Intestinal atresia

Intestinal atresia accounts for about one third of all cases of neonatal intestinal obstruction. The survival rate has improved to 90% in most of the series with the operative mortality being <1%. The survival rate improves with distal atresias. An increased mortality is observed in multiple atresias (57%), apple peel atresia (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%) and gastroschisis (66%). Although appearance of echogenic bowel on prenatal ultrasonography is suggestive of GI, it is confirmed in only 27% cases. Prenatal ultrasonography is more reliable in detection of dudenal atresia than more distal lesions. Short bowel syndrome is the major impediment in the management of jejunoileal atresia. Although total parenteral nutrition (TPN) is the main adjunctive treatment, it delays intestinal adaptation and may cause cholestasia and subsequent liver damage. Graduated enteric feedings, use of growth hormone, glutamine and modifed diets containing low fat, complex carbohydrates and protein supplements have been used in a adults with short bowel syndrome to successfully diminish TPN requirements and enhance nutrient absorption in nearly half of the patients. Utilization of growth factors to facilitate intestinal adaptation and advances in small bowel transplant may improve the long-term outcomes in future.     

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     

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     

Colonic atresia: spectrum of presentation and pitfalls in management. A review of 14 cases

Fourteen cases of colonic atresia seen over a 38-year period are reviewed with particular reference to clinical presentation and pitfalls in management. Seven had Type I atresia, two Type II and five Type IIIa. Ten had associated gastrointestinal anomalies. Management varied considerably. Six had primary colonic anastomosis. Two of these developed complications due to unrecognized distal hypoganglionosis, two had associated jejunal atresias resulting in short bowel syndrome, and two had primary anastomosis protected by proximal ileostomies. Seven had a staged repair with initial defunctioning enterostomy with only one complication, an unfixed mesentery that later resulted in midgut volvulus. The only mortality was a patient in which a jejunal atresia repair leaked as a result of a missed colonic atresia. Operative strategy should depend on the clinical state of the patients, the level of atresia, associated small bowel pathology and exclusion of distal pathology. Primary anastomosis would only rarely be advised with a circumspect approach. Long-term outlook, as in small bowel atresia is generally excellent. An erratum to this article can be found at     

Cycle loading tissue expansion of rat jejunum

Cycle loading was applied to 40 Sprague-Dawley rat jejunums to increase their diameters using a balloon catheter filled with saline over a 30-min period, with each load lasting 3 min at 1-min intervals. The dilations were performed using 0.2 ml saline in group I, 0.4 ml saline in group II, 0.6 ml saline in group III, and no dilation in the control group. The percentage increments of increase in external jejunal diameter were 10.52 in group I, 48.38 in group II, and 68.08 in group III. Histologic changes were reversible in groups I and II and irreversible in group III. It is suggested that cycle-loading tissue expansion could be used as an alternative method for the treatment of intestinal atresias to correct discrepancies in the caliber of gastrointestinal lunens.     

Intestinal atresia: an end-to-end linear anastomotic technique

In a series of 19 neonates with small-bowel atresia, 16 were treated by end-to-end linear anastomosis (ELA) without resection and 3 by resection anastomosis (RA). Seven atresias were jejunal, 11 were ileal, and 1␣jejunoileal; 3 cases were type II, 12 type IIIa, 3 type IIIb, and 1 type IV. There were 4 deaths, 1 after ELA and 3 after RA. The overall mortality decreased from 68 to 20.80% and for linear anastamosis to 6.25% presumably, because the intestinal contents are propelled along the lumen in a linear fashion and not at an angle as in end-to-back anastamosis, avoiding shearing of the suture line. The additional plicating sutures reduce the radius and increase the propelling force. We recommend this technique because it is based on sound principles of physics and preserves the entire available length of intestine. Accepted: 8 January 2001     

Notochord-gut failure of detachment and intestinal atresia

A spectrum of congenital anomalies have been described in an adriamycin-treated model with common features to the human pattern. Multiple intestinal atresias was part of this spectrum occurring in 25% of full-term experimental rat fetuses. The aim of this study was to examine the underlying developmental mechanism that results in intestinal atresia. Virgin timed-pregnant Sprague-Dawley rats were injected with Adriamycin i.p. at a dose of 2 mg/kg on days 6–9 of gestation. Embryos were removed on different gestational days during organogenesis and serial transverse histologic sections were examined and compared with control specimens. In experimental embryos, hindgut atresia was seen in day 12 embryos. Attachment of the intestine with the notochord was obvious observation resulting in abnormal position of the intestine. In some specimens the atretic intestine was splitting the dorsal aorta or even located behind the dorsal aorta. It is concluded that in the adriamycin-animal model, notochord–intestinal failure of detachment resulted in intestinal atresia during the beginning of organogenesis period. The possible underlying mechanisms are pinching of some endodermal cells as well as interference with normal intestinal circulation resulting in ischemic necrosis.     

Embryogenesis of adriamycin-induced hindgut atresia in rats

It was proposed that the pathogenesis of multiple intestinal atresias (MIA) in human fetuses is a consequence of malformative processes of the gastrointestinal tract rather than an ischemic process. Recently, MIA has been described in adriamycin-exposed rat fetuses. The aim of this study was to describe the embryogenesis of hindgut atresia (HA) in the adriamycin animal model. Timed-pregnant Sprague-Dawley rats were injected with adriamycin on days 6–9 of gestation. Embryos were removed on different gestational days during organogenesis and histologic sections were examined and compared with control specimens. In experimental embryos, HA was seen on day 13; however, the lumen was patent on day 12. HA was associated with abnormal vascular anatomy that was obvious on days 12 and 13. It is concluded that HA in adriamycin-exposed embryos occurs at the beginning of organogenesis. Although it was associated with an obvious vascular anomaly, further studies are required to find out whether it is ischemic in origin. Accepted: 26 March 2001     

Multiple Congenital Septal Atresias of the Intestine: Histomorphologic and Pathogenetic Implications

Congenital atresias of the gastrointestinal tract are usually single and divided into three forms on a morphologic basis. The septal or diaphragmatic type (type I) is the least common. We report an infant with multiple type I atresias involving both the small and large intestine and describe the unique histologic features of the septa: (1) fragmentation of muscularis mucosa, (2) multiple septal cysts lined by columnar epithelium, (3) circular and longitudinal muscular layers, and (4) absence of inflammation. This infant had a sibling who died with multiple intestinal atresias of the septal type.     

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.     

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     

Congenital malformations and perinatal morbidity associated with intestinal neuronal dysplasia

A close relation between different forms of dysganglionosis such as intestinal neuronal dysplasia (IND) type B and aganglionosis has been established. No systematic analysis of other malformations and diseases accompanying IND has been made as yet. Congenital malformations and perinatal morbidity were analyzed in 109 patients with IND seen at the Department of Pediatric Surgery in Mainz from 1977 to 1996. IND was associated with Hirschsprung's disease in 47 cases; 22 children with IND had other abdominal malformations, including anal atresia, rectal stenosis, sigmoidal stenosis, ileal atresia, pyloric stenosis, and esophageal atresia. A cystic bowel duplication, a choledochal cyst, and a persisting urachus were also found. Extra-abdominal malformations such as Down's syndrome, congenital diaphragmatic hernia, aortic stenosis, and malformations of vertebral bodies were seen. Twin siblings of children with IND were either healthy (n=3) or died in utero (n=1). Seventeen children with IND developed severe intra-abdominal complications during the perinatal period such as necrotizing enterocolitis (NEC), meconium ileus, or bowel perforations. NEC was frequently associated with preterm birth. Bowel perforations were seen in mature and preterm newborns with IND. Taken together, IND is found in a variety of obstructive bowel diseases. This may support the hypothesis that IND is a secondary phenomenon or that congenital atresias and stenoses of the digestive tract have a pathogenesis similar to that of intestinal innervation disturbances. IND may also be a part of complex malformation patterns since it occurs with a number of extraintestinal and non-obstructive intestinal malformations.     

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.     

Pyloric atresia associated with duodenal and jejunal atresia and duplication

A case of congenital pyloric atresia associated with duodenal atresia, jejunal atresia, and a duplication is described. A review of the literature revealed nine previously reported cases of congenital pyloric atresia and multiple intestinal atresias, but this is the first report of such an association and a duplication. Accepted: 13 July 1998     

Duodenal motility after tapering duodenoplasty for high jejunal and multiple intestinal atresia

Duodenal motility after tapering duodenoplasty was investogated by radiography and video-fluoroscopy. Two patients underwent tapering duodenoplasty, one at the time of duodenojejunostomy for double high jejunal atresias and another 6.5 months after the restoration of intestinal continuity for multiple intestinal atresias. Both patients showed a satisfactory postoperative course and were doing well. Radiographic and video-fluoroscopic studies demonstrated that the tapered portion of the duodenum had restored active peristalsis and excellent passage of contrast material, while the proximal, non-tapered portion remained dilated and dismotile soon after the operation. Tapering duodenoplasty proved to be a valuable procedure for restoring duodenal motility. Correspondence to: M. Honzumi     

Gastrointestinal maturation and implications for infant feeding

The level of gastrointestinal (GI) maturity of an individual infant is a major determinant of whether the infant will be able to meet nutritional needs by sole use of the GI tract or if parenteral means will be necessary. The GI tract is not only an organ for digestion and absorption of nutrients; it also performs major endocrine, neural and immunologic functions. In this review, anatomic, functional and biochemical development will be described and related to means by which enteral nutrition can be used in the prematurely born infant to optimize health and prevent disease.     

Application of endoscopic Ho:YAG laser incision technique treating urethral strictures and urethral atresias in pediatric patients

Endoscopic holmium:yttrium–aluminum–garnet (Ho:YAG) laser incision is a new method applied in pediatric urology recent years. To evaluate its therapeutic efficacy on treating the pediatric patients with urethral strictures and urethral atresias, a retrospective study was performed from June 2001 to July 2005 in a total of 28 pediatric patients who underwent endoscopic internal urethrotomy using Ho:YAG laser in our center. In these patients, 25 had urethral strictures and 3 urethral atresias. Follow-up was done ranging from 2 months to 4 years to assess the treatment. Of the 28 patients, 25 (89.3%) have achieved satisfied result without complications following initial incisions. Two patients with urethral atresias and another with long lesion of stricture (> 2 cm) have postoperative stenosis (10.7%). Among the three reoccurred patients, two were successfully reoperated by Ho:YAG laser and open end-to-end anastomosis, respectively. One patient failed to follow-up. With the advantages of safety, efficacy and minimal invasion, endoscopic Ho:YAG laser incision technique could be used as a primary treatment in urethral stricture patients and is worthy to be popularized further in pediatric surgery.     

Implications of antenatal diagnosis of small-intestinal atresia in the 1990s

To assess the prevalence of antenatal diagnosis of small-intestinal atresias (SIA) in the modern era and determine its effect on management and outcome, the records of neonates admitted to a single institution in 1991–1996 with a diagnosis of SIA were reviewed. Duodenal atresia, atresias complicating meconium ileus, and those associated with gastroschisis were excluded. Of 14 neonates with SIA, 10 had jejunal atresia (JA) (Grosfeld type I, n = 1; type II, n = 2; type IIIa, n = 3; type IIIb [apple peel], n = 3; type IV, n = 1) and 4 had ileal atresia (IA) (type II, n = 1; type IIIa, n = 3). Antenatal diagnosis was made in 4 neonates (overall rate = 28%), including all 3 type IIIb JA, and 1 type II JA. None of the IAs were diagnosed antenatally. There were 2 deaths, both in patients with high JAs with less than 10 cm viable bowel. Only 1 of these was antenatally diagnosed. The median (range) times to full enteral feeding were 20 days (17–22) for antenatally-diagnosed JA, 14 days (11–26) for other JAs, and 8 days (6–15) for IAs. Antenatal diagnosis of SIA thus remains relatively infrequent (less than one-third of cases). When an antenatal diagnosis is made, the atresia is more likely to be proximal in location, requiring intensive and prolonged postnatal treatment. Provided a reasonable length of bowel remains, the outcome of SIA, whether antenatally diagnosed or not, is favorable. Accepted: 4 December 1998     

Gastrointestinal malformations associated with prune belly syndrome: three cases and a review of the literature

Prune belly syndrome (PBS), a triad consisting of abdominal musculature hypoplasia, urinary tract malformations, and cryptorchidism, is frequently associated with other congenital malformations. Although it is acknowledged that gastrointestinal (GI) malrotation and mesenteric anomalies are frequent in PBS, other GI anomalies are generally considered to be exceedingly rare. Here we describe 3 autopsy cases with severe malformations of both midgut and hindgut derivatives and review the world literature to evaluate the spectrum of GI malformations associated with this syndrome. The relatively high frequency of distal stenoses and atresias suggests that the anomalous mesenteric attachments may predispose to prenatal volvulus and subsequent anatomic bowel obstruction. Postnatal volvulus is also occasionally observed. Infants with PBS also appear to be at a higher risk for persistence of the common fetal cloaca.     

Long-gap esophageal atresia: reconstruction preserving all portions of the esophagus by Schärli's technique

For distances of over 4–5 cm, esophageal replacement is almost always necessary in esophageal atresias. We present the technical details and describe our experience with esophageal reconstruction by elongation of the lesser curvature (Schärli's technique) in four cases of very long atresias. A retrosternal transposition was made without a thoracotomy in two children, and an orthotopic mediastinal route through a right thoracotomy was done in two others. There were two main complications: anastomotic leaks in three patients that closed spontaneously, and too-rapid gastric emptying, resulting in dumping symptoms that improved with time and diet. One patient developed an anastomotic stricture that responded to bouginage, while another had temporary feeding problems. Esophageal reconstruction by elongation of the lesser curvature provides a relatively simple method of esophageal replacement in children in that all portions of the esophagus are preserved. We propose this technique for early establishment of esophageal continuity in neonates.