Multiple neural tube defects: a rare combination of limited dorsal myeloschisis, diplomyelia with dorsal bony spur, sacral meningocoele, syringohydromyelia, and tethered cord

Abstract

Multiple neural tube defects are relatively rare. They account for less than 1% reported neural tube defects. Cases of limited dorsal myeloschisis (LDM) and diplomyelia (two cords in single sac without intervening bony or fibrous septae) with dorsal bony spur are also a rare event. Here, the authors report a rare case of neonate with thoracic LDM, diplomyelia with dorsal bony spur, sacral meningocoele with syringohydromyelia, and low-lying tethered cord. The child also had a ventricular septal defect (VSD) and bilateral rocker bottom feet. Various environmental factors and genetic mutations in transmembrane proteins have been studied in animal models explaining the origin of neural tube defects. To the best of author's knowledge, this is the first case of varied multiple neural tube defects with diplomyelia reported in world literature.

Limited Dorsal Myeloschisis with and without Type I Split Cord Malformation: Report of 3 Cases and Surgical Nuances

“…Lee et al reported 33 patients with LDM and none of them had SCM associated with LDM [2]. Shashank et al [7] reported a case of LDM associated with diplomyelia with dorsal bony spur, sacral meningocele, and syringohydromyelia. This case was a newborn and they performed early surgical treatment for each lesion [7].…”

“…Shashank et al [7] reported a case of LDM associated with diplomyelia with dorsal bony spur, sacral meningocele, and syringohydromyelia. This case was a newborn and they performed early surgical treatment for each lesion [7]. Huang et al [11] reported 10 patients with cervical myelomeningocele in 2010 and only one had Type I SCM as associated malformation.…”

“…Since the first description of LDM, a few reports have been published on the coexistence of LDM and SCM [1,6,7]. None of them had type I SCM and no detailed surgical technique was described.…”

Praktischer Ansatz für die Diagnose von Wirbelsäulendysplasien

“…Multiple Formen der spinalen Dysraphie oder Neuralrohrdefekte an mehreren Stellen sind selten und machen weniger als 1 % der Neuralrohrdefekte aus [49,50]. Sie sind durch das Vorhandensein von mehr als einer Fehlbildung mit normalem Nervengewebe dazwischen definiert.…”