Congenital myelomeningocele - do we have to change our management?

Abstract

BackgroundEagerly 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 methodsA 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.ResultsPatients 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.ConclusionsWith 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.

Comparison of Follow-Up Length-Matched Single-Center Myelomeningocele Postnatal Closure Cohort to the Management of Myelomeningocele Study (MOMS) Trial Results

“…For patients with myelomeningoceles, hydrocephalus is common [6][7][8][9][10][11][12][13]. Traditionally, shunting rates in the literature have ranged from 57 to 86% [14][15][16][17][18][19]. In 2018, Kim et al [20] reported that the rate of surgical treatment for hydrocephalus in the National Spina Bifida Patient Registry was 79.99%.…”

Body composition and energy expenditure in a population of children and adolescents with myelomeningocele

“…1 The incidence of myelomeningocele is approximately 1-2 cases every 1000 live births but differs widely from one country to the other. [2][3][4] A n a b n o r m a l i n t r a u t e r i n e position causes significant congenital orthopedic diseases that worsen during early childhood due to muscle imbalance, postural effects, growth, etc. Eighty percent of children with upper lumbar injuries have short stature; the causes include lower limb hypoplasia, scoliosis, 1 recurrent urinary tract infections, chronic renal failure, etc.…”

Postnatal myelomeningocele closure in smallest reported neonate: illustrative case

“…For comparison, the literature puts the average weight of a patient undergoing ex utero MMC closure at approximately 3 kg. 7 , 8 Performing an operation on an extremely low birth weight neonate presents many risks and challenges, regardless of the type of surgery. The surgical technique is complicated by diminutive anatomical structures; meticulous hemostasis is essential; and maintenance of physiological conditions for the patient (temperature, hemodynamics) requires specialized anesthesiology care.…”