Successful childbirth in a Fontan with protein losing enteropathy.

As survival rates and functional status of the adult single ventricle population have grown, some may become pregnant inadvertently or against our advice. The outcomes are often poor, being worse for the fetus/baby rather than the mother with a Fontan circuit. No reports of a successful delivery of a healthy baby to a Fontan mother with protein losing enteropathy were found in the literature. We present one such case.

Single center protocol driven care in 150 patients with gastroschisis 1998-2017: collaboration improves results.

PURPOSE: The treatment of gastroschisis (GS) using our collaborative clinical pathway, with immediate attempted abdominal closure and bowel irrigation with a mucolytic agent, was reviewed. METHODS: A retrospective review of the past 20 years of our clinical pathway was performed on neonates with GS repair at our institution. The clinical treatment includes attempted complete reduction of GS defect within 2 h of birth. In the operating room, the bowel is evaluated and irrigated with mucolytic agent to evacuate the meconium and decompress the bowel. No incision is made and a neo-umbilicus is created. Clinical outcomes following closure were assessed. RESULTS: 150 babies with gastroschisis were reviewed: 109 (77%) with a primary repair, 33 (23%) with a spring-loaded silo repair. 8 babies had a delayed closure and were not included in the statistical analysis. Successful primary repair and time to closure had a significant relationship with all outcome variables-time to extubation, days to initiate feeds, days to full feeds, and length of stay. CONCLUSION: Early definitive closure of the abdominal defect with mucolytic bowel irrigation shortens time to first feeds, total TPN use, time to extubation, and length of stay.

Perinatal management of gastroschisis: analysis of a newly established clinical pathway.

PURPOSE: The authors developed a clinical pathway for optimal management after antenatal diagnosis of gastroschisis. This is the outcomes analysis of our first 30 consecutive patients. METHOD: Antenatal counseling was provided for all families with in-utero diagnosis of gastroschisis. Bowel dilatation, thickness, motility, amniotic fluid volume, and fetal development were followed by ultrasonography every 4 weeks. Babies were delivered by cesarean section between 36 and 38 weeks gestation if the lungs were mature or earlier for bowel complications. Gastroschisis repair was scheduled 90 minutes after birth. Primary repair was attempted in all through the abdominal wall defect without an additional incision, resulting in an umbilicus with no abdominal scar. RESULTS: Primary repair was achieved in 83%. Babies needed assisted ventilation for 3 days, reached full feeds by 19 days, and were discharged by 24 days (all medians). There were 3 (10%) deaths, all after staged repair. CONCLUSIONS: Our new protocol of both scheduled elective cesarean section and early gastroschisis repair resulted in a higher proportion of primary repair, shorter duration of mechanical ventilation, earlier full feeds, and shorter length of stay. There was no increase in mortality or morbidity. The primary-repair babies had no mortality and had excellent cosmesis.