Presentation and endoscopic management of sigmoid volvulus in children.

UNLABELLED: The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children, focusing on endoscopic reduction. In this retrospective multicenter study, we reviewed the charts of 13 patients with sigmoid volvulus. We recorded clinical symptoms, diagnostic methods, endoscopic or surgical therapy, and outcome. The children (seven girls, six boys) had a median age of 12.8 years (range, 15 months to 17 years) at initial presentation. Eight patients had associated diseases (e.g., chronic constipation, mental retardation, or myopathy). The initial symptoms were abdominal pain (13/13), abdominal distension (11/13), and vomiting (7/13), which were associated with abdominal tenderness in all patients. Abdominal X-ray showed dilated sigmoid loops and air-fluid levels in all patients. Endoscopic reduction by exsufflation was successful without any complications in 12 patients, whereas the youngest patient underwent a first-line sigmoidectomy. Recurrence occurred in 7/12 patients after endoscopic exsufflation. Finally, 11 patients underwent a sigmoidectomy. CONCLUSION: Although rare in children, sigmoid volvulus should be advocated when abdominal pain is associated with dilated sigmoid loops. Sigmoidoscopic exsufflation can be considered as the first-line management in the absence of perforation. However, sigmoidectomy is often required for prevention of recurrence. WHAT IS KNOWN: • Sigmoid volvulus is uncommon in childhood. • Diagnosis is often missed or delayed. What is New: • This is the first pediatric series showing that endoscopic exsufflation is an efficient and safe treatment option. • Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.

Immunization status in children with inflammatory bowel disease.

Inflammatory bowel diseases have an increased risk of infections due to immunosuppressive therapies. To report the immunization status according to previous recommendations and the reasons explaining a delay, a questionnaire was filled in by the pediatric gastroenterologist, concerning outpatients, in six tertiary centers and five local hospitals, in a study, from May to November 2011. One hundred and sixty-five questionnaires were collected, of which 106 Crohn's diseases, 41 ulcerative colitis, and 17 indeterminate colitis. Sex ratio was 87:78 M/F. Median age was 14.4 years old (4.2-20.0). One hundred and nine patients (66 %) were receiving or had received an immunosuppressive therapy (azathioprine, infliximab, methotrexate, or prednisone). Vaccines were up to date according to the vaccine schedule of French recommendations in 24 % of cases and according to the recommendations for inflammatory bowel disease in 4 % of cases. Coverage by vaccine was the following: diphtheria-tetanus-poliomyelitis 87 %, hepatitis B 38 %, pneumococcus 32 %, and influenza 22 %. Immunization delay causes were as follows: absence of proposal 58 %, patient refusal 41 %, fear of side effects 33 %, and fear of disease activation 5 %. Therefore, immunization coverage is insufficient in children with inflammatory bowel disease, due to simple omission or to refusal. A collaboration with the attending physicians and a targeted information are necessary.

Characterization of esophageal motility following esophageal atresia repair using high-resolution esophageal manometry.

BACKGROUND: Esophageal dysmotility, a considerable issue following esophageal atresia (EA) repair, has been reported but has not been precisely described and characterized. Using high-resolution esophageal manometry (HREM), we characterized the esophageal motility patterns in children with repaired EA and compared these patterns of dysmotility with symptomatology. METHODS: HREM was performed as an outpatient procedure in patients with repaired EA. The tracings were analyzed using the software provided by the company and were then reviewed visually. Charts were reviewed for medical/surgical histories and symptoms were assessed by a standardized questionnaire. RESULTS: Forty patients (25 boys, 15 girls) with a median age of 8 years (11 months-18 years) underwent an HREM. Thirty-five patients had type C EA and 5 had type A EA. Only 7 patients were asymptomatic at the time of the examination. HREM results were abnormal in all of the patients. Three different esophageal motility patterns were derived from HREM tracing analysis: aperistalsis (15 patients, 38%), pressurization (6 patients, 15%), and distal contractions (19 patients, 47%). Distal contractions pattern was found exclusively in type C EA. Dysphagia was encountered in the 3 groups. Gastroesophageal reflux disease-related symptoms predominated in the aperistalsis group. CONCLUSIONS: HREM improves our understanding and allows precise characterization of esophageal dysmotility in patients who have undergone EA repair.

Hereditary pancreatitis in children: surgical implications with special regard to genetic background.

PURPOSE: Hereditary pancreatitis (HP) is the primary etiology of chronic pancreatitis during childhood, progressing through recurrent episodes of acute pancreatitis and finally leading to pancreatic insufficiencies. Hereditary pancreatitis is because of mutations of the cationic trypsinogen (PRSS1) gene. Some other genes, such as SPINK1 or CFTR, have been associated with familial idiopathic chronic pancreatitis. The aim of our study was to clearly define diagnostic and therapeutic strategies for HP patients, through an analysis of our study group and a review of the literature. METHODS: All children admitted from 1995 to 2007 with a final diagnosis of hereditary pancreatitis were restrospectively included in the study. We analyzed all medical records with special attention given to cases involving genetic screening (PRSS1, SPINK1, and CFTR genes). RESULTS: Ten children were included. Eight had HP with PRSS1 mutation, 2 of them without a familial history of chronic pancreatitis. The 2 others patients had SPINK1 mutations. Three HP patients were operated on for acute complications of pancreatitis and are well with a mean follow-up of 5.5 years. No patient had pancreatic insufficiencies or weight loss. CONCLUSIONS: Hereditary pancreatitis is associated with severe pancreatitis, with a greater risk of developing pancreatic cancer. It must therefore be diagnosed correctly and treated to prevent its considerable complications.