Duodenoplasty in the management of duodenal atresia

When the duodenum is congenitally obstructed it becomes distended to varying degrees. Our case of combined duodenal and multiple jejunal atresias indicates the value of duodenoplasty for symptomatic duodenomegaly diagnosed in late childhood. The technique used has restored duodenal motility and is recommended for selective use as part of the primary surgery for duodenal and proximal jejunal atresia. Offprint requests to: E. J. Guiney     

Tapering duodenoplasty

Atresia of the distal duodenum or duodenojejunal region may result in massive duodenomegaly. We report three such cases managed by extensive tapering duodenoplasty. The operative technique described is relatively straightforward and has resulted in rapid restoration of duodenal motility. Offprint requests to: D. F. M. Thomas     

Modified plication technique for the treatment of intestinal atresia

Three patients with intestinal atresias were treated by seromuscular stripping and mucosal plication to eliminate the risk of anastomotic leakage through the plication line and reduce the bulk of the plicated mass. The patients all had uncomplicated postoperative courses and tolerated oral feedings within 10 days. Offprint requests to: F. C. Tanyel     

Multiple gastrointestinal atresias with cystic dilatation of the biliary duct

A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome. Accepted: 19 April 1999     

Clinical experience of complex jejunal atresia

Purpose

Small intestinal atresia is relatively common anomaly that causes intestinal obstruction in neonates. Although surgical interventions are usually successful, critical problems could raise in certain cases. This study aimed to identify the distinct clinical characteristics of complex cases of jejunal atresia by retrospective analysis.

Methods

Overall, 91 cases of small intestinal atresia, which occured in infants between 2001 and 2010 at Pusan National University Children’s Hospital, were reviewed retrospectively. The clinical characteristics of complex jejunal atresia were analyzed.

Results

Of the 91 small intestinal atresias, 11 cases of complex jejunal atresia were found: high jejunal atresia with distal deletion, 3; high jejunal atresia with distal multiple atresias, 4; jejunal atresia with distal apple peel appearance, 1; jejunal atresia with colonic atresia, 1; jejunoileal atresia with distal volvulus, 2. Short bowel syndrome was found in four patients and bowel-lengthening procedure was performed in all. Three patients presented with an adhesive intestinal obstruction during the early postoperative period. Postoperative mortality occurred in one patient with distal volvulus.

Conclusions

From a surgical perspective, complex jejunal atresia can cause many critical problems after the correction operation. An aggressive and multidisciplinary approach is necessary for managing this condition.     

Electromyographic study of the mechanism of postoperative cholangitis in congenital biliary atresia

Cholangitis after biliary reconstruction for congenital biliary atresia is a troublesome postoperative complication. In order to clarify its mechanism and the changes in intestinal movement after biliary reconstruction by Roux-en-Y anastomosis, we performed electromyographic (EMG) studies of the motility of the reconstructed intestine in dogs. Monitoring the basic electrical rhythm (BER) and migrating myoelectric complexes (MMC), EMG analysis was carried out. As most of the Y-loop MMCs were propagated smoothly to the anal side, according to the continuity of the intestine, the intestinal contents were transported without stagnation. These intestinal movements appear to be useful as a biliary drainage route and to prevent ascending cholangitis. A comparison of short- and long-term intestinal motility after biliary reconstruction showed adaptation of the intestinal movement following the procedure. The outflow of bile appeared to accelerate intestinal motility because of prolongation of the MMC interval in the duodenum and oral jejunum without bile flow. Offprint requests to: M. Yagi     

A very rare type of multiple intestinal atresias: “A string of pearls”

Intestinal atresia is themost frequently encountered cause of ileus in the neonate. Of all atresias combination of small and large bowel atresias is extremely rare. In 1973, Guttman presented a case with multiple, widespread atresias of small and large bowel, intraluminal calcifications and a hereditary nature. This paper describes the detailed pathological findings of a similar case of multiple intestinal atresias and discusses for the pathogenesis of this rare condition. An erratum to this article is available at .     

Laparoscopy-assisted surgery for neonatal intestinal atresia and stenosis: a report of 35 cases

Purpose

Jejunal?Cileal atresias are the most common causes of intestinal occlusion in neonatal period. Treatment is classically performed by a right upper quadrant transverse laparotomy. Our study aimed to present our initial experience of intestinal atresia in newborn treated with laparoscopic assisted approach.

Methods

Overall 35 small intestinal atresias, which occurred in infants from September 2009 to July 2012 in our hospital, were treated by laparoscopy-assisted procedure. After carefully inspecting through laparoscope by a multi-port or single-site approach, these were definitely diagnosed. The anastomosis of intestinal atresia was manually performed after exteriorization of the bowel via the umbilical port site incision.

Results

There were no conversions to an open procedure and no intraoperative various complications. The incision of umbilical port was about 2?C2.5?cm. The post-operative course was uneventful.

Conclusion

Laparoscopy-assisted procedure could be safely accomplished in neonates with intestinal atresia. Comparing to open surgery, parents were extremely satisfied with the cosmetic results. The early experience suggests that the outcomes are excellent.     

Multiple atresias with extensive intraluminal calcifications in a newborn with cystic fibrosis

A newborn patient with cystic fibrosis and multiple intestinal atresias demonstrated intraluminal calcifications on a plain abdominal radiograph. Cystic fibrosis may be an aetiological factor for intraluminal calcifications secondary to multiple intestinal atresias. Received: 9 July 1997 Accepted: 13 October 1997     

Erythromycin establishes early oral feeding in neonates operated for congenital intestinal atresias

Purpose  The recovery of gastrointestinal function following surgery for congenital intestinal atresias can be prolonged and may increase morbidity and hospital stay. This study was conducted to investigate the prokinetic effect of erythromycin in neonates undergoing surgery for small bowel atresias. Methods  A randomized-controlled trial was conducted at the Departments of Paediatrics and Paediatric Surgery, Military Hospital, Rawalpindi, Pakistan, from January to December 2007 to study the prokinetic effect of erythromycin (3 mg/kg per dose 4 times daily). Thirty consecutive neonates undergoing primary anastomosis for congenital small bowel atresias were randomly divided into two groups: group I (erythromycin) and group II (control). The groups were similar in terms of gestational age, sex, mode of delivery, birth weight and types of atresias. Postoperative recovery of intestinal functions was measured as time taken to achieve full enteral feed (150 ml/kg per 24 h), duration of total parenteral nutrition (TPN) and hospital stay. Results  Neonates receiving oral erythromycin achieved full enteral feeding early (13.07 vs. 16.13 days) required TPN for shorter duration (10.53 vs. 13.73 days) and their hospital stay was less (16.2 vs. 18.0 days) as compared to the neonates in the control group who did not receive any erythromycin. The differences were statistically significant. Conclusion  The administration of oral erythromycin following primary anastomosis for small intestinal atresias results in early recovery of intestinal function, fewer days on TPN and a trend for shorter hospital stay.     

Comparative outcomes in intestinal atresia: a clinical outcome and pathophysiology analysis

Objective  

To describe the outcomes of 130 intestinal atresias between 1982 and 2007.     

Colonic atresia: surgical management and outcome

Colonic atresia (CA) is a very rare cause of intestinal obstruction, and little information has been available about the management and predictors of outcome. A retrospective clinical trial was performed to delineate the clinical characteristics of CA with special emphasis on surgical treatment and factors affecting outcome. Children with CA who were treated in our department between 1977 and 1998 were reviewed: 14 boys and 4 girls aged 1 day to 5 months. All but 2 referred patients and 1 with prenatal diagnosis presented with intestinal obstruction. Plain abdominal X-ray films showed findings of intestinal obstruction in 14 cases; a barium enema demonstrated a distal atretic segment and microcolon in 4. The types of atresia were IIIa (n=9), I (n=6), and II (n=3). Type IIIa atresias were located proximal to the splenic flexure (n=8) and in the sigmoid colon (n=1), type I atresias were encountered throughout the colon; and all type II atresias were proximal to the hepatic flexure. Associated anomalies were multiple small-intestinal atresias (MSIA) (n=4), gastroschisis (GS) (n=2), pyloric atresia (n=1), Hirschsprung's disease (n=1), and complex urologic abnormalities (n=1). The initial management was an enterostomy in 15 patients (83%), including 2 referred and 2 with GS, and primary anastomosis in the remaining 3 (17%). Secondary procedures were the Santulli operation (n=2), colostomy closure and recolostomy followed by a Swenson operation (n=1), sacroabdominoperineal pull-through (n=1), and colostomy closure (n=1). Leakage was encountered in all primarily anastomosed patients. The overall mortality was 61%. Deaths occurred in patients with associated major anomalies (GS 2, MSIA 3, pyloric atresia 1) (55%) and in 3 patients who were initially managed by primary anastomosis (27%). Two additional patients died of sudden infant death syndrome (18%). Type I atresia was more common than in previously reported series and was associated with proximal multiple atresias. The initial management of CA should be prompt decompression of the intestine by an ostomy procedure, preferably end- or double-barrel. The type of surgery (primary anastomosis without prior colostomy) and associated abnormalities are the major determinants of poor outcome. Accepted: 19 December 2000     

Multiple gastrointestinal atresias

Three neonates with numerous mucosal septal atresias that occluded the intestinal lumen, commencing at the prepyloric region and ending at the rectum, are reported. The clinical and pathological features are presented and an attempt is made to offer a concept for the pathogenesis of multiple gastrointestinal atresias.     

Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination.     

Disseminated intestinal hypoganglionosis treated by colectomy and tapering of the small intestine. A case report.

A girl suffering from chronic constipation and abdominal distension from her first year of life underwent internal anal sphincter myectomies at 5 and 7 years of age without resolution of her symptoms. At the age of 8, an ileostomy was performed because of excessive colonic dilation and hypomotility. Biopsies from the colon and distal ileum showed intestinal neuronal dysplasia TYPE B (INDB) with hypoganglionic areas. Colectomy and ileorectal anastomosis were done at the age of 10. Three years later, however, an ileostomy was re-established because of recurrent episodes of pseudo-obstruction. In the hope of improving intestinal motility, the dilated small intestine was tapered over its entire length of 3.6 meters. Histological findings still demonstrated oligoneuronal hypoganglionosis and INDB all along the resected strip of bowel wall. After 6 months, the stoma was closed. At the age of 15 years, tapering of the distal 80 cm of the ileum was repeated in combination with cholecystectomy for cholecystolithiasis. Intestinal transit time decreased from 55 hours before the first to 18 hours after the second tapering procedure. Now, 7 years after the last operation, the patient passes 3 - 4 soft stools daily, is physically active, on a normal diet and not on any regular medication.     

TTC7A mutation must be considered in patients with repeated intestinal atresia associated with early inflammatory bowel disease: Two new case reports and a literature review

TTC7A mutations cause multiple neonatal intestinal atresias with early inflammatory bowel disease and severe combined immunodeficiency. There are no treatment protocols for this rare disease. Two new cases are described for which radical early treatment measures – total enterectomy, home parenteral nutrition, immunoglobulin therapy and intravenous antibiotic prophylaxis – have allowed both patients to develop optimally.     

Multiple gastrointestinal atresias result from disturbed morphogenesis

 Multiple gastrointestinal atresias (MGA) have been reported to account for 6% to 32% of all intestinal atresias. Controversy exists regarding the pathogenesis. Many investigators believe MGA to be the result of multiple ischemic infarctions of the intestinal tract. However, some have suggested that MGA results from a malformative process early in fetal life. Prenatal exposure to adriamycin in a rat model has been reported to lead to a spectrum of tracheoesophageal and associated malformations of the gastrointestinal tract, including intestinal atresias, identical to these observed in humans. The aim of this study was to determine the incidence and histopathologic findings of MGA in order to understand the pathogenesis. Timed-pregnant Sprague-Dawley rats were injected with adriamycin (1.75 mg/kg) in nine different gestational-day protocols. MGA was only seen in those rats who received adriamycin on gestational days 7, 8, and 9. The litters were recovered on day 21 by cesarean section. The digestive tracts (DT) of the fetuses were harvested for macroscopic and microscopic examination. Ten rats who received adriamycin on gestational days 7, 8, and 9 produced 87 newborns; 1 was damaged during dissection. DT anomalies occurred in 80 (93%) of the 86 newborns; 94% of these demonstrated MGA. There was a very high incidence of associated anomalies in newborns with MGA. Histologically, the blind-ending atresias showed different degrees of villous hyperplasia with or without intraluminal material. This is the first report demonstrating a high rate of occurrence of MGA in the adriamycin rat model. The injection of adriamycin early in gestation, the high incidence of associated malformations, and the anatomic and histologic findings in MGA indicate that MGA is a result of a malformative rather than an ischemic process. Accepted: 22 September 2000     

Irreversible intestinal failure

Intestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life-long dependency on PN. Non-transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon-like peptide-2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo-obstruction syndrome may also cause permanent IF. PN and home-PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long-term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico-surgical strategies for prevention and treatment of these conditions at several stages of IF.     

Pyloric atresia associated with ileal and rectal atresia

Multiple gastrointestinal atresias is a rare, autosomal-recessively inherited condition. A case of congenital pyloric atresia associated with ileal and rectal atresias is reported. Corrective surgery was carried out, but the child died at 1 month of age from associated sepsis. The syndrome of multiple atreasias affecting various levels in the gastrointestinal tract is probably a distinct entitiy; its pathogenesis is still unknown and it carries a high mortality. Offprint requests to: S. Chittmittrapap     

Gastrointestinale Atresien

Gastrointestinal atresias represent an important group of congenital malformations in the field of pediatric surgery. They may affect all intestinal segments from the esophagus to the anorectum and are often accompanied by anomalies of other organs. The etiology of the different atresias is not uniform and is characterized by disrupted developmental processes, genetic abnormalities or impaired blood supply of the affected organ. All atresias share the need for interdisciplinary care and therapy in specialized centers by pediatric surgeons, pediatricians and representatives of other medical subspecialities experienced in the treatment of these rare anomalies. This is not only important for initial therapy but also for follow-up which is essential for most atresias until adulthood.