Bowel Atresia Research Articles

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

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. 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. 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. 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.

Feasibility of complex minimally invasive surgery in neonates

The aim of this study was to assess the feasibility of complex minimally invasive surgery in neonates (and infants <5 kg). A systematic search of databases was performed. Inguinal herniotomy and pyloromyotomy were specifically excluded. Various operations were assessed for operative times, conversion rates, complications and centres performing the procedures. From 479 abstracts, 50 papers were identified describing 1,215 patients. Common operations performed were fundoplication (n = 473), operations for ovarian pathology (n = 151) and oesophageal atresia repair (n = 123). Other not-so-common operations included Kasai procedure (n = 61), Ladd's procedure (n = 55), bowel atresia surgery (n = 45) and congenital diaphragmatic hernia repair (n = 41). Mean operation times varied from 43 min for fundoplication to 209 min for Kasai procedure, with other operation times falling in between the two extremes. Likewise, conversion rates varied from 0.2% for fundoplication to 15% for congenital diaphragmatic hernia, with other conversion rates falling in between. Complications ranged from 0 to 67%. About 70-94% of the cases were performed in six pioneering centres. Fundoplication and operations for ovarian pathology have low complication and conversion rates and appear to be well suited to the average-trained paediatric laparoscopic surgeon. More complex procedures are feasible and safe, but technically demanding and need further expertise.

Concavo-convex oblique anastomosis technique for jejuno ileal atresia

Aim:To evaluate the role of end-to-end oblique bowel anastomosis in bowel atresia.Methods:End-to-end oblique anastomosis was done in 25 neonates of bowel atresia and the results were compared with traditional method of end to back anastomosis in 25 cases.Results:We found less mortality and morbidity (5%) in our technique as compared to end to back technique (20%).Conclusions:We recommend this technique, as anastomosis is wide with less angulations, flow of effluent is linear, and there is less force exerted over post-anastomotic side wall.

Atresia of ileocecal junction, ileocecal valve: Rare variants of bowel atresia

Atresia of ileocecal junction and isolated atresia of ileocecal valve are rare types of intestinal atresia with very few reports in literature. We report two such cases. Radiology showed dilated ileal segment and distal micro colon in both the cases. At laparotomy there was atresia of ileocecal junction in the first case and isolated ileocaecal valve atresia with normal ileocecal junction in the other case. Both the babies were managed by ileocolic resection with an end to end anastomosis. The prognosis of ileocecal atresias is satisfactory.

Hepatic derangement following N-Acetylcysteine enemas in an infant with cystic fibrosis

We discuss an infant with MI secondary to cystic fibrosis, who was managed surgically by a double barrel ileostomy for mid - small bowel atresia and developed severe faecal impaction in the post - operative period. The faecal impaction was treated successfully with oral NAC and 0.2% NAC contrast enemas. The patient's liver function tests revealed a dramatic increase in transaminases and bilirubin contemporaneous with the administration of the enemas. The levels showed a spontaneous improvement after discontinuation. This is only the second reported case of hepatotoxicity secondary to NAC enemas in the literature. While our experience offers modest support for the use of NAC, its efficacy is not yet proven and paediatric surgeons using NAC in the enema form need to closely monitor liver function contemporaneous with this agent's administration and adjust their treatment accordingly.

Obstructive mesenteric cyst is not always the cause of obstruction

BACKGROUND: In newborns presenting with clinical signs of obstruction of the small bowel, atresia of the small bowel as well as mesenteric cyst can be one of the differential diagnoses. Whereas clinically these two different diagnoses cannot be distinguished from each other, the operative therapy is different. Mesenteric cysts should, if possible, be enucleated, whereas small bowel atresias should be resected and anastomosed. METHODS (CASE REPORT): We describe the case of a newborn, where not only atresias of the small bowel but also multiple mesenteric cysts of the small bowel were intraoperatively found. RESULTS: During surgery multiple mesenteric cysts, which are the cause of small bowel stenosis, were found in the proximal part of the small bowel. Nevertheless, the rest of a small bowel was evaluated and further 2 isolated type I atresias, clearly distally to the mesenteric cysts, were found. CONCLUSIONS: To our knowledge, this has been the first case described where concomitantly small bowel atresia type I and mesenteric cysts were found in one patient. Even knowing that this is a rare combination of diagnosis we would like to point out that an enucleation of mesenteric cyst in case of bowel obstruction might not be enough and that the small bowel should always be carefully evaluated for further pathologies.

Gastroschisis: sonographic diagnosis, associations, management and outcome

Gastroschisis is a defect in the abdominal wall, typically on the right side of a normally inserted umbilical cord through which bowel and other abdominal contents herniate. Classically, no membrane covers the herniated abdominal contents, which distinguishes the defect from exomphalos, an important differential diagnosis. Gastroschisis is usually diagnosed prenatally using ultrasound examination. The prevalence is increasing worldwide from approximately 0.1 per 10,000 total births in the 1970s to over 5 in the early 2000s. The reasons for this are unknown, but factors such as maternal smoking, recreational drugs and young maternal age are strongly associated with the defect. The increasing prevalence is causing concern because the cost of treating gastroschisis is high. Neonatal morbidity depends on significant complicating factors such as bowel atresia or necrosis and prolonged post-operative ileus. Foetuses with gastroschisis are more likely to be born premature and with intra-uterine growth restriction, both of which contribute to the morbidity. Gastroschisis requires early surgery after birth, often followed by prolonged neonatal care. However, advances in surgical and post-operative care in the last decade have meant that currently 90% of affected neonates survive, with few long-term problems.

Prepartum sonographic demonstration of ‘to‐and‐fro’ motion in fetal intestinal obstruction: a novel sign for immediate postnatal surgery

Prepartum sonographic demonstration of ‘to‐and‐fro’ motion in fetal intestinal obstruction: a novel sign for immediate postnatal surgery

A population-based study of the outcome after small bowel atresia/stenosis in New South Wales and the Australian Capital Territory, Australia, 1992-2003

Purpose The purpose of the study was to describe the incidence, epidemiology, and survival of infants with small bowel atresia/stenosis in New South Wales (NSW) and the Australian Capital Territory (ACT), Australia. Methods A population-based cohort study was conducted of infants diagnosed with small bowel atresia/stenosis in NSW and the ACT from 1992 to 2003. Data were obtained from the prospectively collated NSW and ACT Neonatal Intensive Care Units' data collection. Individual risk factors for mortality were assessed using the χ 2 test. Results The incidence of small bowel atresia/stenosis in NSW and the ACT was 2.9 per 10,000 births. Of 299 infants identified with small bowel atresia, 13 were stillborn. Of the 286 live born infants, most (52%) were delivered preterm (<37 weeks' gestation) with an 87% survival, whereas 48% were term with a 98% survival. More than half the infants (54%) had an associated birth defect. The overall mortality was 8%. Prematurity and low birth weight were identified as independent risk factors for mortality ( P < .001). Conclusions This study of small bowel atresia/stenosis provides population-based outcomes for clinicians and families. It is important to investigate infants with small bowel atresia for associated birth defects. Although the mortality rate has decreased over the last 50 years, it remains substantial at 8% and is higher in premature and low birth weight infants.

The outcome of gastroschisis after a prenatal diagnosis or a diagnosis only at birth: Recommendations for prenatal surveillance

Objectives To establish in infants with gastroschisis whether outcome is different when comparing a prenatal diagnosis with a diagnosis only at birth with the intention to develop a prenatal surveillance protocol. Intestinal atresia established after birth and preterm versus term delivery were studied as risk factors. Study design All 24 fetuses and 9 infants diagnosed with gastroschisis and referred to our tertiary center between January 1991 and June 2003 were studied retrospectively. Results The infants of the prenatal subset delivered at our tertiary center and 18 survived. There were two pregnancy terminations, three intrauterine deaths at 19, 33 and 36 weeks respectively and one neonatal death. All nine infants in the postnatal subset survived. Eight were out born and one was delivered at our tertiary center. Prenatal bowel dilatation did not correlate with outcome. Between the prenatal and postnatal subset no significant difference in outcome of live-born infants was established. For four infants with intestinal atresia a significant difference was demonstrated for induction of preterm labour ( P < 0.05), duration of parenteral nutrition ( P < 0.01), number of additional surgical procedures ( P < 0.001) and length of hospital stay ( P < 0.01). The fifteen infants born prior to 37 weeks of gestation spent a significantly longer period in hospital compared to those delivered at term. When the cases with bowel atresia were excluded this difference was no longer present. Five of the 33 cases were diagnosed with associated anomalies which mainly involved the urinary tract. Conclusion Neonatal outcome of live born infants following a prenatal diagnosis of gastroschisis is not different from a diagnosis at birth. The presence of intestinal atresia is the most important prognostic factor for morbidity. The supplemental value of prenatal diagnosis to the outcome of infants with gastroschisis may be in the prevention of unnecessary intrauterine death and detection of intestinal complications. A proposed surveillance protocol for fetuses with gastroschisis focused on intrauterine signs of pending distress such as a dilated stomach, intra abdominal bowel dilatation with peristalsis, notches in the umbilical artery Doppler signal, development of polyhydramnios and an abnormal CTG registration may improve outcome.

Multiple Atresia of the Small Intestine: A 20-Year Review

Multiple intestinal atresia (MIA) presents with a wide spectrum of bowel pathologies. Its treatment is a challenging task since restoration of anatomical continuity of the affected intestine must be balanced against preservation of the intestine's maximal length. A retrospective analysis of the medical notes of 26 patients with MIA treated over a 20-year period between 1986 - 2006 was undertaken with a special emphasis on the clinical and surgical perspectives. All 26 cases of MIA were sporadic with no familial history. The mean gestational age and birth weight were 36.1 weeks and 2781 g, respectively. Twenty-three of the infants underwent operative repair within the first days of life. Three patients with gastroschisis had a delayed diagnosis of bowel atresia. The number of atresias per patient ranged from 2 to 10. In 24 newborns atresias were confined to the small bowel, with 2 other patients having additional obstruction of the ascending colon. Various combinations of anatomical types of atresias were found, with type I and type III occurring in 19 patients each. Type II was diagnosed in 7 newborns. Surgical management of MIA consisted of one-stage restoration of bowel continuity with multiple anastomoses and/or enteroplasties in 22 patients. Four patients had an enterostomy performed at initial operation. Early and late postoperative complications requiring operative treatment occurred in 8 patients. The duration of parenteral nutrition ranged from 6 days to 20 months, exceeding 100 days in 6 children. The follow-up ranges from 3 months to 16 years. All the patients are alive and are on a full oral diet. Clinical observations of sporadic cases of MIA confined to the small bowel lend support to the hypothesis of a vascular incident etiology. One-stage restoration of intestinal continuity with preservation of maximal intestinal length should be the basic principle of any operative management of MIA. Despite a relatively high morbidity related to the primary damage of the fetal intestine, excellent results with 100 % survival rates can be obtained. After taking the differences in pathogenesis, anatomical and histological features, and the prognosis for sporadic and hereditary forms of MIA into account, these two entities should be classified separately in a modified classification of intestinal atresia.

OP09.11: Intra‐abdominal bowel dilatation in fetuses with gastroschisis is associated with bowel atresia or intestinal complications

fetus, so one needs to perform the right scan at the right time; we were lucky to get the last one in 10 minutes. Conclusions: Measuring the distance between the aorta and the trachea is easy to learn and takes less than 20 seconds to obtain, with easier recognition of the landmarks on the parasagittal view than the axial one. Its reliability in assessing the pouch sign in more than 10 000 fetuses is interesting, but the pouch sign is so difficult to visualize that it cannot be utilized as a first sign of pathology. Moreover, it occurs only in atresia without fistula of the upper part of the esophagus.

Gestion anténatale et issue postnatale des fœtus atteints de laparoschisis

To assess the relevance and the quality of gastroschisis's care in a mid level referral centre. A retrospective analysis was performed for infants diagnosed or born with gastroschisis between 1992 and 2003 at the Citadelle hospital, Department of Obstetrics and Gynaecology, University of Liège. Twenty-four cases of gastroschisis were identified. For 22 of them (92%) antenatal sonographic diagnosis was performed at a mean gestational age of 23 weeks. Antenatal diagnosis did not allow to identify additional malformation or chromosomal anomaly. Postnatal diagnosis allows to identify 3 infants with minor cardiac anomalies without functional consequence and one X fragile syndrome. One pregnancy was electively terminated at 24 weeks and one late intrauterine death was reported at 35 weeks. Bowel atresia, stenosis or ischemia were present at birth for 8 cases (33%). Out of 24 cases 22 were live born. 10 infants out of 22 (45%) underwent uncomplicated primary surgical repair. Three infants out of 22 (14%) underwent delayed closure without complications. Nine infants out 22 (41%) underwent multiple surgery (2 to 6). In this group all had postnatal complications, some with multisystem complications, including 3 deaths, 6 with infectious complications, 5 with gastrointestinal complications and 2 with genitourinary or haematological complications. Hospital stay range from 19 to 378 days (median, 51 days). Length of stay and time to full enteral feeding were longer if oligohydramnios or sonographic signs of intestinal damage were found. Among infants born before 35 weeks, only those with intestinal damage at birth had length of stay or time to full enteral feeding longer. Out of 22 live born infants 19 survived (86%) after one year. Survival rate without handicap due to gastroschisis is 84%. Sonographic examination is a valid method for prenatal diagnosis and surveillance. Our survival rate agrees with recent data in the literature. It has to be noticed that hospital stay is lengthy and complications are frequent. The most important prognostic factor is the condition of the bowel at birth and there is no antenatal means to predict severe damage.

Outcome of antenatally diagnosed abdominal wall defects

To examine the natural history and detailed outcome of antenatally diagnosed abdominal wall defects. This was a retrospective review of the antenatal reports, pediatric surgery records and subsequent follow-up information of all cases of omphalocele and gastroschisis diagnosed in a 10-year period in our tertiary referral center. There were 109 cases of abdominal wall defects, including omphalocele in 67 cases and gastroschisis in 42 cases. Of the 67 cases of omphalocele there were 26 (39%) with chromosomal abnormalities and 22 (33%) underwent termination of pregnancy, mainly for associated structural abnormalities. Of the ongoing 19 cases there were five (26%) in-utero deaths, 12 (63%) survivors and two (11%) neonatal deaths, both associated with prematurity. Excluding chromosomal abnormalities, the survival rate in isolated omphalocele was 7/16 (44%) whilst it was 5/25 (20%) in those with associated abnormalities. Gastroschisis was isolated in 40 (95%) cases. Among these 40 isolated cases there were two (5%) terminations. Of the 38 ongoing cases, there were two (5%) in-utero deaths, and 36 (95%) live births. Four of the 36 liveborn infants (11%) died in the postoperative period owing to complications of small bowel atresia. Although only 18% of infants with antenatally diagnosed omphalocele were alive in the neonatal period, postoperative morbidity was low. The majority (90%) of fetuses with antenatally diagnosed gastroschisis survived to delivery, but the mortality in affected newborns was 11%.

Postoperative pyloric stenosis in the newborn: a forgotten problem

Purpose Postoperative vomiting in newborns is a common finding usually attributed to the original surgery. We report 10 newborns whose postoperative course was complicated by pyloric stenosis. Methods We reviewed the charts of all newborns who had pyloric stenosis diagnosed after a major abdominothoracic operation over the past 30 years (1973-2003) at a single children's hospital. Results Ten cases of pyloric stenosis were diagnosed in postoperative newborns (esophageal atresia [3], small bowel atresia [2], diaphragmatic hernia, fetus-in-fetu, imperforate anus, lung biopsy, malrotation [1]). Their mean gestational age was 36 weeks, birth weight was 3.2 kg, and age at newborn operation was 2 days. Their postoperative feeds started on the sixth day (mean), and the pyloric stenosis vomiting started on the 14th day. It took a mean of 12 days to make the diagnosis of pyloric stenosis, and this was done entirely by diagnostic imaging. Mean age at pyloromyotomy was 3 1/2 weeks. A new incision was used in 7 patients. Postoperative feedings started on the first day; all did well. There were 3 complications (bowel obstruction), all requiring operation. Conclusion Unfortunately, this review offers no specific insight or novel advice to help the readership think about the diagnosis in such a postoperative setting.

Factors determining outcome in gastroschisis: clinical experience over 18 years

Although the survival in gastroschisis has improved to more than 85%, serious morbidity with consequent long hospital stay is still very common. Alternative strategies such as planned early delivery and amniotic fluid exchange are being explored. In order to evaluate these approaches, adequate risk stratification of patients with gastroschisis is required. The aim of this study was to identify the risk factors associated with increased morbidity and mortality. A retrospective analysis of all patients with gastroschisis treated at our institution between 1988 and 2005 was conducted. Patients' data were collected from the files and compared for statistical significance with three outcome measures: (1) complicated post-operative course; (2) time to full enteral feeding (TFE); (3) length of hospital stay (LOS). SPSS was used for statistical analysis. Fifty-nine newborns with gastroschisis were admitted during the study period. Antenatal diagnosis was made in 40 cases (68%). Associated malformations (other than intestinal anomalies) were present in eight patients. One patient with trisomy 13 was excluded from the analysis. Ten patients (17%) had compromised bowel (atresia, severe ischaemia or a combination). Primary repair was successful in 47 (81%) and in 11 patients a silo was applied. Overall survival was 54/58 children (93%), four patients died. Complications associated with closure occurred in eight patients. Post-operative course was uncomplicated in 45 patients and complicated in 13. The RR for patients with compromised bowel to have a complicated post-operative course is 9 (95% CI 2-39) and the RR to die is 20 (95% CI 2-222) for these patients. Risk stratification of patients with gastroschisis appears possible based on the presence of compromised bowel before closure.

Developmental changes of the adrenergic network in the myenteric plexus of the porcine small bowel

Knowledge about the foetal development of the normal enteric nervous system (ENS) is crucial for the understanding of congenital and acquired functional abnormalities of the gut. The ENS is the largest and most complex division of the peripheral nervous system and consists of intrinsic and extrinsic components. Although previous studies have described sympathetic innervation of the myenteric plexus, little is known regarding its age-related changes. The aim of this study was to investigate the age-related changes in the sympathetic innervation of the myenteric plexus. Whole mount and paraffin sections of the small bowel specimens from six different age groups (60 and 90 days gestation; newborn; 4 and 12 weeks old; and adult) were stained using tyrosine hydroxylase immunohistochemistry. Specimens were then analysed using fluorescence and laser scanning microscopy in detail. The tyrosine hydroxylase positive nerve fibres were first seen within the myenteric plexus at 90 days of gestation (E90). There was a significant increase in nerve fibres and varicosities observed from E90 to 12 weeks of age and stabilisation thereafter. The degree of varicosities around the ganglia, clearly seen on the whole-mount preparations, was also noted to increase up to 12 weeks of age, after which time there was no general variation noted into adulthood. Our findings show, for the first time, that sympathetic innervation of the myenteric plexus starts in the last quarter of gestation and continues till 12 weeks of age. Segmental sympathetic denervation, following bowel resection and anastomosis, during this developmental period may explain the motility dysfunction seen in newborn infants operated for necrotising enterocolitis, bowel atresia and Hirschsprung's disease.

Cholestasis associated with small bowel atresia: do we always need to investigate?

Background/Purpose Cholestasis occurs frequently in patients with small bowel atresia (SBA) and is often attributed to prolonged parental nutrition. When severe or prolonged, patients may undergo unnecessary intensive or invasive investigation. We characterized cholestasis and analyzed the pertinence of investigating this patient population. Methods With Research Ethics Board approval, patients with SBA between 1996 and 2005 were retrospectively reviewed. Demographics, location of atresia, operative findings, complications, investigations, resumption of feeding, duration of prolonged parental nutrition, and follow-up information were examined. Cholestasis was evaluated for incidence, severity, and evolution. Results Fifty-five patients (29 male, 26 female), with a median gestational age and birth weight of 36 weeks and 2025 g, respectively, were reviewed. Care was withdrawn for 2 patients before repair. For the remaining 53 patients, SBA were duodenal atresia in 18, jejunoileal atresia in 32, and multiple atresia in 3. Of 53, 24 (45%) patients developed cholestasis postoperatively (direct/total bilirubin >20%). All patients with short bowel (4) and 60% (6/10) of patients with a delay of enteral feeding more than 14 days postoperatively had cholestasis. Ten patients (36%) proceeded with in-depth evaluations for cholestasis, with 8 (28%) undergoing liver biopsy. No patient had biliary atresia. No deaths were related to isolated cholestasis/cirrhosis. Cholestasis resolved spontaneously in all the survivors. Conclusions Small bowel atresia is frequently associated with postoperative cholestasis that will resolve with time. We recommend a more selective and expectant approach to SBA-associated cholestasis to minimize unnecessary investigations.

Intestinal transplantation for short bowel syndrome secondary to gastroschisis

Background/Purpose Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome. Methods A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994. Results Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years. Conclusions Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.

Second‐trimester intra‐abdominal bowel dilation in fetuses with gastroschisis predicts neonatal bowel atresia

To determine in fetuses with gastroschisis the association between intra-abdominal bowel dilation in the second trimester and neonatal bowel atresia. We reviewed ultrasound and medical records of fetuses with gastroschisis from January 1998 to August 2004. Fetuses with intra-abdominal bowel dilation in the second trimester were identified and followed into the neonatal period. We identified 58 mother-infant pairs showing fetal gastroschisis, with at least one prenatal ultrasound at our hospital and which were delivered there, or were transported there as newborns. Forty-eight of the 58 fetuses had no intra-abdominal bowel dilation and none of these neonates had bowel atresia. Ten of the 58 fetuses had intra-abdominal bowel dilation and all had bowel atresia at birth (P<0.0001). In eight cases in which ultrasound was performed at <25 weeks' gestation, intra-abdominal bowel dilation was already present. Intra-abdominal bowel dilation in the second trimester predicts neonatal bowel atresia in fetuses with gastroschisis.