Association between cloacal exstrophy and two limited dorsal myeloschisis lesions: A case report and literature review

Abstract

Background: The term limited dorsal myeloschisis (LDM) was coined by Pang in 1993. It involves incomplete obliteration of the dorsal neural fold in primary neurulation. On the other hand, Cloacal Exstrophy is one of the most complex endodermal anomalies and is usually associated with spinal anomalies; hence, the term OEIS complex was coined (Omphalocele, Exstrophy of the cloaca, Imperforate anus, and Spinal deformities complex). Case Description: We report the coexistence of two LDM lesions in tandem (originating at L2 and S2 levels) in a child diagnosed with cloacal exstrophy. Initially, prenatal ultrasound detected the lumbar but not the sacral lesion. The patient was surgically untethered under intraoperative neurophysiologic monitoring (IOM) at four years of age, and this paper reports his one-year follow-up. Conclusion: Cases of cloacal exstrophy must always be investigated for spinal cord malformations. LDM is rare and requires careful diagnosis. MRI should be done for the whole neuroaxis to rule out associated conditions, including multiplicity. Surgery should be done under IOM to avoid long-term complications.