Erdal Türk

Waardenburg syndrome with extended aganglionosis: report of 3 new cases

PURPOSE The Waardenburg-Shah syndrome is an autosomal recessive disease with varied penetration where Hirschsprungs disease and the Waardenburg syndrome are seen together. Although the length of the involved intestinal segment varies in this syndrome, most patients had total colonic aganglionosis with or without small bowel involvement. We present in this study 2 siblings and one first-degree relative for a total of 3 male patients with Waardenburg syndrome and total colonic aganglionosis with or without small bowel involvement, together with their clinical characteristics and treatment methods. PATIENTS The patients who presented with intestinal obstruction findings within the first 48 hours after birth were operated on with 2 patients under elective conditions and 1 as an emergency. The ganglionic segment lengths were 6, 8, and 20 cm, respectively. Aganglionic enterostomy was performed, and the Ziegler operation was used for these patients. The enterostomies started to function on the third postoperative week, and they started to gain weight. However, all died because of sepsis on the 5th to 12th month. CONCLUSION Waardenburg-Shah syndrome patients have a higher incidence of total colonic aganglionosis with or without small bowel involvement. The Ziegler operation may be used in patients with inadequate ganglionic bowel length to gain some time for the child to grow and to decrease total parenteral nutrition complications.

Surgical management of complete penile duplication accompanied by multiple anomalies

Diphallus (penile duplication) is very rare and seen once every 5.5 million births. It can be isolated, but is usually accompanied by other congenital anomalies. Previous studies have reported many concurrent anomalies, such as bladder extrophy, cloacal extrophy, duplicated bladder, scrotal abnormalities, hypospadias, separated symphysis pubis, intestinal anomalies and imperforate anus; no penile duplication case accompanied by omphalocele has been reported. We present the surgical management of a patient with multiple anomalies, including complete penile duplication, hypo-gastric omphalocele and extrophic rectal duplication.

Farklı bir klinik tabloyla başvuran, sigmoid ve inen kolon yerleşimli mezenterik kist: Olgu Sunumu

The colon mesentery is the origin of approximately 20% of childhood mesenteric cysts. Although these cysts are mostly asymptomatic, they may cause various clinical pictures according to the dimension, localization, and content of the cyst and whether it is complicated. Children may therefore present with symptoms of abdominal pain, nausea, vomiting, constipation, diarrhea, weight loss, abdominal mass, distention, and may also suffer from various findings related to the cyst such as torsion, infection, hemorrhage and rupture. We present a mesenteric cyst case originating from the descending and sigmoid colon mesentery that presented with symptoms of fever, abdominal pain and diarrhea considered to be due to the infection of the cyst in the descending colon