Tethered cord secondary to focal nondisjunction of the primary neural tube: experience from a Singapore children's hospital.

Abstract

Purpose: Tethered cord due to focal nondisjunction of primary neuralisation (FNPN) is a rare form of spinal dysraphism. We present our institutional experience in managing children diagnosed with FNPN.

Materials and methods: This is a single institution, retrospective study approved by the hospital ethics board. Patients below 18 years of age diagnosed with CDS, LDM or their mixed lesions, and subsequently underwent intervention by the Neurosurgical Service, KK Women's and Children's Hospital, are included.

Results: From 2001 to 2021, 16 FNPN patients (50% males) were recruited. Eight of them had CDS (50.0%), seven had LDM (43.8%), and one patient had a mixed CDS and LDM lesion (6.2%). The average duration of follow up was 5.7 years and the mean age of surgery was 6 months old. Thirteen patients underwent prophylactic intent surgery (81.2%) and three had therapeutic intent surgery (18.8%). All patients did not have new neurological deficit or required repeat surgery for cord retethering. We observed that detethering surgery performed at or less than three months old was associated with having a wound infection (p = .022).

Conclusions: Our study reports that early recognition and timely intervention are mainstays of management for FNPN. We advocate a multi-disciplinary approach for good outcomes.