Gut lumen formation defect can cause intestinal atresia: evidence from histological studies of human embryos and intestinal atresia septum.

Intestinal atresia (IA), a common cause of neonatal intestinal obstruction, is a developmental defect, which disrupts the luminal continuity of the intestine. Here, we investigated (i) the process of lumen formation in human embryos; and (ii) how a defective lumen formation led to IA. We performed histological and histochemical study on 6-10 gestation week human embryos and on IA septal regions. To investigate the topology of embryonic intestine development, we conducted 3D reconstruction. We showed that a 6-7th gestation week embryonic gut has no lumen, but filled with mesenchyme cells and vacuoles of a monolayer of epithelial cells. A narrow gut lumen was formed by gestation week-9, the gut was filled with numerous vacuoles of different sizes, some vacuoles were merging with the developing embryonic gut wall. At gestation week-10, a prominent lumen was developed, only few vacuoles were present and were merging with the intestine wall. At IA septal regions, vacuoles were located in the submucous layer, covered by a single layer of epithelium without glandular structure, and surrounded with fibrous tissue. The mucosal epithelium was developed with lamina propria and basement membrane, but the submucosa and the longitudinal smooth muscle layers were not properly developed. Hence, the vacuoles in IA septum could represent a remnant of vacuoles of embryonic gut. In conclusion, the fusion of vacuoles with the developing intestine wall associates with the disappearance of vacuoles and gut lumen formation in human embryos, and perturbation of these developmental events could lead to IA.

Epithelial changes of congenital intestinal obstruction in a rat model.

INTRODUCTION: Intestinal atresia is a rare congenital affliction that is often associated with severe bacterial infections despite adequate neonatal surgery. Previous studies have focused on enteric nervous system variations. We hypothesized that epithelial systems (ES) may also be involved in the pathophysiology of postnatal disorders. MATERIALS AND METHODS: Global gene expression was measured by transcriptomic analysis in a rat model of induced intestinal atresia. The analyses then focused on genes involved in ES (enterocytes and goblet cells). Rat fetus small intestines at various stages of development (ED15, ED17, ED19, and ED21, n = 22), were used as non-operated controls and compared to the upper and lower segments of rat fetus small intestines with an induced atresia (n = 14; ligature at ED18). The pattern of gene expression was then confirmed by histochemistry, electron microscopy, and RT-qPCR. RESULTS: From ED15 to ED21, the expression of several genes exhibited a physiological increase of ES markers, with a significant increase at the end of gestation. The operated embryos exhibited significantly higher variations of gene expression in the proximal segment than in the distal segment in terms of absorption and the epithelial barrier. An increase in goblet cells and markers was observed in the proximal segment compared to the controls. CONCLUSION: Fetal intestinal obstruction accelerates maturation in the proximal segment and disrupts the intestinal wall in the distal segment, with a decrease in the number of mucosal cells. Moreover, the epithelial cells underwent significant changes, supporting the notion that intestinal disorders involve more than the ENS.

Quality of life outcomes in children born with duodenal atresia.

PURPOSE: The aim of this study was to determine long term quality of life (QoL) outcome for children who underwent surgery for duodenal atresia (DA). METHODS: Patients were identified from a prospective database of neonatal DA cases managed at a tertiary pediatric surgical centre. The QoL was measured using the validated PedsQL™ 4.0 core score and PedsQL™ gastrointestinal module; higher score equates to better QoL. Participants' scores were compared to published control cohorts, age-matching the core score. Trisomy 21 was identified a priori as a possible confounder, informing subgroup analyses for children with and without trisomy 21. RESULTS: Fifty-five families were invited to participate, with 38 surveys returned (39% male; median age 6.7y, range 2.7-17.3y). Seven participants had trisomy 21. There were no differences in QoL measures between all DA participants and controls. The PedsQL™ core score was significantly lower for DA participants with trisomy 21, but there was no accompanying difference in PedsQL™ gastrointestinal score. CONCLUSIONS: Children undergoing DA surgery in the neonatal period typically grow up to have a QoL comparable to a healthy population. Children with DA and trisomy 21 were more likely to have reduced overall QoL, albeit without an associated difference in gastrointestinal QoL score. LEVEL OF EVIDENCE: Prognosis study - level II (prospective cohort study).

Duodenal atresia with familial apple peel syndrome: case study with review of literature.

This is a case report of a neonate who was antenatally diagnosed with jejunal atresia which turned out to be duodenal atresia with apple peel syndrome. A previous sibling, who also had apple peel but with jejunal atresia, succumbed to sepsis after surgery. The first sibling had jejunal stenosis and had died of sepsis following surgery. Combination of duodenal atresia with apple peel is extremely rare. This coupled with a familial condition is rarer still. This case was challenging due to the short length of the gut and prolonged need for total parenteral nutrition and sepsis in postoperative period.

Clinicopathological study of intestinal smooth muscles, interstitial cells of Cajal, and enteric neurons in neonatal jejuno-ileal atresia with special reference to muscle morphometry.

PURPOSE: To assess the thickness of the intestinal smooth muscle layer and analyze the distribution and density of interstitial cells of Cajal (ICC) and enteric neurons in the proximal and distal segments of neonatal jejuno-ileal atresia. METHODS: This is an observational study done over a period of one year in which fifteen cases of jejuno-ileal atresia were included. All the cases underwent laparotomy and resection of the atretic segment with variable portions of the dilated proximal segment and distal segment. Histopathological analysis was done on the sections taken from proximal segments (at 3 cm, 5 cm & 8 cm) and the distal segment (at 2 cm) from the atretic portion. The mean thickness of the inner circular muscle layer (ICML) and outer longitudinal muscle layer (OLML) was assessed in the above segments using image morphometry. In addition, we also analyzed the distribution and density of the ICCs and enteric neurons in the different segments using immunohistochemistry for c-kit and S-100, respectively. Controls included normal jejuno-ileal segments resected from postmortem cases (n=7) and other nonrelated surgeries (n=3). The findings were then compared with each-other and with normal controls. RESULTS: Mean thickness of ICML and OLML of the proximal segments at 8 cm was significantly lower than at 3 cm and 5 cm of ileal and jejunal atresias (p≪ 0.5). The mean thickness of ICML and OLML of distal segments at 2 cm was similar to the controls in all the atretic cases (p≫ 0.5). The mean ICML thickness at proximal 8 cm segment was similar to the distal segment of both ileal & jejunal atresias (p= 0.06 & 0.37 respectively). The mean thickness of the OLML of the proximal 8 cm segments was significantly more than that at the distal segment (p=0.008) in ileal atresias but was similar in cases of jejunal atresias (p=0.07). Both the proximal and distal segments of ileal as well as jejunal atresias showed reduction in distribution and density of ICCs, as compared to normal controls. The density of ICCs in proximal segments at 3 cm and 5 cm was similar in both ileal (p=0.33) and jejunal segments (p=0.41) but was significantly lower than the proximal 8 cm segments (p≪0.05).The distribution of ICCs in the proximal segment at 8 cm was similar to the distal segments (p≪0.05). S-100 staining showed dense expression of neurons and glial cells with presence of submucosal giant ganglia within the proximal dilated segments as compared to the distal segments and the controls, which were more marked at 3 cm and 5 cm levels than at 8 cm level. CONCLUSION: Muscle morphometry using image analysis is a simple technique to assess the thickness of the intestinal smooth muscle layers. There is significant smooth muscle hypertrophy along with marked alteration in density and distribution of ICCs and ENS in the dilated proximal segments up to 5 cm, and relatively milder changes at 8 cm levels, as compared to the distal segments and the controls. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.

Oesophageal atresia with tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease: outcome of a rare phenotype.

Oesophageal atresia with or without tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease are surgical malformations of the gastrointestinal tract typically diagnosed early in the neonatal period and varying in severity and prognosis. This report describes a full-term male newborn presenting simultaneous oesophageal atresia with distal tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease. In addition to the complex types of gastrointestinal malformations involved, the combination of ileal atresia and Hirschsprung's disease, as well as ganglion cells distal to intestinal atresia, resulted in a challenging diagnosis. Despite a successful outcome, the patient presented increased morbidity and prolonged hospitalisation. We highlight some important findings that may aid the early diagnosis of Hirschsprung's disease in this clinical setting. To our knowledge, the association of oesophageal atresia/tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease has not been previously reported.

Early diagnosis and surgical intervention untie the Gordian knot in newborns with colonic atresia: report of two cases and review of the literature.

Incidence of colonic atresia in living infants ranges from 1:5,000 to 1:60,000 (average 1:20,000). It constitutes 1.8 to 15% of all cases of atresia of the gastrointestinal tract. In 58.56-75% of all cases is right-sided. We aim, through the presentation of two cases of colonic atresia which we encountered and after systematic research of the current literature, at addressing three major issues: diagnostic approach, operative strategy and management of the prognostic parameters of the colonic atresia. The common parameter in these two cases was the early diagnosis, which played a significant role in the uncomplicated postoperative course. The first case was a type I sigmoid atresia. Contrast's escape during contrast enema examination due to accidental rupture of the distal part of the colon led to diagnosis. Side-to-side anastomosis, restoration of the rupture and a central loop sigmoidostomy were urgently performed. The second case was a type III atresia at the level of the ascending colon, which was early diagnosed via pregenital ultrasonography, in which colonic dilation was depicted. Restoration of the intestinal continuity early after birth was performed at a time. In conclusion, we believe that early diagnosis, selection of the appropriate operative strategy and prompt recognition of potential post-operative complications, especially rupture of the anastomosis, contribute to the optimization of the prognosis in patients with colonic atresia.

Involvement of the enteroendocrine system in intestinal obstruction.

INTRODUCTION: Intestinal atresia, a rare congenital condition, is often associated with intestinal motility disorders despite adequate neonatal surgery. Previous studies have focused on changes in the enteric nervous system (ENS). We hypothesized that other components of the digestive tract could be involved in this condition. MATERIAL AND METHODS: In a rat model of surgically-induced intestinal obstruction, a transcriptome analysis was performed to measure the global gene expression. Then, analyzes were focused on genes expressed in ENS and neuroendocrine cells. Rat fetus small intestines at different developmental stages (ED15, ED17, ED19 and ED21, (n = 22)) were studied as controls and compared to the upper and lower segments of small intestines from rat fetuses with surgically-induced obstruction (n = 14; ligature at ED18). The gene expression pattern was confirmed by immunohistochemistry, electron microscopy and RT-qPCR. RESULTS: From ED15 to ED21, there was a physiological decrease in the gene expression of ENS markers and an increase in that of neuroendocrine genes. Regarding operated embryos, the changes in global gene expression were significantly higher in the proximal segment compared to the distal segment (18% vs. 9%). More precisely, a decrease in ENS gene expression and an increase in neuroendocrine gene expression were observed in the proximal segment compared to controls, indicating an accelerated maturation pattern. Immunohistochemistry and electron microscopy confirmed these findings. CONCLUSION: Fetal intestinal obstruction seems to induce an accelerated maturation in the proximal segment. Moreover, neuroendocrine cells undergo significant unexpected changes, suggesting that ENS changes could be associated with other changes to induce intestinal motility disorders.

Bile-stained amniotic fluid: a case report.

BACKGROUND: Green-stained amniotic fluid does not always indicate that meconium was passed in utero. CASE PRESENTATION: We report the case of a 2280-g Hispanic preterm female born at 32 weeks of gestation with congenital jejunal atresia. The amniotic fluid was greenish stained; the initial impression was meconium-stained amniotic fluid. Postnatal findings revealed no meconium in her rectum. The content of her first stool appeared sticky and white. CONCLUSION: In the absence of meconium in the rectum, the pediatrician should consider the possibility that the greenish amniotic fluid is not meconium stained, but rather stained with bile due to the fetus vomiting in utero secondary to intestinal obstruction.

A further family of Stromme syndrome carrying CENPF mutation.

Stromme syndrome is a rare genetic disorder characterized by microcephaly, anterior ocular chamber anomalies, and "apple peel" type jejunal atresia. Here, we report a Stromme syndrome family with two affected siblings with a homozygous truncating frameshift mutation in CENPF. A 3-month-old girl was hospitalized due to prenatally diagnosed microcephaly, microphthalmia, and dysmorphological features. The history of a previous child with the same findings in addition to "apple peel" intestinal atresia had been noted. Regarding the clinical features of both affected siblings, a diagnosis of Stromme syndrome was established. Exome-sequencing of these two cases showed the homozygous mutation (c.5912_5913insA)/(p.T1974Nfs*9) in CENPF. While confirmation of this gene being responsible for Stromme syndrome was pending our results, Filges et al. reported that CENPF was indeed underlying the reason for Stromme syndrome. This is the second case report identifying CENPF mutation as the cause of Stromme syndrome.

More than fetal urine: enteral uptake of amniotic fluid as a major predictor for fetal growth during late gestation.

UNLABELLED: The purpose of our study was to investigate the importance of amniotic fluid (AF) for fetal growth during late gestation using esophageal atresia (EA) patients as a model. In this retrospective cohort study, we compared the z-scores adapted for birth weights (BW z-scores) for each of 517 European newborns with congenital pre-gastric intestinal atresia, i.e., EA, to a European reference population. To account for the influence of the intestinal atresia on fetal growth per se, we compared adapted birth weights for each of 504 European newborns with post colonic intestinal atresia (anorectal malformation (ARM) with atresia of the anus) to the same European reference population. Analysis of the complete cohort showed (i) a significantly higher rate of small for gestational age newborns among EA compared to ARM newborns (p < 0.001) and (ii) significantly lower BW z-scores among EA compared to ARM newborns (p < 0.001). BW z-scores of EA newborns were significantly lower in term compared to preterm newborns with an inverse correlation with gestational age (GA) (Spearman correlation coefficient, r = -0.185, p < 0.001). CONCLUSIONS: Enteral uptake of AF seems to play a pivotal role in fetal growth during late gestation. WHAT IS KNOWN: • Peak velocity of fetal weight gain occurs at 33 weeks of gestation and continues until birth. During this period, fetal growth is mainly characterized by cellular hypertrophy. • Amniotic fluid (AF) comprises large amounts of hormones and growth regulators. What is New: • A significantly higher rate of small for gestational age and lower birth weights and z-scores are observed among newborn infants with congenital pre-gastric intestinal atresia. • These findings suggest that enteral uptake of AF is a major predictor for fetal growth during late gestation.

A Newly-Discovered Mutation in the RFX6 Gene of the Rare Mitchell-Riley Syndrome.

Mitchell-Riley syndrome is a genetic disorder characterized by neonatal diabetes, pancreatic hypoplasia, intestinal atresia and/or malrotation, biliary atresia, and gallbladder aplasia or hypoplasia. It was considered a variant of the Martinez-Frias syndrome with similar phenotypic characteristics, except for neonatal diabetes and tracheoesophageal fistula. However, the genetic mutation in (regulatory factor X on chromosome 6) RFX6 was only detected in babies who had diabetes, making it different from the previously known mutations for the disease. This is the first reported case of a classical Mitchell-Riley syndrome in the Arab peninsula along with additional features and novel mutations in the RFX6 gene.

Factors Conducive to Catch-Up Growth in Postoperative Jejunoileal Atresia Patients as Prognostic Markers of Outcome.

INTRODUCTION: Jejunoileal atresia (JIA) is a major congenital anomaly that requires surgical intervention in the neonatal period. During follow-up after surgery, there is usually a period of catch-up growth (CUG) that is sufficient for patients to regain normal weight for age. However, in some cases, CUG is inadequate. The aim of this study was to assess postoperative JIA patients to determine factors that may be associated with good CUG. MATERIAL AND METHODS: We retrospectively reviewed JIA patients treated at our institution by classifying them into three groups based on a comparison of postoperative weight with standard weight for healthy matched controls; that is, more than mean at 12 months after surgery (group M+), less than mean at 12 months after surgery but more than mean at 24 months after surgery because of CUG (group M-CUG+), less than mean at 24 months after surgery because there was no CUG (group M-CUG-). The following parameters were evaluated: gestational age, birth weight, sight of atresia: jejunum or ileum, length of residual small intestine, ratio of the length of residual small intestine to the predicted length of small intestine for matched gestational age (RP ratio), and duration of parenteral nutrition. RESULTS: A total of 42 patients were reviewed and classified into group M+ (n = 13), group M-CUG+ (n = 11), and group M-CUG- (n = 18). There were no significant differences in gestational age, birth weight, and duration of parenteral nutrition between the three groups. Incidence of JIA according to site was also similar. Length of residual small intestine was not significantly different between the three groups, but RP ratios were significantly higher in M+ (84.7 ± 15.4%) and M-CUG+ (83.8 ± 17.7%) compared with M-CUG- (69.2 ± 18.1%) (p = 0.02, respectively). CONCLUSIONS: A higher RP ratio (approximately 84%) would appear to be conducive to CUG while a lower ratio (less than 70%) was not. Actual length of residual small intestine was not relevant to CUG. We recommend calculating the RP ratio in postoperative JIA patients and using 70% as a cutoff value to predict patients with poor potential for CUG who may benefit from more aggressive nutritional support to achieve normal growth.

Prenatal intestinal obstruction affects the myenteric plexus and causes functional bowel impairment in fetal rat experimental model of intestinal atresia.

BACKGROUND: Intestinal atresia is a rare congenital disorder with an incidence of 3/10,000 birth. About one-third of patients have severe intestinal dysfunction after surgical repair. We examined whether prenatal gastrointestinal obstruction might effect on the myenteric plexus and account for subsequent functional disorders. METHODOLOGY/PRINCIPAL FINDINGS: We studied a rat model of surgically induced antenatal atresia, comparing intestinal samples from both sides of the obstruction and with healthy rat pups controls. Whole-mount preparations of the myenteric plexus were stained for choline acetyltransferase (ChAT) and nitric oxide synthase (nNOS). Quantitative reverse transcription PCR was used to analyze mRNAs for inflammatory markers. Functional motility and permeability analyses were performed in vitro. Phenotypic studies were also performed in 8 newborns with intestinal atresia. In the experimental model, the proportion of nNOS-immunoreactive neurons was similar in proximal and distal segments (6.7±4.6% vs 5.6±4.2%, p = 0.25), but proximal segments contained a higher proportion of ChAT-immunoreactive neurons (13.2±6.2% vs 7.5±4.3%, p = 0.005). Phenotypic changes were associated with a 100-fold lower concentration-dependent contractile response to carbachol and a 1.6-fold higher EFS-induced contractile response in proximal compared to distal segments. Transcellular (p = 0.002) but not paracellular permeability was increased. Comparison with controls showed that modifications involved not only proximal but also distal segments. Phenotypic studies in human atresia confirmed the changes in ChAT expression. CONCLUSION: Experimental atresia in fetal rat induces differential myenteric plexus phenotypical as well as functional changes (motility and permeability) between the two sides of the obstruction. Delineating these changes might help to identify markers predictive of motility dysfunction and to define guidelines for post-surgical care.

Medical management of motility disorders in patients with intestinal failure: a focus on necrotizing enterocolitis, gastroschisis, and intestinal atresia.

BACKGROUND: Intestinal failure (IF) is the dependence upon parenteral nutrition to maintain minimal energy requirements for growth and development. It may occur secondary to a loss of bowel length, disorders of motility, or both. Short bowel syndrome (SBS) is a malabsorptive state resulting from surgical resection, congenital defect, or diseases associated with loss of absorptive surface area. A particularly vexing problem is associated with whole bowel and/or segmental intestinal dysmotility. Motility disorders within the context of SBS and IF may relate to rapid intestinal transit secondary to loss of intestinal length, dysmotility associated with loss or poor antegrade peristalsis, or gastroparesis. Therapy may be classified into medical (prokinetic and antidiarrheal agents) and surgical to deal with the overdistended poorly motile bowel. METHODS: We performed a systematic review of the literature pertaining to IF, SBS, and dysmotility in the pediatric population with gastroschisis, necrotizing enterocolitis, and intestinal atresia. In addition to the available treatment options, we have provided a review of the literature and a summary of the available evidence. CONCLUSION: Despite relatively poor level of evidence regarding the application of promotility and antidiarrheal medications in patients with SBS and IF, these agents continue to be used. Herein, we provide a review of the physiology and pathophysiology of intestinal motility/dysmotility and available strategies for the use of promotility and antidiarrheal agents in patients with IF/SBS.

Antenatal diagnosis of bowel dilatation in gastroschisis is predictive of poor postnatal outcome.

PURPOSE: Although gastroschisis infants usually have a good outcome, there remains a cohort of babies who fare poorly. We inquired whether the presence of bowel dilatation in utero is predictive of postnatal course in infants with gastroschisis. METHODS: We compared the clinical course of infants who had bowel dilatation with those who did not. Bowel dilatation was defined as more than 20 mm in cross-sectional diameter on ultrasound at any gestational age. Outcome measures used were length of time of parenteral nutrition, death, and surgery for intestinal failure. RESULTS: A review of 170 infants with gastroschisis identified 74 who had dilatation of more than 20 mm (43.5%). There was no significant difference in the incidence of intestinal atresia in those with bowel dilatation and those without (P = .07). Those with bowel dilatation spent a longer period on parenteral nutrition. There were significantly more deaths in the group with bowel dilatation (P = .01). There was no significant difference in the number of infants requiring surgery for intestinal failure between the 2 groups (P = .47). CONCLUSIONS: We found that sonographically detected bowel dilatation more than 20 mm in utero in fetuses with gastroschisis may have value in predicting clinically significant adverse postnatal outcomes.

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

OBJECTIVE: To describe the outcomes of 130 intestinal atresias between 1982 and 2007. METHODS: Records were analyzed for location, demographics, prenatal diagnosis, birth weight, associated anomalies, surgery, establishment of oral intake, re-interventions and mortality. Statistical analyses were performed using Fisher test and ANOVA. RESULTS: There were 59 duodenal (30 male), 63 jejuno-ileal (34 male) and 8 colonic atresias (3 male). Prenatal diagnosis was established in 27 (46%) duodenal (DA), 26 (41%) jejuno-ileal (JIA) and 1 (12.5%) colonic atresias (CA). The mean birth weights, 2,380.5 g (SD 988) DA, 2,814 g (SD 755) JIA and 3,153 g (SD 527) CA were significantly different (p = 0.011). The mean gestational ages were 36, 37 and 37 weeks in DA, JIA and CA, respectively (p-NS). Associated congenital anomalies were seen in 41 (76%) DA, 32 (52%) JIA and 3 (38%) CA (p = 0.08, NS). The median time to full oral feeds after surgery was 18 days in DA, 20 days in JIA and 15.6 days in CA, respectively (p > 0.05). Eight patients with DA and nine patients with JIA underwent repeat surgery for adhesive obstruction. Adhesive bowel obstruction was most common in the first year after surgery in both groups (15/17). Gastroschisis was seen in six (10%) of JIA and three (35%) of CA. Two patients in the JIA group underwent bowel lengthening. Patients with gastroschisis and those with associated anomalies needed prolonged duration of TPN after JIA correction. There was no mortality in the duodenal atresia and colonic atresia groups. Six patients in the JIA group died, three of severe atresias coupled with multiple anomalies and three of cholestasis and sepsis. CONCLUSION: Distal atresias are difficult to diagnose antenatally. Proximal atresias have a significantly lower birth weight than distal atresias. Associated anomaly screening is important in all atresias.

An alternative management option for colonic atresia preventing loss of the ileocecal valve.

PURPOSE: Management of colonic atresia is contentious, with primary anastomosis having a notable risk of anastomotic leak. In addition, resection of the terminal ileum and ileocecal (i-c) valve is frequently performed, risking side effects such as diarrhea, vitamin B(12) deficiency, and gall stone formation. METHODS: The hospital coding system was searched for all patients with a diagnosis of colonic atresia between July 2005 and July 2008. Four term neonates were managed by formation of an ileostomy, a "blow hole" stoma just proximal to the atresia, and a mucus fistula distal to the atresia. RESULTS: Average time to full feeds was 7.5 days (range, 3-12 days), and average length of stay was 23 days (range, 13-47 days). Stoma management, problematic in 2 infants, was individualized by a specialist stoma nurse. Ileostomy output was refed into the mucus fistula. Complications included 3 episodes of prolapse of the blow hole stoma in infant 2. All of the infants returned to the operating theater at 1 to 3 months of age for restoration of bowel continuity and closure of the ileostomy. The atretic segment was resected, and an end-to-end anastomosis was performed. Recovery was straightforward in all cases. CONCLUSION: A procedure that retains the i-c valve and most of the colon through creation of a blow hole stoma in the distended proximal colon with a diverting ileostomy and mucus fistula is described. The technique is recommended in selected infants as bowel length and anatomy can be preserved, despite the use of multiple stomas.