Spinal dysraphism in exstrophy: a single-center study of a 39-year prospective database
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Brendan F Judy1, Joshua Materi1, Ryan P Lee1
1Departments of1Neurosurgery and.
Insights
Spinal dysraphism occurs in 8.1% of patients with bladder or cloacal exstrophy, most frequently in cloacal exstrophy cases. Neurosurgery timing did not impact lower-extremity motor outcomes in this patient group.
Area of Science:
- Pediatric Surgery
- Neurology
- Urology
Background:
- Spinal dysraphism is a congenital condition affecting the spine.
- Exstrophy is a rare condition involving the bladder or cloaca.
- Co-occurrence of spinal dysraphism and exstrophy requires comprehensive management.
Purpose of the Study:
- To determine the incidence of spinal dysraphism in patients with bladder and cloacal exstrophy.
- To analyze the impact of spinal dysraphism surgery timing on urological and neurological outcomes.
Main Methods:
- Retrospective review of a prospectively maintained IRB-approved database (1982-2021).
- Analysis of 54 pediatric exstrophy patients with documented spinal dysraphism.
- Categorization of spinal dysraphism subtypes and assessment of surgical interventions and outcomes.
Main Results:
- Spinal dysraphism was identified in 114/1401 (8.1%) exstrophy patients; detailed data available for 54.
- Most common in cloacal exstrophy (83.3%), with lipoma-based closed defects (63.0%) being the most frequent subtype.
- Hydrocephalus (24.1%) and Chiari malformation (11.1%) were noted; no significant difference in motor outcomes based on surgery timing.
Conclusions:
- Spinal dysraphism is a significant comorbidity in exstrophy, particularly cloacal exstrophy.
- Surgical intervention for spinal dysraphism has a manageable complication rate.
- Timing of spinal dysraphism surgery relative to exstrophy closure does not appear to affect lower-extremity motor function.
Objective:
The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal dysraphism surgery, including timing of spinal dysraphism surgery, with urological and neurological outcomes.
Methods:
A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function.
Results:
Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
Conclusions:
The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
