History of Management of Myelomeningoceles in Children

Understanding of Disease

  • Described in 500 BC: In the 4th and 5th centuries B.C., Aristotle and Hippocrates both described myelomeningocele (1, 48). Most children were left to die.
  • 1610 – First surgery: As early as 1610, the first surgical excision of a myelomeningocele was reported (2)
  • 1860 – first pathological description: John Cooper Forster published the first account of our modern pathologic understanding of myelomeningocele in his 1860 text Surgical Disease of Children (2)
  • Mid-20th Century – improved apparatus: Little progress was made until clean intermittent bladder catheterization (CIC), valved shunt systems, and external orthoses were developed in the middle of the 20th century; these interventions revolutionized care, improving both the life expectancy and quality of life in this population (1, 49).
  • 1971 – Lorber treatment criteria: Selective criteria for treatment established by Lorber in 1971 led to slightly higher IQ and no other significant functional improvements at the cost of drastically increased mortality. Lorber’s suggestion to treat only mildly affected cases was rejected by the medical community led by McLone. Currently, all myelomeningoceles that are not associated with other fatal anomalies are repaired (1, 3-7).
  • 1991 – Folate supplementation: The addition of folate to the diet was recommended by the FDA after the multi-center, multi-national randomized prevention trial by the British Medical Research Council (MRC) Vitamin Study Group was stopped early due to a clear benefit in folic acid supplementation. The incidence of myelomeningocele in live births dropped by ~50% (1, 50).
  • 2011 – MOMS trial: The MOMS trial reports a reduction in the need for shunting and improved motor outcomes at 30 months in a randomized trial of prenatal (in utero) versus postnatal repair of myelomeningocele. There were associated fetal and maternal complications (8).

Technological Development

  • 1950s – valved shunt systems: The advent of valved shunt systems reduced mortality from hydrocephalus
  • 1970s – clean intermittent bladder catheterization: The introduction of clean intermittent bladder catheterization improved the quality of life and reduced incontinence and urinary tract infection rates.
  • Prenatal diagnosis: Prenatal diagnosis via maternal serum AFP, amniocentesis, high-resolution ultrasound, and fetal MRI allows parents and providers to discuss options for termination of pregnancy, modes of delivery, and, more recently, prenatal surgery.

Surgical Technique

  • Early treatments focused on elimination of sac: Serial tapping, ligature/excision, and instillation of sclerosing agents were early attempts at treatment from the 17th to the 19th centuries (2).
  • Reconstructive closure with introduction of aseptic technique: At the end of the 19th century, aided by aseptic technique, several surgeons published successful multilayer closure techniques, some including muscle and rotational skin flaps. Nonsurgical techniques were abandoned (2).
  • Multilayer reconstructive closure by 1920s: By the 1920s, formal pial, dural, muscular, fascial, and skin closure was described. Surgical timing and patient selection were debated over the remaining decades of the 20th century (2).
  • Prenatal closure: Prenatal surgery performed in specialized centers is an intriguing new option, as suggested by the MOMS trial (8).