Treatment of jejunoileal atresia by primary anastomosis or enterostomy: Double the operations, double the risk of complications.

PURPOSE: No study has evaluated complication rates of the combined operations needed for temporary Enterostomy compared to primary anastomosis in the treatment of Jejunoileal Atresia. Therefore the aim of this study is: 1) to compare the occurrence of severe postoperative complications (defined as Clavien-Dindo ≥III within 30 days) and 2) to compare the occurrence of different short- and long-term complications following treatment for Jejunoileal atresia either by primary anastomosis or the combined Enterostomy procedures. METHODS: All consecutive neonates treated for Jejunoileal Atresias between January 1998 and February 2021 at our tertiary academic centres were retrospectively included. Perioperative characteristics and severity of postoperative complications (Clavien-Dindo) were extracted and evaluated, using chi-squared statistics, following each operation per treatment. RESULTS: Eighty patients were included of whom 48 (60%) received a primary anastomosis and 32 (40%) an Enterostomy. Perioperative baseline characteristics were comparable, apart from significantly more patients with a gastroschisis and significantly less patients with jejunum atresia in the Enterostomy group. Our results showed that 1) significantly (p ≤ 0.01) more CD ≥III occur following treatment by Enterostomy. 2) Both short-term (surgical site infection, wound dehiscence) and long-term (short bowel syndrome, adhesive bowel obstruction) complications occurred significantly more in those treated by Enterostomy. We showed no significant difference in anastomotic leakage/stenosis and mortality rates between both treatment strategies. CONCLUSION: Although perioperative factors might necessitate an Enterostomy, we advise a low threshold for performing a primary anastomosis when in doubt, taking into account the double risk of major complications found in patients treated with a temporary Enterostomy.

Congenital Glucagon-like Peptide-1 Deficiency in the Pathogenesis of Protracted Diarrhea in Mitchell-Riley Syndrome.

CONTEXT: Mitchell-Riley syndrome due to RFX6 gene mutations is characterized by neonatal diabetes and protracted diarrhea. The RFX6 gene encodes a transcription factor involved in enteroendocrine cell differentiation required for beta-cell maturation. In contrast to the pathway by which RFX6 mutations leads to diabetes, the mechanisms underlying protracted diarrhea are unknown. OBJECTIVE: To assess whether glucagon-like peptide-1 (GLP-1) was involved in the pathogenesis of Mitchell-Riley syndrome protracted diarrhea. METHODS: Two case report descriptions. in a tertiary pediatric hospital. "Off-label" treatment with liraglutide. We describe 2 children diagnosed with Mitchell-Riley syndrome, presenting neonatal diabetes and protracted diarrhea. Both patients had nearly undetectable GLP-1 plasma levels and absence of GLP-1 immunostaining in distal intestine and rectum. The main outcome was to evaluate whether GLP-1 analogue therapy could improve Mitchell-Riley syndrome protracted diarrhea. RESULTS: "Off-label" liraglutide treatment, licensed for type 2 diabetes treatment in children, was started as rescue therapy for protracted intractable diarrhea resulting in rapid improvement during the course of 12 months. CONCLUSION: Congenital GLP-1 deficiency was identified in patients with Mitchell-Riley syndrome. The favorable response to liraglutide further supports GLP-1 involvement in the pathogenesis of protracted diarrhea and its potential therapeutic use.

A rare case of intrauterine intussusception causing ileal atresia.

Intrauterine intussusception is a rare but evident cause of intestinal atresia and is usually detected intraoperatively. We report on a term neonate who presented to the Department of Paediatric Surgery, Sabah Women and Children's Hospital, Malaysia with delayed passage of meconium and intestinal obstruction, wherein the lower contrast showed a claw sign. This was a good clue that this neonate had intrauterine intussusception and this suspicion was confirmed upon laparotomy. We found an ileo-ileal intussusception causing ileal atresia, requiring resection and primary anastomosis.

Endoscopic balloon dilation in duodenal stenosis secondary to intestinal lymphoma in a heart transplant patient.

Intestinal lymphoma can appear as a result of a state of immunosuppression, secondary to the transplantation of solid organs. Treatment with chemotherapy can result in various complications such as intestinal stenosis due to fibrotic scar tissue, which is a complication barely described in the literature. We present the case of a 12-year-old male with a heart transplant, who was diagnosed with intestinal lymphoma. After chemotherapy treatment, he developed 1-2 daily vomiting episodes as a result of intestinal stenosis due to fibrosis without tumor infiltration. Endoscopic balloon dilation was used and the symptoms were resolved without the need for surgical treatment.

Closing gastroschisis: The good, the bad, and the not-so ugly.

PURPOSE: The diagnosis of "closing" or "closed gastroschisis" is made when bowel is incarcerated within a closed or nearly closed ring of fascia, usually with associated bowel atresia. It has been described as having a high morbidity and mortality. METHODS: A retrospective review of closing gastroschisis cases (n = 53) at six children's hospitals between 2000 and 2016 was completed after IRB approval. RESULTS: A new classification system for this disease was developed to represent the spectrum of the disease: Type A (15%): ischemic bowel that is constricted at the ring but without atresia; Type B (51%): intestinal atresia with a mass of ischemic, but viable, external bowel (owing to constriction at the ring); Type C (26%): closing ring with nonviable external bowel +/- atresia; and Type D (8%): completely closed defect with either a nubbin of exposed tissue or no external bowel. Overall, 87% of infants survived, and long-term data are provided for each type. CONCLUSIONS: This new classification system better captures the spectrum of disease and describes the expected long-term results for counseling. Unless the external bowel in a closing gastroschisis is clearly necrotic, it should be reduced and evaluated later. Survival was found to be much better than previously reported. TYPE OF STUDY: Retrospective case series with no comparison group. LEVEL OF EVIDENCE: Level IV.

Neonatal intestinal obstruction associated with situs inversus totalis: two case reports and a review of the literature.

BACKGROUND: The association of neonatal intestinal obstruction with situs inversus totalis is extremely rare with only few cases reported in the literature to date. This association poses dilemmas in management. We present two such cases (of Indian origin), and briefly discuss the pertinent literature and measures to prevent unfavorable outcome. CASE PRESENTATION: Case 1: a 1-month-old preterm (1300 g) male neonate belonging to Hindu (Indian) ethnicity presented with recurrent bile-stained vomiting, non-passage of stools and epigastric fullness. A babygram and upper gastrointestinal contrast studies revealed situs inversus and suggested proximal jejunal obstruction with midgut volvulus. Exploration confirmed situs inversus totalis along with reverse rotation and midgut volvulus. There was a small gangrenous area in the proximal jejunal loop. A Ladd's procedure, resection of the gangrenous jejunal loop, and jejunojejunal anastomosis was performed. Note was made of the unusual appearance of the intestines suggestive either of fibrous or fatty infiltration. Postoperatively, our patient developed septicemia and died. Case 2: a 4-day-old female neonate belonging to Hindu (Indian) ethnicity, small (1320 g) for gestation, presented with history of non-passage of meconium since birth, refusal to accept feeds, and episodes of recurrent bilious vomiting with abdominal distension. A plain radiogram revealed situs inversus and proximal jejunal obstruction. Ultrasonography of her abdomen revealed renal dysplastic changes in both her kidneys. Laparotomy confirmed multiple jejunoileal atresias with situs inversus totalis. Resection anastomoses was performed for multiple atresias. Our patient passed a few pellets of meconium stools postoperatively; feeds were started gradually on the sixth day. Our patient gradually developed oliguria and renal failure, followed by respiratory distress and generalized edema requiring ventilatory support. She died later due to multiorgan failure. CONCLUSIONS: Clinicians should have high index of suspicion for malrotation with midgut volvulus or intestinal atresias in neonates of situs inversus presenting with bilious vomiting. The surgical treatment should follow the same surgical principles. In situs inversus, because of transposition of viscera, midgut volvulus may occur in an anticlockwise direction, hence derotation is performed clockwise. Prognosis was poor in our series because of low birth weight, late presentation, presence of gangrenous locus in the small bowel and development of septicemia in our first case and multiorgan fibrosis/dysplasia in our second case. Early diagnosis and timely referral is paramount for favorable outcome.

Maternal occupational exposure to ionizing radiation and major structural birth defects.

BACKGROUND: Ionizing radiation (IR) is known to be carcinogenic and mutagenic, but little is known about the association between maternal occupational exposure to IR and birth defects. METHODS: We studied 38,009 mothers who participated in the National Birth Defects Prevention Study and delivered between 1997 and 2009. We assessed odds ratios [ORs] for the association between maternal occupations with potential exposure to IR and 39 birth defects. RESULTS: We observed significant odds ratios (ORs) for isolated hydrocephaly (adjusted OR [AOR], 2.1; 95% confidence interval [CI], 1.1-4.2), isolated anotia/microtia (AOR, 2.0; 95% CI, 1.0-4.0), isolated colonic atresia (crude OR, 7.5; 95% CI, 2.5-22.3), isolated omphalocele (AOR, 2.3; 95% CI, 1.1-4.6) and isolated anencephaly (crude OR, 0.23; 95% CI, 0.06-0.94). We also observed a nonsignificant OR for birth defects in aggregate (AOR, 2.0; 95% CI, 0.9-4.6) among mothers with potential occupational exposure to fluoroscopy. CONCLUSION: We assessed 39 birth defects, observing that maternal occupations with potential exposure to IR were associated with a significantly increased risk for 4 birth defects and a significantly protected risk for 1 birth defect. These results should be interpreted cautiously because our measurement of exposure is qualitative, some of these associations may be due to occupational exposures that are correlated with IR and some may be due to chance. However, these findings serve as the first evaluation of these relationships in a large study and may be useful for generating hypotheses for future studies.

Acquired ileal atresia induced by adhesive intestinal obstruction.

Three female infants, aged 13, 7 and 18 mo respectively with acquired ileal atresia induced by adhesive small bowel obstruction are reported. Case 1 was diagnosed with urachal cyst and cyst excision was performed when she was 7-mo-old. Case 2 was diagnosed with mesenteric cyst and cyst excision was performed by laparoscopic-assisted procedure when she was 3-mo-old. Case 3 had no history of abdominal operation previously. At laparotomy ileal atresia induced by adhesive band was found.

Type IV jejunal atresia with an unusual variation of enteric duplication: report of a case.

We report a rare case of type IV jejunal atresia with enteric duplication and multiple diverticuli, found in a 3-day-old baby girl. To our knowledge, this association has never been reported before.

Humans, mice, and mechanisms of intestinal atresias: a window into understanding early intestinal development.

INTRODUCTION: Intestinal atresias have long been hypothesized to result from either failure of recanalization of the intestinal lumen or in utero vascular accidents. Recent work in animal models is now calling for a reassessment of these widely held paradigms. PURPOSE: In this review, we will examine the data that led to the original hypotheses and then evaluate more recent work challenging these hypotheses. Furthermore, we will discuss how defining the mechanism of atresia formation in animal models may provide insight into early intestinal development and the mechanism of lengthwise intestinal growth. CONCLUSION: Such insight will be critical in developing regenerative therapies for patients with intestinal failure.

Intestinal complications after antenatal fetoscopic laser ablation in twin-to-twin transfusion syndrome.

Twin-to-twin transfusion syndrome (TTTS) occurs in 9% of monochorionic twin pregnancies. An imbalanced blood flow across placental vascular communications produces a hypovolemic condition in the donor fetus and hypervolemia in the recipient fetus, leading to a variety of postnatal complications. We report 3 cases of intestinal injury in TTTS after fetoscopic laser ablation of the communicating vessels: 2 cases of intestinal atresia, and 1 case of necrotizing enterocolitis of 1 twin. Intestinal ischemic diseases have been reported after prenatal laser treatment in TTTS; with this report, we add 3 more cases.

Colonic atresia: a clinicopathological insight into its etiology.

BACKGROUND: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Mechanical compression with secondary ischemia as a possible cause of atresias associated with omphalocele.

We report 2 cases of omphalocele associated with intestinal atresia. In the context of this unusual sequence, we discuss the etiopathogenesis of intestinal atresias in association with tight abdominal wall defects.

Intrauterine intussusception: a rare cause of intestinal atresia.

Intrauterine intussusception is an uncommon cause of intestinal atresia. We report a case of ileal atresia owing to antenatal intussusception revealed as an intraluminal polypoid lesion after surgical intervention.

Gastroschisis, atresia, dysmotility: surgical treatment strategies for a distinct clinical entity.

PURPOSE: Intestinal atresia (IA) occurs in 10% to 20% of infants born with gastroschisis (GS). We describe a distinct subset of these children with severe dysmotility, without mechanical intestinal obstruction, and with adequate intestinal length for enteral nutrition and propose possible treatment strategies. METHODS: A total of 177 patients with GS managed at a single institution between 1993 and 2007 were retrospectively reviewed. RESULTS: Twenty-one (12%) patients had IA. Six (29%) did well, with gradual progression to full diet; 1 died at birth because of complete midgut infarction; 1 died of necrotizing enterocolitis; 4 with short bowel syndrome (SBS) (small bowel length, 30-41 cm) died of liver failure because of total parenteral nutrition (3) or sepsis (1). Nine (43%) were felt to have gastroschisis/atresia/dysmotility, defined as adequate small bowel length for survival as measured at time of laparotomy (mean, 146 cm; range, 66-233 cm), massive intestinal dilatation, and stasis. Of 9 patients with gastroschisis/atresia/dysmotility, 5 (56%) survived. All had surgery to "rescue" their dysfunction intestine, at mean age 128 days (range, 52-271 days): 4 had tapering enteroplasties of mean 37 cm (range, 5-115 cm)-all 4 survived; 3 had diverting stomas created for intestinal decompression, followed by stoma closure-1 survived; 1 nonsurvivor had a Kimura "patch"; 1 nonsurvivor underwent redo anastomosis. Survivors weaned off total parenteral nutrition at mean age 331 days after rescue surgery (range, 42-814 days). CONCLUSIONS: More than one third of patients with GS/IA appear to have significant intestinal dysmotility without true SBS nor obstruction. Successful treatment of these infants may be achieved with the use of tapering enteroplasty and/or temporary diverting stomas.

Preservation of extracorporeal tissue in closing gastroschisis augments intestinal length.

BACKGROUND: Prenatal closure of the umbilical ring in gastroschisis may result in an amorphous, nonviable appearing extracorporeal tissue that is resected during the repair. However, it is unclear whether such remnant intestine is truly nonviable. METHODS AND RESULTS: We examined the outcomes of patients when this tissue is preserved. We identified 8 patients who presented with a closing gastroschisis and a mass of tissue connected by a vascular pedicle. Four patients underwent abdominal exploration with resection of the mass and gastroschisis closure. Histologic examination revealed normal intestinal wall architecture. All patients in this group developed short bowel syndrome, requiring long-term parenteral nutrition. Conversely, 4 patients underwent abdominal exploration with internalization of the remnant tissue, a maneuver referred to as "parking," along with either silo placement, or primary closure of the gastroschisis. At re-exploration, 3 of 4 patients were found to have viable intestine, and bowel continuity was reestablished. The mean parenteral nutrition requirement for this group was significantly shorter than the resected group. CONCLUSION: In this series, we show that this amorphous tissue, when preserved, may exhibit normal intestinal architecture and absorptive function. Therefore, such remnant tissue should be preserved as it may significantly increase bowel length and minimize parenteral nutrition requirement.