Long-term outcomes of children with transanal endorectal pull-through and a review of the literature.

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.

How to manage an unresectable or recurrent sialoblastoma.

Only 2-5% of all salivary gland tumors occur in children. Sialoblastoma is an extremely rare salivary gland tumor diagnosed at birth or shortly thereafter with significant variability in histological range and clinical course, so that it may be difficult to predict the most appropriate therapy. In cases where surgical removal is not curative or technically feasible, chemotherapy may be attempted. We report herein a patient with progression of a huge partially resected sialoblastoma who was successfully treated with chemotherapy. Systemic chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) seems to be an effective adjuvant or neoadjuvant treatment option for unresectable or recurrent sialoblastoma.

Is there a determining factor that predicts mortality in patients with congenital diaphragmatic hernia?

AIM: This study was designed to investigate the factors affecting the prognosis in neonates with congenital diaphragmatic hernia (CDH) who were treated in our clinic. These factors included prenatal lung-head ratio (LHR), prenatal stomach and liver presence in the thorax, blood gases in the first 24 h and the modified ventilation index (MVI). MATERIAL AND METHODS: The study was carried out retrospectively in 30 neonates with prenatally diagnosed left CDH who were treated in our clinic between January 2007 and 2013. Data were collected, evaluated, and statistically analyzed for gender, birth weight, gestational age, prenatal LHR, prenatal presence of stomach and liver in the thorax, postnatal initial blood gases in the first 24 h and MVI. RESULTS: The median LHR for non-survivors was 1.49 and for survivors 1.51. No statistically significant difference in LHR was detected between survivors and non-survivors. In 19 neonates, prenatal ultrasonography (USG) revealed intrathoracic stomach, and 9 of these infants died. Intrathoracic liver was seen in 15 neonates, and 9 of these died. A statistically significant difference was not found between survivors and non-survivors in the intrathoracic liver or intrathoracic stomach neonates. A comparison between the non-survivors and survivors showed a median pH value of 7.10 in non-survivors and 7.24 in survivors (p = 0.002). The median PaCO2 value was 69.4 mm Hg in non-survivors and 51.9 mm Hg in survivors (p = 0.01). There were statistically significant differences in pH and PaCO2 values. The median value of MVI was 33 in survivors and 100 in non-survivors. There was a statistically significant difference between overall non-survivors and survivors in the MVI value (p < 0.05). CONCLUSIONS: Based on the findings, postnatal pH, and PaCO2 and MVI values are favorable prognostic factors in CDH in our selected group of patients.