Myelomeningocele - a single institute analysis of the years 2007 to 2015.

BACKGROUND: After the introduction of folate supplementation, the number of open spinal dysraphism was successfully reduced over time. In 2007, the department for pediatric neurosurgery was established in the children's hospital. Since then, newborns with myelomeningocele (MMC), the most common form of open neural tube defects (NTD), are treated here. The aim of this study is to present the concepts applied to and experiences resulting from treatment of MMC. METHODS: Records of all newborns with MMC treated surgically during the period January 2007 to August 2015 in our institution were analyzed. Children, who were previously operated in utero were excluded. The type of neural tube defect, its location, associated comorbidities, and ambulation were recorded. RESULTS: Forty-eight children (25 males, 23 females) with spinal dysraphism were included in the analysis. In nearly 90 % of the cases, the repair of the MMC was done on the day of delivery. The follow-up period ranges from 9 weeks to 8 9/12 years (loss of follow-up in 2 cases). In 19 %, the defect remained undetected during gestation and in one case, carbamazepine was taken despite pregnancy. In 36 children (75 %), we found a Chiari malformation type II (CMII) associated with myelomeningocele. 85.4 % suffered from hydrocephalus and implantation of a shunt was necessary. In cases of bladder impairment, an intermittent catheterization was the most common management (83.3 %); no bladder augmentation was required. Twelve children required orthopedic surgery. Twenty-three of 33 patients (70 %) are ambulatory w/wo orthoses and devices. The 13 children who are younger than 2 years were considered separately to assess the motor activity safely. CONCLUSIONS: Our data show that neural tube defects to this day can remain undetected despite medical care during pregnancy. The most common associated diseases with MMC are Chiari II malformations and hydrocephalus. In the seven cases of simultaneous repair of MMC with shunt implantation, no additional complications were encountered. An interdisciplinary approach was allowed in a high percentage independence and social continence.

Antigen-positive platelet transfusion in neonatal alloimmune thrombocytopenia (NAIT).

Neonatal alloimmune thrombocytopenia (NAIT) is a fetomaternal incompatibility most commonly induced by maternal anti-HPA-1a, IgG alloantibodies against a polymorphic epitope of the glycoprotein IIb/IIIa complex in approximately 97.5% of white patients. Current guidelines recommend transfusion of immunologically compatible platelets to prevent cerebral hemorrhage, the most severe complication in affected newborns. Such platelet concentrates, however, are often not readily available. In a retrospective analysis in German and Canadian centers, 27 newborns with NAIT were identified who received platelets from random donors. Unexpectedly, 24 of 27 newborns showed an increase above a threshold of 40 x 10(9) platelets per liter, with moderate (n = 8) or significant (n = 16) platelet count increments (more than 80 x 10(9)/L). We conclude that transfusion of platelet concentrates from random donors is an appropriate strategy in the management of unexpected, severe NAIT predominantly in first pregnancies, pending the availability of compatible platelets.