Surgery for spinal deformity in Parkinson's disease patients: What are we missing?

INTRODUCTION: Deformity associating coronal and sagittal malalignment can severely impair quality of life in Parkinson's disease (PD). Realignment using patient-specific rods (PSRs) is useful for achieving alignment goals. METHODS: This was a retrospective single-center analysis of a prospectively maintained database of all PD patients who underwent surgery between January 2013 and January 2017. Clinical evaluation, preoperatively and at 1 year's follow-up, used the Oswestry Disability Index (ODI). Radiological evaluation used systematic preoperative and 1-year postoperative full-spine radiographs. RESULTS: Twelve patients were included: 6 female, 6 male; mean age, 68.4 years. Mean follow-up was 40.8 months [range 12-70]. On average, 14 levels were fused [range 10-18]. Unplanned revision surgery was necessary for 8 patients at a mean 15.625 months after index surgery. Mean preoperative ODI score was 64% preoperatively [range 56-70] versus 52% [range 28-64] at 1 year's follow-up (P=0.004). Lumbar lordosis improved significantly, from -16.7° preoperatively to -41.4° at 1 year (P=0.006). Pelvic tilt was the least effectively corrected parameter, with a mean preoperative value of 31.6° vs. 27.8° at 1 year (P=0.19). Mean preoperative sagittal vertical axis was 149.7mm versus 73.6mm at 1 year (P=0.013). Mean preoperative coronal tilt was 68.2mm versus 22.9mm at 1 year (P=0.007). CONCLUSION: Parkinson's disease is a degenerative disease frequently associated with major spine malalignment. The severity of the postural disorders in these patients needs special precautions to avoid complications.

Multiple neurosurgical treatments for different members of the same family with Currarino syndrome.

INTRODUCTION: Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons. FAMILIAL CASE REPORT: Here, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome. CONCLUSION: In these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings).