Questioning the rationale and conduct of the management of myelomeningocele study

Surgical intervention in fetal spina bifida developed from the belief that amniotic fluid damages the spinal cord in utero and low spinal pressure from failure of neural tube closure causes hindbrain herniation leading to hydrocephalus after birth for many infants with open spinal lesions. Intrauterine intervention is undergoing a randomised human trial known by the acronym MOMS. It is hoped that randomisation and long-term follow up will demonstrate whether benefits to the infant may result from closure of the vertebral defect before birth. It is argued here that the premise upon which the pathogenesis of neural injury in human spina bifida used to launch this study is mistaken. This has implications for the conduct and conclusions of the trial.It is proposed that fetal surgery improves central nervous system outcome by improving cerebrospinal fluid flow at the foramen magnum. Successful intervention results in a more normal development of both neural and skeletal components of the neuraxis. Closure of the defect is required before signs of hindbrain herniation and ventriculomegaly are evident on ultrasound imaging as these are indicators of the presence of fetal hydrocephalus.     

Prospects for fetal surgery

Until about forty years ago, the womb shielded the fetus from observation and therapy. The rapid changes in the diagnosis and treatment of human fetal anatomical abnormalities are due to improved fetal imaging studies as well as fetal sampling techniques (e.g. amniocentesis, chorionic villus sampling), and a better understanding of fetal pathophysiology derived from laboratory animals. Fetal therapy is the logical culmination of progress in fetal diagnosis. In other words, the fetus is now a patient. The fetal surgical treatment of the most severe form of spina bifida – myelomeningocele (MMC) – will be used as a paradigm to illustrate progress in and future prospects for fetal surgery. This review will focus on the rationale for in utero repair in the context of pathologic observations and animal models of MMC, outcomes from human fetal MMC repair including the recently completed Management of Myelomeningocele Study (MOMS trial), and future research directions.     

Multiple organ malrotation syndrome with duodenal atresia: a case report

Duodenal atresia (DA) can be associated with multiple anomalies. Multiple organ malrotation syndrome (MOMS) involves laevoversion of the liver and gall bladder and dextroversion of the stomach and spleen. We report a case of MOMS with DA. Embryologic aspects, investigation, and treatment are discussed. Accepted: 30 October 1997