Long gap esophageal atresia repair using the foker tecnique: a 19 years experience.

Esophageal atresia (EA) with a 3-cm gap or longer and a very short distal segment represents the extreme of this disorder's spectrum, the treatment of which can be challenging. Management of newborns with long-gap esophageal atresia (LGEA) remains a challenge for pediatric surgeons. The inclusion criteria in the study were as follows: long gap esophageal atresia, Foker technique, at least 10 years follow up. We sought to examine our experiences with LGEA using Foker technique. RESULTS: From 2001 to 2020 were operated for esophageal atresia at the UOC Surgery of the AORN Santobono 290 children of whom 16 patients meet our inclusion criteria. 6 had a type I EA, 2 had a type II and 8 had a type III. Mean gap length was 4 cm (range 3-5), In 9 patients gastrostomy and Foker technique was performed in the same time (around the 1stto the 4th day of life), while in 7 patients gastrostomy was performed in 1st-2nd day of life and the Foker technique was delayed due to clinical needs with a mean of 76 days (range 26-96 days) . In the study group, 11/16 (68,75%) patients underwent multiple endoscopic dilatations ( means 5 sessions per patient); 3 out of 16 cases of early complications occurred: 2 perforations and 1 dehiscence. Gastroesophageal reflux was common during the first 6 years of life and 2 had a fundoplication due to gastroesophageal reflux disease resistant to maximal medical therapy and 1 had an esophageal diverticulum at 5 years. 7 out of 16 (43,7%) (average age 13,5 years) perform therapy cycles with pump inhibitors for an average period of 12 weeks. CONCLUSIONS: Foker procedure for esophageal lengthening in patients with LGEA was successful in mobilizing the esophagus and obtaining a repair in the majority of our patients after 12 to 15 days of traction and it should be considered in LGEA cases to preserve the native esophagus as often as possible.

Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 81 patients.

BACKGROUND: Despite significant developments in understanding the pathological anatomy and physiology of Hirschsprung's disease (HD), the follow-up of children with HD remains far from clear. Treatment results for HD are not uniformly successful and the interpretation of the result by the parents and child may differ from that of the surgeon. Our goal is to analyze quality of life and differences in functional outcomes in children operated on using the pediatric incontinence / constipation scoring system (PICSS) validated in a regulatory group. METHODS: A retrospective questionnaire was performed for all children operated for HD between 1995 and 2017 at the Pediatric Surgery Unit. The use of PICSS defined children with continence or incomplete constipation. RESULTS: 125 patients were operated on for HD between 1995 and 2017 in our Pediatric Surgery Unit and 81 families gave their consent to participate. The median age at the interview was 94.5 months (range 12-283 months). Complete aganglionosis was in 2% of cases. Treatments included a Soave procedure in 96%. Colostomy was performed in 15.8% (13/81). Decompressive enterostomy was performed in a child. The rate of normal bowel function was 66.6% (n = 54). The percentage of cases with incomplete continence and with constipation were 4.9% (n = 4) and 16% (n = 13), respectively. CONCLUSIONS: HD results aren't always as good as surgeons may perceive; long-term follow-up is important. Over time, most schoolage children improve significantly for fecal continence, but it can also be acquired in late adolescence.

Clinical, HLA, and small bowel immunohistochemical features of children with positive serum antiendomysium antibodies and architecturally normal small intestinal mucosa.

BACKGROUND: Antiendomysium antibodies have a high sensitivity and specificity for celiac disease. A small percentage of subjects positive for these antibodies have a small intestinal mucosa hitherto considered normal. OBJECTIVES: The aim of this study was to characterize the clinical, serological, immunogenetic, and immunohistological features of these subjects. METHODS: From 409 patients who were positive for celiac-related antibodies, we selected 24 (5.9%) patients who had an architecturally normal small intestinal mucosa. One hundred age-matched celiac patients with a "flat" small intestinal mucosa, and 50 age-matched nonceliac children were also studied. The number of CD3+ and gammadelta+ intraepithelial lymphocytes and of CD25+ lamina propria mononuclear cells, and the expression of crypt HLA-DR and lamina propria ICAM-1 were assessed. HLA haplotyping was also performed. RESULTS: Eleven (45.8%) of the 24 patients had a distinct infiltrative pattern, i.e., an increase in CD3+ intraepithelial lymphocytes (> 2SD of the nonceliac group), whereas 17 (70.8%) had a higher density of intraepithelial gammadelta+ cells. In 17 (70.8%) patients, the number of lamina propria CD25+ cells was increased and/or the expression of ICAM-1 and crypt HLA-DR was enhanced. All 24 patients carried the celiac disease-associated HLA haplotypes. Two of the six patients who remained on a normal diet and underwent a second jejunal biopsy developed villous atrophy. CONCLUSIONS: Most of the patients with serum antiendomysium antibodies and normal jejunal histology showed immunohistochemical signs of immune activation in the epithelium, lamina propria, and crypts. We recommend that such patients be monitored to assess their progress and to determine whether they need a gluten-free diet.