Search

Introduction: The transanal endorectal pull-through (TERPT) procedure, the latest advancement in the surgical treatment of Hirschsprung's disease, has replaced most other surgical techniques in the last decade., Patients and Methods: Between October 2002 and March 2014, a total of 22 patients diagnosed with Hirschsprung's disease underwent a one-stage TERPT operation., Results: Resected segments included the rectosigmoid (seven patients), the descending colon (10 patients), and the transverse colon (five patients). The minimum length of the resected segments was 15 cm and the maximum length was 65 cm. The mean length was 39.18 ± 12.05 cm. Following surgery, the start of oral ingestion was 1-8 days (mean 3 ± 1.69 days) and the hospital stay after the operation lasted 4-11 days (mean 7.04 ± 2.05 days). The mean follow-up period was 48 ± 6 months (range of 24-166 months). Out of 22 patients, three patients had an anal stricture, which responded to anal dilatations; three patients had an enterocolitis episode that required hospitalization; two patients experienced constipation; and two patients had incontinence/soiling., Conclusion: Our data suggest that the TERPT operation can be safely performed in terms of long-term complications.

Triple A syndrome, also known as Allgrove syndrome, is a rare disease, and presents mainly in children. Its cardinal symptoms are achalasia, alacrima, and adrenocorticotropic hormone (ACTH) insensitivity. We report three cases of Triple A syndrome. Our aim is to inform pediatric surgeons about the existence of this rare syndrome and to highlight the need for suspicion of alacrima and ACTH insensitivity in cases of pediatric achalasia. Triple A syndrome should be considered in patients presenting with achalasia. Alacrima should be investigated by a Schirmer test, and adrenal dysfunction should be tested in cases of suspected triple A.

Introduction The aim of this study was to review a single institution's experience with surgical interventions in children with achalasia and to determine treatment strategies for this rare disorder. Patients and methods This study is a retrospective analysis of 22 cases of childhood achalasia from 1991 to 2013. The patients were evaluated in terms of age, symptoms, interventions, intraoperative complications, and recurrent dysphagia. Results There were 13 boys and nine girls (7 months to 17 years old). The clinical symptoms were vomiting (68%), dysphagia (36%), wheezing (18%), coughing (13%), and weight-loss (13%). The mean duration of symptoms was 2.4 years (1 month to 6 years). A barium contrast X-ray study was performed in all of the patients. Oesophageal manometry was performed in eight patients. Six patients underwent multiple oesophageal dilatations (ED) as a first intervention. A Heller myotomy (HM) and fundoplication were performed in all the patients except two patients who recovered with dilatation. In the long term, one patient had a stricture due to the operation and had to undergo a reoperation. Of the Heller myotomy patients, one had a recurrent stricture that responded to dilatation. No other complications were present. All the patients are now asymptomatic. Conclusion Early diagnosis and prompt surgical treatment is important to prevent growth impairment in childhood achalasia cases. A Heller myotomy followed by a partial anti-reflux procedure is an effective treatment for achalasia in children. Based on our experience, it is superior to oesophageal dilatation therapy.

Phyllodes tumor is a rare primary tumor of the breast. In children and adolescents, it is even rarer with only 20 cases, treatment of which vary in the literature. Herein we report the case of a 13-year-old female patient with a giant benign phyllodes tumor eroding the bottom of the breast skin and causing nipple retraction. We performed breast conservative surgery by mobilizing the areola, using skin flaps and inserting an implant. Breast malignancy, including phyllodes tumor (PT), is very rare in adolescents. PT, previously called cystosarcoma phylloides, consists of leaf-like fronds, from which the tumor gets its name (1, 2). Although PT is most often seen in the fourth decade of life, almost 20 cases have been reported in the adolescent period, most of which are benign. The histologic types are benign, borderline, and malignant, depending on the mitotic rate of the tumor (3, 4)., (© Acta Chirurgica Belgica.)