Ileocecal valve atresia: introduction of a new surgical approach.

Ileocecal valve atresia is a very rare anomaly with only a few cases reported in the literature. The conventional therapy includes ileocecal resection and ileocolic anastomosis. Here, we present a patient with inborn ileocecal valve atresia in whom we successfully preserved the ileocecal valve and the terminal ileum through opening a valve and tapering the ileum in one operation.

Delayed primary serial transverse enteroplasty as a novel management strategy for infants with congenital ultra-short bowel syndrome.

BACKGROUND: Congenital ultra-short bowel syndrome (USBS) is a challenging problem with a poor outcome. We report a new management approach for USBS infants that attempts to optimize gut growth potential. METHODS: We report five neonates with USBS in whom no correction was performed at primary surgery except placement of a gastrostomy (G) tube. Sham feeds were started with intermittent G-tube clamping to induce bowel dilatation/growth. Serial fluoroscopy was done until bowel caliber reached 5 cm. STEP was performed and continuity established to the colonic remnant. Small bowel length (SBL) and enteral caloric intake were tabulated. RESULTS: Patients were born with a mean residual SBL of 19 ± 7.6 cm (14.8% of expected). Median duration of sham feeds prior to STEP was 108 (range 27-232)days. Mean SBL at STEP was 47 ± 12.1cm, which increased post-STEP to 70 ± 12.7 cm (a mean increase of 296% from birth, representing 36.4% ± 13.1% of expected gut length). With a median follow-up time of 20 months (range 8-28), 4/5 achieved >50% enteral calories and have normal liver function. One has undergone liver transplantation. CONCLUSIONS: In USBS patients, delayed surgical correction with sham feeds accelerates gut growth, optimizing potential for autologous reconstruction. This approach may offer greater opportunity for intestinal adaptation than traditional options.

Ileocaecal valve: how important is it?

PURPOSE: Our aim was to investigate the importance of the ileocaecal valve and its reconstruction in patients that are not suffering from short bowel syndrome and Crohn's disease. METHODS: Casenotes of 99 children with hemicolectomy and 24 children with terminal ileal resection were reviewed and sorted into three groups. Group 1: ileocaecal valve resection (limited hemicolectomy), Group 2: hemicolectomy, Group 3: terminal ileal resection between 10 and 25 cm. Patients with Crohn's, short bowel syndrome and incomplete follow-up were excluded. RESULTS: Chronic diarrhoea was documented in 7/26 cases (27%) in Group 1, 6/23 patients (26%) in Group 2, and none of the 13 patients had diarrhoea in Group 3. Pearson Chi-square test showed significant difference between Group 1 and Group 3 (p = 0.038) and between Group 2 and Group 3 (p = 0.043). But there was no significant difference between Group 1 and Group 2 (p = 0.947). CONCLUSION: Chronic diarrhoea is a significant complication after limited hemicolectomy not only in Crohn's disease and short bowel syndrome. This is likely to originate from the loss of the ileocaecal valve itself rather than the loss of the ileal or colonic segment. Our results justify attempts to reconstruct the ileocaecal valve.

Intestinal loop lengthening: early treatment of vanishing bowel.

Bianchi's procedure experience for short bowel syndrome in children is positive. This technique is generally performed after the first year of life. Here the authors propose a case of gastroschisis with prenatal spontaneous closure of abdominal defect and vanishing gut presenting as intestinal atresia, absence of ileo-cecal valve, and residual short intestinal dilatation, treated by early gut lengthening (ILP).

Spectrum of normal and abnormal CT appearances of the ileocecal valve and cecum with endoscopic and surgical correlation.

Because of advances in imaging technology, evaluation of the gastrointestinal tract is increasingly being performed with cross-sectional imaging (eg, computed tomographic [CT] colonography, CT enterography). However, the diagnosis of disease involving the ileocecal valve (ICV), cecum, and appendix with CT can be challenging. The normal ICV can have many different appearances, depending on cecal distention and mobility, whether the valve is open or closed, and inherent variable morphologic characteristics. In addition, flat cecal lesions are difficult to detect, and larger masses are sometimes mistaken for the ICV or residual stool. Familiarity with the typical appearances of the normal anatomy and various pathologic conditions of the gastrointestinal tract on two- and three-dimensional cross-sectional images is useful in making the correct diagnosis.

Atresia of the ileocecal junction with agenesis of the ileocecal valve and vermiform appendix: report of a case.

Intestinal atresia involving the ileocecal region is a very rare intestinal malformation, and the presence or absence of the ileocecal valve influences its surgical management. We report the case of a male newborn with a provisional diagnosis of distal ileal atresia, in whom laparotomy revealed that the entire ileocecal region was atretic with an absent ileocecal valve and appendix vermiformis. We resected the dilated terminal ileum together with the atretic segment and performed an ileocolic anastomosis between the terminal ileum and the transverse microcolon without valve reconstruction. When last seen, 8 months after the operation, the baby was developing normally. Ileocolic anastomosis without valve replacement appears to be sufficient if an ileocecal valve is completely absent and only a short segment of the terminal ileum is lost.

[Rare cases of intussusceptions in children]. / Seltene Formen der Invagination im Kindesalter.

Intussusception is common in children under two years of age. Most of them are idiopathic whereas intussusceptions secondary to focal pathologies are rare. We report of two unusual cases of ileocolic intussusception. In one child a mobile cecum was seen, in the second child a duplication cyst of the ileocecal valve.

Ileocaecal valve atresia: our surgical approach.

BACKGROUND: Atresia of ileocaecal valve is the rarest type of intestinal atresia; there are very few reports on it in the literature and all cases described to date were treated with ileocolic resection. METHODS: We present a rare case of ileocaecal valve atresia, the third in the literature, in which a "different" technical approach was employed as an alternative to the usual surgical technique (ileocolic resection). The described case came to our attention in a patient presenting with complicated intestinal perforation in whom we performed an ileal stoma after an unsuccessful conservative approach. Approximately four months later we operated her to close the stoma: during this procedure we discovered the colic valve atresia and treated it by creating a "new ileocaecal valve" and carrying out an appendectomy. After a 7-year follow-up no complications have occurred and the girl enjoys very good health. CONCLUSIONS: We conclude that performing a plasty of Bauhin's valve is a valid alternative to intestinal resection in such cases of atresia even if, according to some authors, the resection of the ileocaecal valve was not associated with increased morbidity and mortality (3).

Massive enterolithiasis associated with ileal dysgenesis.

A 20-year-old man with massive ileal enterolithiasis was investigated with plain radiography, ultrasound, computed tomography, barium follow through and double contrast barium enema. Ileocecal valve agenesis was found at surgery. The enteroliths were located in the distal ileum, which communicated with the large intestine via an ileotransverse fistula.

Congenital multiple intestinal atresia successfully treated with multiple anastomoses in a premature neonate: report of a case.

We report herein the case of a 2080-g premature male infant born at 34 weeks' gestation with multiple intestinal atresia, for whom multiple anastomoses were successfully performed. A total of 11 atresias were found in the small bowel, and five anastomoses were performed to preserve 59 cm of small bowel and the ileocecal valve. Postoperatively, he developed several episodes of sepsis caused by persistent enterostasis, but was able to be weaned from total parenteral nutrition (TPN) by postoperative day (POD) 106. Thus, multiple anastomoses may be the appropriate procedure to prevent short-gut syndrome for congenital multiple intestinal atresia, even in premature infants.

Atresia de válvula ileocecal / Ileocecal valve atresia

Se comunica un caso de atresia de válvula ileocecal. Se presentan sus manifestaciones clínicas, los procedimientos diagnósticos empleados, el abordaje quirúrgico y su evolución. Destacamos las anomalías asociadas encontradas, recomendándose su intervención quirúrgica inmediata

Outcome and intestinal adaptation in neonatal short-bowel syndrome.

We reviewed 52 consecutive patients with short-bowel syndrome (SBS) treated with long-term parenteral nutrition (PN) from 1978 through 1990. The SBS etiologies included necrotizing enterocolitis (NEC) in 26 patients (50%), abdominal wall defects in 11 (22%), jejunoileal atresia in 6 (12%), midgut volvulus in 4 (8%), Hirschsprung's disease in 3 (6%), and segmental volvulus and cloacal exstrophy in 1 (2%) each. The average initial small bowel length was 48.1 cm, and only 31% of the patients retained an ileocecal valve (ICV). The mean duration of PN therapy was 16.6 months, and 39 patients (75%) were successfully weaned from it. Forty-three patients (83%) survived. Significant differences between the initial 20 patients treated from 1978 through 1984 and the next 32 from 1985 through 1990 were duration of PN therapy (25.1 v 11.4 months; P = .04), incidence of PN-associated jaundice (80% v 31%; P = .001), and survival (65% v 94%; P = .02). NEC patients had a significantly lower mean birthweight than those with other etiologies (mean, 1,367 v 2,544 g; P less than .0001) but did not differ in initial small bowel length, ICV retention rate, duration of PN treatment, incidence of successful PN weaning, or outcome. The presence of an ICV did not correlate with successful PN weaning but did affect the mean duration of PN therapy (7.2 months with ICV v 21.6 months without; P = .03).(ABSTRACT TRUNCATED AT 250 WORDS)

Short-bowel syndrome in children. Quality of life in an era of improved survival.

A number of disorders in childhood can result in short-bowel syndrome (small bowel length, less than 100 cm). Improved care has increased survival in patients with short-bowel syndrome, but the quality-of-life factors associated with such improved survival have not been examined, to our knowledge. Sixteen consecutive pediatric patients with short-bowel syndrome (bowel length range, 22 to 98 cm) were followed up for 2 to 10 years. The original diagnoses were as follows: necrotizing enterocolitis (n = 6), multiple intestinal atresias (n = 4), extensive aganglionosis (n = 2), meconium peritonitis (n = 2), and midgut volvulus (n = 2). The range of initial hospitalization was from 62 to 395 days, and 13 of 16 patients have required readmission (two to 14 times). All patients required multiple operations (range, two to 14 operations), including combinations of venous access, adhesiolysis, tapering enteroplasty, reversed intestinal segments, and pull-through procedure. Nine of 16 patients received home total parenteral nutrition, and 12 of 16 patients required home elemental diets, usually via pump feedings. Fifteen patients (94%) survived. Two survivors are deaf, and one of these has mild developmental delay. Seven survivors (age range, 6 to 10 years) attend a regular school, four while receiving total parenteral nutrition or an elemental diet. Ten of 15 survivors are off all nutritional support (including the child with a 22-cm small bowel), with four others weaning. The presence or absence of an ileocecal valve did not affect outcome. Modern nutritional support methods provide excellent survival and quality of life for children with short-bowel syndrome.