reinvestigate patients with bilious vomiting despite a previous normal upper GI series. The case raises the issue of whether bilious vomiting, with a normal upper GI series, should be investigated further with an abdominal ultrasound scan to identify more unusual causes such as jejunal lymphatic vascular malformations.

[Left paramesocolic hernia with retroperitoneal incarceration of jejunum]. / Incarceración retroperitoneal del yeyuno en una hernia paramesocólica izquierda.

We present a case left paramesocolic hernia and review the literature. The patients was a 14 years old male and had suffered periods of relapses into abdominal pain from the age of three. When for years old he was operated on using the Nisses technique. Also a laparoscopy had already been performed on this same patient when he was 13 years old, without discovering any pathological conditions. In a new upper gastrointestinal serie, realized one year later, a retroperitoneal incarceration and a paraduodenal loop of jejunum was observed, which obstructed, partially the second part of the duodenum. We have not found the anatomical characteristics of this case in any previous report. In the ample literature pressured, it was confirmed that almost all the clinical cases, previously published had suffered a relapse abdominal pain crisis, diagnosed in advanced states and in/or emergency situations. This produces a mortality rate higher than 20% and or irreparable digestive damage. In cases involving paraduodenal hernias, there exists a low rate of diagnostic suspicion. These delays and diagnostic errors cause irreparable damage. In those patients who suffer relapses into abdominal pain syndrome, one should always include the differential diagnosis of PMH, applying a complete gastrointestinal serie or a CT-Scan with contrast.

Prenatal diagnosis of bowel obstruction initially manifested as isolated hyperechoic bowel.

Fetal bowel obstruction has a prevalence of 1 in 3000 to 5000 live births. Ultrasonographic diagnosis is made by demonstrating distended loops of bowel. Echogenic bowel, defined as small bowel more echogenic than liver or bone, has been associated with congenital infections, cystic fibrosis, chromosomal abnormalities, and bowel obstruction. Fetal ascites, defined as fluid in the peritoneal cavity partly surrounding the liver and bladder, also has been associated with bowel obstruction. We present a case of jejunal atresia whose presenting appearance consists of echogenic bowel, transient ascites, and massive dilatation of intestinal loops.

Additional congenital anomalies in babies with gut atresia or stenosis: when to investigate, and which investigation.

A wide variety of additional congenital anomalies occur in babies born with a gut atresia or stenosis. The specific pattern of anomalies depends on the location of the atresia. The serious nature of many of them makes perioperative diagnosis imperative. Eighty-six babies born with pure oesophageal atresia (OA), duodenal atresia (DA) or stenosis, or jejuno-ileal atresia (JIA) have been studied. These, combined with over 2,000 cases in the literature, have been used to develop a protocol to optimally investigate babies with gut atresia for associated anomalies. The authors recommend routinely obtaining anterio-posterior and lateral chest and abdominal radiographs for babies with pure OA, DA and intestinal atresia, making sure the entire spine can be visualised. Cardiac and renal ultrasonography (US) should be routine in all babies with pure OA or DA. A micturating cystourethrogram should be done in those babies with abnormal urinary tract US or an associated anorectal anomaly. A sweat test should be obtained in babies with JIA, and a rectal biopsy should be taken in babies with the combination of Down's syndrome and DA to exclude Hirschsprung's disease.

Biliary atresia with ectopic proliferation of the bile ducts: a case report.

The authors describe a case of biliary atresia with ectopic proliferation of the bile ducts in the jejunal wall. This case supports a possible causal association between a defect in biliary ductal embryogenesis and the pathogenesis of certain cases of biliary atresia.