Small Bowel Atresia Research Articles

Anorectal Malformation Associated with Small and Large Bowel Atresias: A Rare Association

Journal of Neonatal Surgery is a peer-reviewed and open-access electronic journal. Only a few journals are being published on Neonatal Surgery especially from a developing country, which justifies the need of a new journal on this subject.

Changes of smooth muscle contractile filaments in small bowel atresia

To investigate morphological changes of intestinal smooth muscle contractile fibres in small bowel atresia patients. Resected small bowel specimens from small bowel atresia patients (n = 12) were divided into three sections (proximal, atretic and distal). Standard histology hematoxylin-eosin staining and enzyme immunohistochemistry was performed to visualize smooth muscle contractile markers α-smooth muscle actin (SMA) and desmin using conventional paraffin sections of the proximal and distal bowel. Small bowel from age-matched patients (n = 2) undergoing Meckel's diverticulum resection served as controls. The smooth muscle coat in the proximal bowel of small bowel atresia patients was thickened compared with control tissue, but the distal bowel was unchanged. Expression of smooth muscle contractile fibres SMA and desmin within the proximal bowel was slightly reduced compared with the distal bowel and control tissue. There were no major differences in the architecture of the smooth muscle within the proximal bowel and the distal bowel. The proximal and distal bowel in small bowel atresia patients revealed only minimal differences regarding smooth muscle morphology and the presence of smooth muscle contractile filament markers. Changes in smooth muscle contractile filaments do not appear to play a major role in postoperative motility disorders in small bowel atresia.

US assessment of neonatal bowel (necrotizing enterocolitis excluded)

US plays a main role in evaluating neonatal gastrointestinal disorders. The authors describe the US appearance of normal neonatal bowel, midgut volvulus, small bowel atresia, meconium ileus, meconium peritonitis, different meconium plug syndromes, Hirschsprung disease, intestinal duplication cysts, some other abdominal cysts, some intraperitoneal infections, excluding necrotizing enterocolitis, and anorectal anomalies. The use of sonography in the imaging diagnostic strategies of these congenital and acquired anomalies is emphasized.

494: Antenatal intra-abdominal bowel dilation in the prediction of postnatal bowel atresia in fetuses with gastroschisis

Given the dramatic difference in postnatal hospital course in the setting of bowel atresia with gastroschisis, we aimed to estimate the association between antenatal intra-abdominal bowel dilation (IABD) and postnatal small bowel atresia in gastroschisis and to evaluate its predictive ability. A retrospective cohort study of singleton gestations with an antenatal diagnosis of gastroschisis seen in our ultrasound unit from 2001-2010. Stored images were reviewed, blind to postnatal outcomes, from the last ultrasound exam before delivery, and the transverse diameter of the largest dilated loop of intra-abdominal small bowel measured. Previously published definitions of IABD including >6mm, >10mm, and >14mm were evaluated for association with the primary outcome of bowel atresia. Secondary outcomes included neonatal intensive care unit (NICU) length of stay, necrotizing enterocolitis (NEC), time to complete closure, time on total parenteral nutrition (TPN), and time to initial and full enteral feeding. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and area under the receiver operating characteristic curve (AUC) were calculated. Of 94 consecutive live births with gastroschisis, 39 (41.5%) had IABD at their last ultrasound. The range of IABD was 7mm-75mm. There were 14 (14.9%) cases of bowel atresia. There was no significant association between IABD and bowel atresia when thresholds of >6mm and >10mm were used to define IABD; however, there was a significantly increased risk of bowel atresia with IABD>14mm (28.6% v. 9.1%, RR 3.1, 95% CI 1.2-8.2). Sensitivity, specificity, PPV, and NPV for IABD>14mm and bowel atresia were 57.1%, 75.0%, 28.6%, and 90.9%, respectively. The AUC for IABD and bowel atresia was 0.73 (95% CI 0.57-0.89). IABD>14mm is associated with an increased risk for bowel atresia; however, the PPV remains low due to the rarity of the finding. Given the high NPV, patients can be counseled that in the absence of this finding, the risk of postnatal bowel atresia is low for their neonate with gastroschisis. Tabled 1

Multiple intestinal atresias associated with angiodysplasia in a newborn

This is a report describing the association of multiple small bowel atresias with multifocal angiodysplasia of the intestinal wall in a newborn. To the authors' knowledge, such association has never been reported.

Index of Suspicion in the Nursery

A woman presents with decreased fetal movement, and ultrasonography reveals hydrops fetalis. Specific findings include massive ascites and a small pericardial effusion that was not present on ultrasonography 1 week ago. A 37 weeks' gestation boy is delivered via emergent cesarean section. Apgar scores are 7 and 9 at 1 and 5 minutes, respectively. The baby is intubated endotracheally 10 minutes after delivery because of increased work of breathing from his markedly distended abdomen and transported to the neonatal intensive care unit (NICU). Physical examination findings are normal, except for increased work of breathing and a tight, distended abdomen with visibly dilated veins. Initial laboratory results include a white blood cell count of 18.3×103/mcL (18.3×109/L), hemoglobin of 9.7 g/dL (97 g/L), and platelet count of 331×103/mcL (331×109/L). Chemistry results are within normal limits. Blood is sent for cultures, and empiric therapy with ampicillin and gentamicin is initiated. The baby also receives a 15-mL/kg packed red blood cell transfusion. A Replogle tube is placed to low intermittent suction. The infant is made NPO and started on intravenous fluids. Paracentesis is performed to relieve the pressure on his diaphragm and improve breathing, and 755 mL of green/brown fluid with particulate matter that has the appearance of meconium is aspirated. Umbilical lines are placed and pediatric surgery is consulted. Abdominal radiography shows intra-abdominal calcifications. An 11-day-old term girl whose mother had a positive cervical culture for human papillomavirus (HPV) is referred for admission because of a rectal temperature of 38.6°C and a history of “gasping for air.” Four days after birth, the baby became fussy, especially with feedings, and developed an intermittent cough and hoarse cry. Over several days, she became sleepier, only waking every 4 to 5 hours to breastfeed. On the pediatric ward, the baby …

Alterations of Cajal cells in patients with small bowel atresia

Purpose Interstitial cells of Cajal (ICC) are regarded as the pacemaker cells of the gastrointestinal tract. There are some well-designed studies investigating the structure and function of ICC subsequent to experimentally induced intestinal obstructions. However, it remains unclear whether reduction of number of ICC primarily leads to mechanical obstruction of the bowel such as seen in intestinal atresia. We aimed to investigate the number of ICC in proximal and distal parts of the atresias of patients with small bowel atresia. Patients and Methods Twenty-one patients (13 male and 8 female; median age, 3 days; median gestation age, 38 weeks) with jejunal or ileal atresia underwent primary repair between 2001 and 2009. The demographic data were reviewed. The specimen of the distal and proximal parts of the atretic segments was investigated according to presence and number of ICC in the myenteric plexus using immunohistochemical methods. The jejunum segments of 14 newborns who died from causes other than bowel disease were examined as a control. Scoring and count systems were developed for the evaluation of ICC. A continuous layer of CD-117 immunoreactive Cajal cells around the myenteric plexus was scored as 3, whereas discontinuous and diminished Cajal cells were scored as 2. Few and sparse Cajal cells around the myenteric ganglia and in the muscle layer were scored as 1. If there was no Cajal cell at all, it was scored as zero. In addition, the number of ICC per field was counted. The scores and the numbers of ICC per field were compared in patients with small bowel atresia and control group. Results All patients but one survived. One patient was lost because of congenital cardiac anomalies. The median score of control subjects was 3 (range, 1-3). Both the proximal and distal segments of the atretic bowel had a median score of 1 in patients with atresia. Twenty patients' score of proximal (95%) and 19 patients' score of distal bowel segment (90%) had an ICC score of 2 or less. Only 1 control subject (7%) had an ICC score of less than 2. Results were statistically significant in controls and patients. The mean number of ICC in the control group was 5.36 ± 2.36; in distal segments of patients with atresia, it was 1.03 ± 1.4; and in proximal s\\egments, it was 0.82 ± 1.56. The difference between the control group and the patients was statistically significant ( P < .05). Conclusion We demonstrated a remarkable decrease of ICC in small bowel wall of patients with intestinal atresia; but we could not show whether the reduction of ICC is a primary event, which also participates in the pathogenesis of intestinal atresia, or whether the mechanical obstruction caused by any unknown etiology (eg, ischemia) leads to decrease in number of ICC.

Hereditary multiple intestinal atresias: 2 new cases and review of the literature

Intestinal atresias are a common cause of newborn bowel obstruction (Dalla Vecchia LK, Grosfeld JL, West KW, et al, Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133[5]:490-496). Hereditary multiple intestinal atresias, first reported by Guttman et al in 1973, is the rarest form of multiple atresias (Guttman FM, Braun P, Garance PH, et al, Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum. J Pediatr Surg 1973;8:633-640; Bass J, Pyloric atresia associated with multiple intestinal atresias and immune deficiency. J Pediatr Surg 2002;37:941-942.). It has been proposed to be autosomal recessive, to involve atresias in a variable combination of sites from stomach to rectum, and to be universally fatal (Bilodeau A, Prasil P, Cloutier R, et al, Hereditary multiple intestinal atresia: thirty years later. J Pediatr Surg 2004;39:726-730; Moreno LA, Gottrand F, Turck D, et al, Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family. Am J Med Genet 1990;37:143-146). Patients have significant intestinal dysfunction and unrelenting sepsis stemming from a poorly defined, severe immunologic defect. Our case report presents 2 full siblings to nonconsanguineous parents with pyloric atresia, multiple small bowel and colonic atresias, and severe immune dysfunction. Care was withdrawn within 3 months of life on both siblings after multiple bouts of sepsis. Data suggest that the immune defect may not be primary, but in fact be secondary to intestinal dysfunction. Although the subjects in this article ultimately had fatal outcomes, a comprehensive immunologic/physiologic picture is presented in hopes of furthering the understanding of this grave disease.

Congenital malformations in infants born after in vitro fertilization in Sweden

The risk for congenital malformations is increased in infants born after in vitro fertilization (IVF). Some specific malformations appear to be more affected than others. The presence of congenital malformations in 15,570 infants born after IVF with an embryo transfer between April 1, 2001, and the end of 2006 were compared with all infants born in Sweden during 2001 to 2007 (n = 689,157). Risk estimates were made after adjusting for year of birth, maternal age, parity, smoking, and body mass index. The risks of specific malformations were compared with data from a previous study (1982 to March 31, 2001) of 16,280 infants born after IVF. Different IVF methods were compared to respect to malformation risk. Increased risks of a similar magnitude were found for most cardiovascular malformations and limb reduction defects for both study periods. For neural tube defects, cardiac septal defects, and esophageal atresia, there was still an increased risk, but it was lower during the second than during the first period. For small bowel atresia, anal atresia, and hypospadias, the risk increase observed during the first study period had disappeared during the second period. An increased risk was seen for some syndromes that have been associated with imprinting errors. No difference in malformation risk according to IVF method was apparent. A slightly increased risk for congenital malformations after IVF persists. A decreasing risk is seen for some specific malformations, either true or the result of multiple testing.

Differential changes in intrinsic innervation and interstitial cells of Cajal in small bowel atresia in newborns.

To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel atresia. Resected small bowel specimens from affected patients (n = 7) were divided into three parts (proximal, atretic, distal). Standard histology and enzyme immunohistochemistry anti-S100, anti-protein gene product (PGP) 9.5, anti-neurofilament (NF), anti-c-kit-receptor (CD117) was carried out on conventional paraffin sections of the proximal and distal part. The neuronal and glial markers (PGP 9.5, NF, S-100) were expressed in hypertrophied ganglia and nerve fibres within the myenteric and submucosal plexuses. Furthermore, the submucous plexus contained typical giant ganglia. The innervation pattern of the proximal bowel resembled intestinal neuronal dysplasia. The density of myenteric ICCs was clearly reduced in the proximal bowel, whereas a moderate number of muscular ICCs were found. The anti-CD117 immunoreaction revealed additional numerous mast cells. The distal bowel demonstrated normal morphology and density of the ENS, the ICCs and the mast cells. The proximal and distal bowel in small bowel atresia revealed clear changes in morphology and density of the ENS and ICCs.

Colonic Atresia: A Clinicopathological Insight into its Etiology

The pathogenesis of colonic atresia (CA) has not been discussed in detail, although the theory of a prenatal vascular insult, hypothesized from patients with small bowel atresia, has been accepted in the past. This review questions this etiology by reviewing all patients with CA treated in two institutions. A retrospective analysis was done of the medical notes of 30 patients with CA treated in two tertiary centers of neonatal surgery in Glasgow, UK, and in Wroclaw, Poland, over a 30-year period with special emphasis on the intraoperative findings and morphology of the atretic bowel. Thirteen patients had CA in association with an abdominal wall defect. Eleven patients had gastroschisis. A single fibrous cord atresia was noted in 2 patients and it was located in the ascending colon and transverse colon, respectively. Four patients had type IIIa atresia affecting the ascending colon in 3 and transverse colon in one. In 5 patients an extensive defect of the intestine with two atresias of the small and large bowel was noted. In 3 of them, a separate conglomerate of the prolapsed intestine was found to be necrotic or presenting as a cystic structure. Two of these patients had an unusually narrow abdominal wall defect of less than 1 cm. Isolated CA was noted in 17 patients. A type IIIa atresia affecting the right colon was found in 14 of them. In 2 patients the ascending colonic atresia was accompanied by an extensive defect of the transverse colon. A single sigmoid colon atresia and two fibrous cord atresias of the sigmoid and transverse colon were noted in single patients respectively. Most cases of CA in babies with gastroschisis seem to result from bowel compression within the narrowing abdominal defect. A "two-point constriction" may lead to a wide spectrum of bowel pathologies and the morphology may depend on the viability of the intestinal segment between the atretic jejunum or ileum and the colon. Isolated CA presents with a wider spectrum in terms of anatomical types and location of the bowel pathology, but it seems that type II and IIIa atresias of the right colon may share a similar pathogenesis of temporary constriction within a closing umbilical ring.

P22.06: Prenatal bowel dilatation and congenital chloride diarrhea: a case report

Congenital chloride diarrhoea (CCD) is a rare autosomal recessive disorder in which the transport of chloride in the distal ileum is disrupted. This is medically treatable condition but is easy to be misdiagnosed as a surgically treatable condition such as small-bowel atresia. A 28-year-old primigravida woman was referred at 34 weeks 2 days for evaluation of fetal dilated bowel loops and polyhydramnios. Her familial and medical histories were unremarkable. A sonographic examination showed a single fetus with generalized dilatation of the bowel in right side of the abdomen and polyhydramnios (amniotic fluid index, 30.48). Serial follow-up scans showed no notable change. Fetal growth was appropriate for the gestational age. We suspected distal small bowel obstruction or ileal atresia and consulted to pediatric surgery for postnatal surgery. At 37 weeks 2 day, she was admitted for premature rupture of membrane. The next day she had an uneventful spontaneous delivery of a female infant. After birth the neonate had slightly distended abdomen and yellowish watery diarrhoea without meconium passage. An infantogram after birth showed mild bowel gas distension without evidence of intestinal obstruction, small bowel series showed no small bowel transit time delay and diffuse gas distended colon without obstruction. She had hyponatremic metabolic alkalosis and hypochloridemia. The stool chloride concentration was high, 101 mmol/L (reference value < 90 mmol/L), confirming the diagnosis of CCD. She was discharged with sodium chloride for electrolyte correction. Supporting information can be found in the online version of this abstract. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

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.

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.

Large congenital transmesenteric hernia: a missed small-bowel atresia?

Internal hernias are uncommon diagnoses and represent rare causes of intestinal obstruction. Diagnoses are frequently made perioperatively. We present herein an illustrated case of transmesenteric hernia diagnosed in a pregnant woman who consulted for severe abdominal and dorsal pains. As CT scan was not possible because of the obstetric history, a decision to perform surgery was made because of acute pain, no history of previous surgery, and a plain erect X-ray disclosing early signs of intestinal obstruction. Perioperative findings were an ileal volvulus through a transmesenteric fossa circled by a unique ileocolic branch. This particular vascular disposition suggests transmesenteric hernias may be the first step in the constitution of congenital small-bowel atresia.

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.

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.