Transumbilical management for neonatal ovarian cysts.

BACKGROUND/PURPOSE: Since Tan and Bianchi (Br J Surg. 1986;73:399) reported umbilical incision as an access for pyloromyotomy in infantile hypertrophic pyloric stenosis, many pediatric surgeons have used this approach for a number of other procedures. Because of the long pedicle with good mobility and the frequent intraabdominal position of the neonatal ovarian cyst, we attempted to manage it via the transumbilical route. METHODS: All patients were treated under intubation general anesthesia. Semicircular infraumbilical incision was made, and the abdomen was entered through a transverse fascial incision. The partially collapsed cyst after aspiration was exteriorized through the incision for cystectomy, partial deroofing, or adnexectomy. RESULTS: From May 2000 to December 2006, 6 female newborns with ovarian cysts were treated via the transumbilical route. There were no complications from surgery. The operation time and duration of hospital stay were short. The cosmetic appearance after the procedure was good. CONCLUSIONS: The initial result suggests that transumbilical management for neonatal ovarian cysts may be a good alternative procedure when laparoscopic equipment is unavailable or experienced technique is lacking.

Pneumatic dilation in treatment of late-onset primary gastric outlet obstruction in childhood.

Late-onset primary gastric outlet obstruction in childhood is a rare condition. Only 8 cases of such were reported. Diagnosis should be considered while ruling out mechanical and structural lesions. Up to now, Heineke-Mikulicz pyloroplasty has been the standard treatment. However, we succeeded in treating this condition by using pneumatic dilation. There is no sign of recurrence for 1 year. We propose the etiology, diagnosis, and the relationship between late-onset primary gastric outlet obstruction and esophageal achalasia in childhood.

Low RET mutation frequency and polymorphism analysis of the RET and EDNRB genes in patients with Hirschsprung disease in Taiwan.

Hirschsprung disease (HSCR), or congenital intestinal aganglionosis, is a relatively common disorder characterized by the absence of ganglion cells in the nerve plexuses of the lower digestive tract, resulting in intestinal obstruction in neonates. Mutations in genes of the RET receptor tyrosine kinase and endothelin receptor B (EDNRB) signaling pathways have been shown to be associated in HSCR patients. In this study, we collected genomic DNA samples from 55 HSCR patients in central Taiwan and analyzed the coding regions of the RET and EDNRB genes by PCR amplification and DNA sequencing. In the 55 patients, an A to G transition was detected in two (identical twin brothers). The mutation was at the end of RET exon 19 at codon 1062 (Y1062C), a reported critical site for the signaling pathways. Single nucleotide polymorphisms (SNP) in exons 2, 7, 11, 13, and 15 of RET and exon 4 of EDNRB in the HSCR patients or controls were detected. The differences between patients and controls in allele distribution of the five RET polymorphic sites were statistically significant. The most frequent genotype encompassing exons 2 and 13 SNPs (the polymorphic sites with the highest percentage of heterozygotes) was AA/GG in patients, which was different from the AG/GT in the normal controls. Transmission disequilibrium was observed in exons 2, 7, and 13, indicating nonrandom association of the susceptibility alleles with the disease in the patients. This study represents the first comprehensive genetic analysis of HSCR disease in Taiwan.