2011/11/29 by David Shurtleff · 1 citation
Medicine · #Spinal Dysraphism and Malformations #Congenital Anomalies and Fetal Surgery #Congenital Diaphragmatic Hernia Studies
paper · pdf · doi:10.1111/j.1469-8749.2011.04141.x
openalex publication_date 2011/11/29 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/29
The interesting paper by Verbeek et al.1 describes two separate technological advances applied to myelomeningocele. The first, muscle ultrasound density, is not evident in either the title of the article or the conclusion. It has the potential of being useful in distinguishing between toxicity from late gestational amniotic fluid to exposed nerves2 and the trauma of delivery.3–5 To do so would require comparing patients with prenatally repaired myelomeningocele lesions to children with myelomeningocele and functional leg muscles, and a sac protruding 2cm or more beyond the dorsal plane of the back prior to rupture of the amniotic membranes.4,5 Muscle ultrasound density would be more useful in predicting future lower extremity function in the newborn with myelomeningocele if used to more accurately display individual muscle density over time.6,7 Fetal endoscopic myelomeningocele closure, the second technological advance, is listed in both the title and conclusion of the article. Hence, this article will be quoted as supporting the procedure. This is unfortunate since myelomeningocele is an extremely complicated congenital malformation of the central nervous system and the spine.8,9 Its history reveals introduction of definitive treatments that have later been discarded or altered, followed by decades of controversy. There are two major areas of concern about children with myelomeningocele born following prenatal repair reported in this article and repair following hysterotomy:10 they are improved intellectual function and improved ambulatory status. Hysterotomy and myelomeningocele repair has fewer complications for both mother and infant but still has led to criticism and recommendations for long-term evaluation.11–14 These two major areas of concern apply to intrauterine endoscopic as well as to open myelomeningocele repair. The first concern is improved central nervous system function that cannot be determined until the child with myelomeningocele is much older.8 The frequency of very early preterm birth in both cohorts raises the possibility of superimposed intellectual impairment on the multiple supratentorial malformations associated with myelomeningocele.8,9 Additionally, the 12 children (out of 13) with ‘infant respiratory distress syndrome’ reported in Table I1 raises the question as to whether these infants may have neurological impairment of respiratory regulation associated with the Chiari II malformation and/or lung immaturity. Likewise, the claim of significantly better muscle function needs further, long-term study for three reasons. (1) The severe scarring demonstrated by Cremer and Messing-Junger15 and the reports of very early identification of ‘tethered spinal cord’ in both this paper and the MOMS study10 raise the question of loss of muscle function as these children grow older.11–13 (2) The prognosis for muscle strength and ambulation ability later in life cannot be reliably determined from examination of very young children.16 (3) Muscle strength of newborn infants can either deteriorate or improve during the 18 months after birth and surgery due to slow neuronal recovery from trauma. In summary, there are two major conclusions about this paper. (1) The unique collaboration demonstrated by the authors, the close proximity of the patients’ homes to centers for high quality follow-up care in Europe, and the socioeconomics of European medicine (as opposed to the USA) offer a more practical setting for the study of the comparison of prenatal myelomeningocele repair to delivery by Cesarean section as defined above. Such a study would solve the question as to the relative contribution of toxicity of late gestational amniotic fluid and the trauma of birth. (2) The extremely high complication rates for mother and infant in this study and the principle of primum non nocere indicate that at this time it is unethical to pursue intrauterine endoscopic myelomeningocele repair in humans until the procedure has been perfected in animals.