An unusual case of 4 level spinal dysraphism: Multiple composite type 1 and type 2 split cord malformation, dorsal
Ashutosh Khandelwal1, Vivek Tandon, Ashok K Mahapatra
1Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Insights
This study presents a rare pediatric case of complex spina bifida with multiple split cord malformations (SCM) and associated anomalies, supporting the neuroenteric canal theory. Complete spinal MRI is crucial for diagnosing these complex congenital conditions.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neuroscience
- Spinal Dysraphism Research
Background:
- Spina bifida encompasses a spectrum of congenital vertebral defects.
- Split cord malformation (SCM) is a rare subtype, often presenting with complex associated anomalies.
- The neuroenteric canal theory proposes a link between early embryonic development and these malformations.
Observation:
- A pediatric patient presented with a complex spina bifida involving two distinct types of split cord malformation (SCM type 1 and type 2).
- The patient also exhibited a non-terminal myelocystocele, coccygeal dermal sinus, bifid fatty filum, and hydrocephalus.
- Clinical presentation included a leaking myelocystocele and bilateral lower limb weakness.
Findings:
- Surgical intervention involved multiple procedures including removal of SCM types 1a, 1c, and 2, repair of myelocystocele, shunt placement, and excision of sinus and filum.
- Clinicoradiological findings support the multiple accessory neuroenteric canal theory for the development of composite SCM.
- The case highlights the intricate nature of congenital spinal anomalies.
Implications:
- Complete Magnetic Resonance Imaging (MRI) screening of the entire spine and brain is essential for comprehensive diagnosis of SCM and associated conditions.
- Early and accurate diagnosis is critical for appropriate surgical management and improved patient outcomes.
- This case contributes to understanding the embryological basis of complex spinal dysraphisms.
Abstract:
The authors here have reported a rare case of a child with a complex spina bifida with two different levels of split cord malformation (SCM) type 1 and single level type 2, a non terminal myelocystocele, coccygeal dermal sinus, bifid fatty filum and hydrocephalus, which substantiates the neuroenteric canal theory and have further tried to highlight the importance of complete Magnetic resonance imaging (MRI) screening of the whole spine and brain with SCM to rule out other associated conditions. The patient was admitted with a leaking myelocystocele with bilateral lower limb weakness. MRI of whole spine with screening of brain was done. Patient underwent 5 operations in the same sitting- (According to classification given by Mahapatra et al.) removal of SCM type 1a at D7-8; removal of SCM type1c at L2-3; removal of SCM type 2 at D10; repair of non terminal myelocystocele at D6-D10; low pressure ventriculoperitoneal shunt on right side with excision of dermal coccygeal sinus; and, excision of bifid fatty filum. The clinicoradiological findings in our patient further substantiate the multiple accessory neuroenteric canal theory in the development of composite type of SCM. The physical and neurological signs of SCM and nonterminal myelocystocele should prompt the neurosurgeon to consider performing the screening MRI of whole spine with brain to rule out other composite types of SCM and hydrocephalus.
