Closer location of the tracheoesophageal fistula to the carina in newborns with esophageal atresia and VACTERL association.

BACKGROUND: VACTERL is a complicated syndrome with an unknown etiology where many studies have failed to identify the cause. In esophageal atresia (EA) roughly 10%-23% also have concurrent anomalies that align with VACTERL disorder. The aim of this study is to investigate if there is a difference regarding localization of the tracheoesophageal fistula between patients with VACTERL and non-VACTERL patients. METHODS: Retrospective chart review of newborn operated for esophageal atresia between 2006 and 2016 at our Institution was performed. Children with a C-type fistula according to Gross and reliable preoperative tracheoesophageal fistula to carina distance measurement at rigid tracheoscopy were included in the study. RESULTS: A total of 90 patients were included in the study. Fifteen of those were diagnosed with VACTERL. Before and after adjusting for weight and gestational week patients with VACTERL had significantly shorter carina to fistula distance at perioperative rigid tracheoscopy (p = <0.001 nonadjusted, p = 0.016 adjusted). CONCLUSION: Patients with VACTERL born with EA type C had shorter carina to fistula distance as shown at perioperative rigid tracheoscopy. The significantly shorter distance may not only present surgical difficulties but may also suggest a structural or molecular difference in the development of the esophageal atresia between the two groups. LEVEL OF EVIDENCE: III.

Outcomes in infants with prenatally diagnosed gastroschisis and planned preterm delivery.

BACKGROUND: The timing and mode of delivery of pregnancies with prenatally diagnosed gastroschisis remains controversial. AIM: To evaluate the outcome of patients with gastroschisis managed during two time periods: 2006-2009 and 2010-2014, with planned elective cesarean delivery at 37 versus 35 gestational weeks (gw). A secondary aim was to analyze the outcome in relation to the gestational age at birth. MATERIAL AND METHODS: Retrospective review of all cases with gastroschisis managed at our institution between 2006 and 2014. RESULTS: Fifty-two patients were identified, 24 during the initial period, and 28 during the second. There were a significantly higher number of emergency cesarean deliveries in the first period. There were no differences between groups with regard to the use of preformed silo, need of parenteral nutrition or length of hospital stay. When analyzing the outcome in relation to the gw the patients actually were born, we observed that patients delivered between 35 and 36.9 gw were primary closed in 88.5% of cases, with shorter time on mechanical ventilation, parenteral nutrition and hospital stay. CONCLUSION: Planned caesarian section at 35 completed gestational weeks for fetuses with prenatally diagnosed gastroschisis is safe. We observe the best outcome for patients born between 35 and 36.9 gw.

Partial gastric pull-up in the treatment of patients with long-gap esophageal atresia.

BACKGROUND: This study was to analyze outcomes of long-gap esophageal atresia (LGEA) treated with partial gastric pull-up (PGP) into the thorax. METHODS: The medical records of all children who had undergone PGP for LGEA from 1999 to 2012 were reviewed. Preoperative data, initial postoperative course, complications, time to full oral nutrition, follow-up diagnostics and nutritional status were assessed. RESULTS: Nine children who had undergone PGP were followed up for a mean period of 6.2 ± 3.1 years. Their median gestational age was 37 ± 2 weeks, and mean birth weight 2462 ± 658 g. Eight children were primarily treated with a gastrostomy, their mean age at PGP was 11.4 ± 10.9 weeks and mean weight was 4484 ± 1966 g. Their mean operation time was 199 ± 51 minutes. Leakage was an early postoperative complication in three children, one of whom had a consecutive stricture resection. Late complications were stenosis (n=7) and gastro-esophageal reflux (n=5). The general status of the children was judged as "good" or "very good" on the last presentation. The median percentile of the body-mass-index was 25. Gastroscopy at 3.7 ± 3.2 years after the operation revealed a grade I esophagitis in two children. There was no death in this group of children. CONCLUSIONS: Because of its high complication rate, partial gastric pull-up cannot be recommended as an alternative for the treatment of LGEA at present. A final judgment could be made on the basis of a comparative study.

Growth of spontaneously descended and surgically treated testes during early childhood.

OBJECTIVE: To investigate whether in congenital unilateral cryptorchidism the growth of a spontaneously descended testis is normal, compared with the contralateral scrotal testis or similar to the growth of testes that failed to descend spontaneously and later underwent orchidopexy. METHODS: Ninety-one boys with congenital unilateral cryptorchidism with later spontaneous descent of the initially retained testis were followed from birth (0-3 weeks) up to 5 years of age and compared with boys randomized to surgery at either 9 months (n = 78) or 3 years (n = 85) of age. Testicular volume was determined with ultrasonography. RESULTS: Eighty-two percent of spontaneous descent occurred before 2 months of age. Twenty-two percent of these descended testes were later again found in a retained position. The spontaneously descended testis was smaller than its scrotal counterpart at all ages (P < .001). We also showed a significant difference in the testicular volume between the early and late treated boys from age 2 years and onward. At 2, 4, and 5 years of age, the volumes of the spontaneously descended testes were significantly larger than those of boys operated on at 3 years but similar to those operated on at 9 months. CONCLUSIONS: We have shown that in boys with congenital unilateral cryptorchidism with later spontaneous descent, the originally retained testes show impaired growth compared with its scrotal counterpart from birth and onwards. Also, they are prone to later ascent to a retained position. Furthermore, the longer testes remain untreated the more they exhibit impaired growth.

Surgical treatment of unilaterally undescended testes: testicular growth after randomization to orchiopexy at age 9 months or 3 years.

PURPOSE: We compared the growth of congenital, unilaterally undescended testes following orchiopexy at age 9 months or 3 years. MATERIALS AND METHODS: Patients were randomized to surgery at age 9 months (72) or 3 years (83). Testicular volume was measured by ultrasonography at ages 6, 12, 24, 39 and 48 months. RESULTS: Orchiopexy at age 9 months resulted in an increase in testicular volume at subsequent measurements at ages 2, 3 and 4 years compared to the volume at 6 months (p <0.001). In contrast, no significant growth was noted in the group treated at age 3 years. The improved testicular growth after early orchiopexy was also demonstrated by a gradual increase in the ratio of the previously retained testis and the scrotal testis in individual boys from 6 months to 4 years (0.68 to 0.81, p <0.001). For the late treatment group a significant decrease in this ratio was noted during the same period (0.68 to 0.56, p <0.01). CONCLUSIONS: Surgical treatment at 9 months resulted in partial catch-up of testicular growth until at least age 4 years compared to surgery at 3 years, clearly indicating that early surgery has a beneficial effect on testicular growth. Since testicular volume is an approximate indirect measure of spermatogenic activity, this gives hope that orchiopexy at this age may improve future spermatogenesis.