Cranio-vertebral pathology in Down syndrome

110

The ligamentous laxity associated with atlantoaxial subluxation has been assumed as a normal occurrence in 15–20% of Down’s syndrome patients. The occipitoatlantoaxial instability that accompanies this entity has been poorly recognized. The medical records and radiographic findings of 18 symptomatic patients with Down’s syndrome and cervicomedullary compromise were reviewed (1979–1991). All patients were evaluated prospectively under protocol for abnormalities of the cranial vertebral junction. There were 11 males and 7 females (age 3–42). ‘Fixed’ atlantoaxial luxation was seen in 8 (5 developed precipitous onset of cervical medullary compression). Occipitoatlantoaxial instability was present in 9 and associated rotary luxation in 9. The average predental space was 8 mm in the neutral position in 18 individuals. Two adolescents had previously undergone atlantoaxial dorsal fusion with subsequent progressive basilar invagination due to unrecognized occipitoat-lantal instability. An os odontoideum was seen in 3 patients. Irreducible invagination in 2 was treated with anterior decompression followed by dorsal occipital cervical fixation. The occipital cervical fixation was utilized in 10 individuals. Atlantoaxial dorsal fusion was made in 7 and 2 patients with acute rotary luxation of C1 and C2 were treated with immobilization. Halo immobilization in two individuals following a dorsal occipital cervical fixation produced an anterior fusion at the cranial vertebral complex indicating active vertebral ligamentous pathology. The review focuses on increased incidence of occiput cervical instability in the spectrum of craniovertebral junction abnormalities associated with Down’s syndrome. A large percentage has an odontoid ossicle probably as a result of repeated minor trauma. The results of surgical stabilization have been shown to be excellent.

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Craniovertebral Abnormalities in Down’s Syndrome

Menezes

Ryken²

1992

110

“…These patients have been shown to have an impaired immune system which may decrease their ability to mount an effective initial inflammatory host response to initiate bone graft incorporation [17][18][19][20]. They also have been found to have an inherent collagen defect that may impair union [17].…”

Section: Discussionmentioning

confidence: 99%

C2/3 Instability in a Child with Down’s Syndrome

Citow

Munshi²,

Chang-Stroman³

et al. 1998

Down’s syndrome patients are prone to cervical ligamentous laxity, the vast majority of which is at the C1/2 level. We describe the case of a 2-year-old girl with Down’s syndrome who was found to have cervical instability at the C2/3 level on screening cervical spine radiographs with 9 mm of anterolisthesis of C2 on C3. She was without clinically evident neurological deficit from this condition; however, T₂-weighted magnetic resonance imaging of her cervical spine revealed high intensity signal changes within the spinal cord at and above that level. She underwent posterior fusion that was complicated by poor tolerance of her Minerva-type cervical brace. She eventually developed a stable fusion with 5 mm of anterolisthesis at the C2/3 level. This is the only Down’s syndrome patient with instability at the C2/3 level that we have found reported. Our experience suggests that Down’s syndrome patients can have instability at C2/3 that can be successfully treated with posterior fusion.

“…AOS has been reported to occur in 6-63% of patients with Down's syndrome [2][3][4], However, the majority of cases reported have involved incidental findings in asymptomatic patients [4][5][6][7][8][9][10]. The 11 symptomatic patients represent 10% of the patients reported with AOS and Down's syndrome [10][11][12], All 11 patients have been over 1 year old. Presented here is the first reported case of AOS in a neonate with Down's syn drome.…”

Section: Introductionmentioning

confidence: 99%

Atlantooccipital Subluxation in a Neonate with Down’s Syndrome

Trumble

Myseros²,

Smoker³

et al. 1994

Atlantooccipital subluxation (AOS) occurs in up to 63% of patients with Down’s syndrome. However, presenting symptoms and the age at which pre-operative screening becomes necessary have not been clearly delineated. Recently, a 16-day-old female with Down’s syndrome and AOS presented to our institution. Following patent ductus arteriosus ligation, her neurological examination revealed trace movement, few spontaneous respirations, and left lower extremity clonus. Cervical spine radiographs revealed significant AOS. She was reduced and underwent a fusion from the occiput to C2. She gradually regained full strength and sensation. This case is the youngest example of AOS related to Down’s syndrome described. It emphasizes the need for preopera-tive screening of all patients with Down’s syndrome.