Correlation of Cutaneous Lesions with Clinical Radiological and Urodynamic Findings in the Prognosis of Underlying Spinal Dysraphism Disorders

Abstract

Spinal dysraphism is characterized by a lack of fusion of the vertebral arches that occurs in the absence of spinous processes with variable amounts of lamina. Here, we retrospectively present the importance of cutaneous lesions and their correlation with clinical presentation, radiological examination and urodynamic assessment. We retrospectively reviewed 612 (6.12%) cases with skin lesions from 10,000 consecutive live-born children seen at two institutions between January 1998 and March 2005. We divided all children into a control group and three groups based on clinical assessment, radiological examination and urodynamic evaluation results. Neurological deficits were identified in 113 (18.46%) children, while spinal dysraphism disorders, tethered cord syndrome and associated orthopedic malformations were found in 171 (27.94%), 119 (19.45%) and 28 (4.57%) patients, respectively. The incidence of tethered cord with lumbosacral dimple lesions over the sacrum (32/119, 26.65% of patients) was 3.5-fold higher than that of lesions found over the coccyx (9/119, 7.5% of patients). Uroneurological symptoms were found in 207/612 (33.82%) children. Urodynamic assessment revealed decreased bladder capacity in 10% of patients, detrusor hyperflexia during filling in 47% and a low-compliance detrusor in 71%. Discordance between ultra- sonography and MRI was found in 16.58% of patients. Spinal cord untethering was performed for 109 patients. Nearly all children with resolution were at the end of their follow-up period (24 months). Retethering occurred in 21 (19.26%) patients, and a second untethering surgery was performed in 12 patients. When spina bifida was associated with lumbar skin lesions, there may have been an increased incidence of tethered cord and other spinal cord disorders. MRI scans are more reliable and give an exact diagnosis of tethered cord. Neurological and uroneurological instability are ultimately a clinical diagnosis, and there is controversy about their indications for surgery. However, the correlation between urodynamic assessment and cutaneous lesions with a tethered cord found by MRI examination allow for an early diagnosis and the possibility of prompt treatment.

Intraspinal lesions associated with sacrococcygeal dimples

“…Supporting this view, Sasani et al used ultrasound, MRI, or both to examine 264 subjects with sacral dimples (Table 3) and found 41 cases (15.5%) of tethered cord, including 21 with thickened and fatty terminal fila, 16 with dermal sinuses, 3 with diastematomyelia, and 1 with lipomyelomeningocele. 16 There was a discordance of 16.6% between ultrasound and MRI, with ultrasound often failing to detect abnormalities. Thus, the authors concluded that MRI is preferable for evaluating spinal disorders in neonates.…”

“…10,16 In clinical practice, however, it is important to distinguish between dermal sinus tracts and sacrococcygeal dimples, as they are treated differently. The dural sac ends at S-2, 8 and sacrococcygeal dimples do not extend into the intradural space.…”

Towards Guideline-Based Management of Tethered Cord Syndrome in Spina Bifida: A Global Health Paradigm Shift in the Era of Prenatal Surgery

“…[39][40][41] Among surgical studies (n = 12), there was no clear or standardized description in the untethering technique utilized. [30][31][32][33][34]37,38,[42][43][44] Among these surgical studies, only 6 described their complications in the results section of the respective article. Additionally, 5 out of the 13 studies did not provide information on the length of follow-up after surgical intervention.…”

“…In our systematic review, we observed differences in the diagnostic criteria of TCS and widely variable health outcomes following surgery in Asian countries. 31,[38][39][40] Our findings suggest that among studies conducted in this region, the management of TCS was aggressive in the surgical approach leading to untethering and often did not rely on the pres-ence of progressive neurologic deficits as a criterion for initial surgical treatment of spinal cord tethering. 30,33,34,42 To address the observable variation in the diagnostic criteria for TCS, Lew and Kothbauer 26 defined TCS as "a diverse clinical entity which presents with symptoms and signs resulting from abnormal spinal cord tension. "…”

“…The presence of neurologic symptoms was not a prerequisite for untethering in the study by Shahjouei et al 37 Lastly, only 3 studies used urodynamic testing as part of their patients' examination. 31,35,38…”