Congenital myelomeningocele - do we have to change our management?

BACKGROUND: Eagerly awaiting the results of the Management of Myelomeningocele Study (MOMS) and with an increasing interest in setting up intrauterine myelomeningocele repair (IUMR), the optimal management of patients suffering from congenital myelomeningocele (MMC) has become a matter of debate again. We performed a cross-sectional study at our referral-center for MMC to determine the outcome for our expectantly managed patients. MATERIALS AND METHODS: A computed chart review at our institution revealed 70 patients suffering from MMC. Forty-three patients were eligible for the study and analyzed further. A retrospective analysis was performed only in patients that underwent MMC repair within the first two days of life and were seen at our outpatient clinic between 2008 and 2009 for a regular multidisciplinary follow-up. Data were collected on: gestational age (GA) and weight at birth, age at shunt placement and shunt status after the first year of life, radiological evidence for Arnold-Chiari malformation (ACM) and tethered cord (TC), need for surgery for TC, bladder function, lower leg function and educational level. Data were compared to published results for IUMR and to studies of historical controls. RESULTS: Patients were born with MMC between 1979 and 2009 and are now 13.3 ± 8.9 (mean ± SD) years of age. At birth, mean GA was 37.8 ± 2.3 weeks and mean weight was 2921.3 ± 760.3 g, both significantly higher than in IUMR patients. Shunt placement in our cohort was required in 69.8% at a mean age of 16.0 ± 10.7 days, which was less frequent than for historical controls. Amongst our cohort, radiological observations showed 57.1% had ACM II and 41.9% had TC. Only two of our patients underwent a surgical correction for TC. Clean intermittent catheterization was performed in 69.7% of our patients, 56.4% were (assisted) walkers and 64.1% attended regular classes, both comparable to historical controls. CONCLUSIONS: With a close and interdisciplinary management by pediatric surgeons, neurologists and urologists, the long-term outcome of patients suffering from MMC can currently be considered satisfactory. With respect to the known drawbacks of fetal interventions for mother and child, especially preterm delivery, the results of the MOMS trial should be awaited with caution before proceeding with a complex intervention like IUMR.

Diagnostic value of S100B and neuron-specific enolase in mild pediatric traumatic brain injury.

OBJECT: During recent years, several biomarkers have been introduced for use in the diagnosis of traumatic brain injury (TBI). The primary objective of this investigation was to determine if S100B (or S100 calcium-binding protein B) and neuron-specific enolase (NSE) serum concentrations can effectively be used to discriminate between symptomatic and asymptomatic children with minor head trauma. METHODS: The authors conducted a prospective clinical study that involved patients age 6 months to 15 years who had sustained minor head trauma. Children with concomitant extracranial injuries were excluded. Blood samples were obtained within 6 hours of injury to measure S100B and NSE levels in serum. The authors defined 2 diagnostic groups: a mild TBI group (patients with Glasgow Coma Scale [GCS] scores of 13-15) in whom there were clinical signs of concussion (short loss of consciousness, amnesia, nausea, vomiting, somnolence, headache, dizziness, or impaired vision) and a head contusion group (patients with a GCS score of 15) in whom symptoms were absent. Both S100B and NSE concentrations were compared between the 2 groups. Secondary end points were defined as follows: correlation of S100B/NSE and a) the presence of scalp lacerations, b) GCS score, c) age, and d) correlation between S100B and NSE. RESULTS: One hundred forty-eight patients were enrolled (53 in the contusion group, 95 in the mild TBI group). After adjusting for differences in age and time of injury to blood sample withdrawal, there was no significant difference in S100B or NSE between patients in the 2 groups. Scalp lacerations and GCS score had no affect on posttraumatic S100B or NSE concentrations. The correlation between S100B and NSE was significant. Both markers showed a significant negative correlation with age. CONCLUSIONS: The authors demonstrated that S100B and NSE do not discriminate between symptomatic and asymptomatic children with minor head injury. There seem to be limitations in marker sensitivity when investigating pediatric patients with mild TBI.

Periocular hemangiomas in childhood--functional and esthetic results.

INTRODUCTION: Hemangiomas are the most common tumors of the eyelids and orbit in childhood. These tumors can produce ptosis, strabismus and anisometropia, resulting in amblyopia. The treatment of hemangiomas is a challenge with respect to the functional results (prevention of amblyopia) and cosmetic outcome. MATERIAL AND METHODS: The history, clinical findings, magnetic resonance imaging (MRI), management and outcome of 15 children (3-9 months old) with hemangiomas of the eyelids and/or orbital involvement are reviewed. RESULTS: Seven patients with small superficial hemangiomas were merely kept under clinical observation. In seven other patients with threatened or existing occlusion of the visual axis or refractive errors, treatment was indicated. Four children were given local Neodymium: YAG-laser therapy. In patients with large subcutaneous eyelid hemangiomas and involvement of the orbita we decided to treat with interstitial Neodymium: YAG-laser therapy in combination with systemic corticosteroids. One patient with an unknown tumor in the medial canthus was diagnosed by biopsy. During the follow-up period of 12-24 months, all untreated patients and 6 of 7 treated children showed involution of their tumors. One patient with a large eyelid and orbital hemangioma with occlusion of the visual axis did not respond to laser therapy and systemic corticosteroids; excision of the tumor was necessary. All children with eyelid hemangiomas with orbital involvement (n = 3) suffered from anisometropia with astigmatism and were treated for amblyopia. During 24 months of amblyopia treatment, the visual acuity improved in two of three cases to 0.4 and 1.0. CONCLUSION: Periorbital hemangiomas must be managed by individual and interdisciplinary diagnostic and therapeutic approaches. Therapy of amblyopia remains mandatory in all cases of large eyelid hemangiomas and/or orbital involvement.