BACKGROUND: Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder. We sought to determine the incidence of scoliosis in patients with LDS, characterize the spectrum of spinal deformity, determine the results of bracing and surgery, and define surgical complications. METHODS: Patients were selected from our institution's database of 183 patients with LDS. Imaging measurements were performed for 141 patients whose records permitted spinal evaluation. Deformity changes and complications after intervention were recorded for patients who underwent bracing or surgery, and associations were tested using Student t tests (significance, P<0.05). RESULTS: Eighty-eight of 141 (62%) patients with LDS had scoliosis, with main thoracic and thoracolumbar curves being most common. Fifteen patients were braced (mean age, 9±3 y) for a mean of 2.3 years. They had a mean postbracing curve progression of 12±21 degrees (5±9 deg./y). There were no significant differences in age, sex, curve type, or prebracing curve magnitude between successfully braced (n=4) and unsuccessfully braced (n=11) patients (P>0.05). Nine patients, (mean age, 12±3 y), underwent 24 surgical procedures (16 growing rod procedures, 8 fusions). Mean curve corrections were 61% for growing rods and 73% for fusions. Associated blood loss for these procedures was 400 mL and 1293 mL, respectively, and normalized blood loss for fusion was 2.34 mL/kg/level. Fifteen of 24 surgical procedures involved complications (63%), including cerebrospinal fluid leaks (n=7) and blood loss >20% of estimated total blood volume (n=11). CONCLUSIONS: Scoliosis was present in 62% of our sample of LDS patients. Bracing did not halt curves in 11 of 15 patients, whose curves progressed >5 degrees or to >50 degrees by completion of bracing. At latest follow-up, 47% of the braced patients had undergone surgery after prior bracing attempts. The high blood loss associated with these operations is believed to be related to vascular fragility in patients with LDS. LEVEL OF EVIDENCE: Level IV-retrospective cohort study.
BACKGROUND:Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder. We sought to determine the incidence of scoliosis in patients with LDS, characterize the spectrum of spinal deformity, determine the results of bracing and surgery, and define surgical complications. METHODS:Patients were selected from our institution's database of 183 patients with LDS. Imaging measurements were performed for 141 patients whose records permitted spinal evaluation. Deformity changes and complications after intervention were recorded for patients who underwent bracing or surgery, and associations were tested using Student t tests (significance, P<0.05). RESULTS: Eighty-eight of 141 (62%) patients with LDS had scoliosis, with main thoracic and thoracolumbar curves being most common. Fifteen patients were braced (mean age, 9±3 y) for a mean of 2.3 years. They had a mean postbracing curve progression of 12±21 degrees (5±9 deg./y). There were no significant differences in age, sex, curve type, or prebracing curve magnitude between successfully braced (n=4) and unsuccessfully braced (n=11) patients (P>0.05). Nine patients, (mean age, 12±3 y), underwent 24 surgical procedures (16 growing rod procedures, 8 fusions). Mean curve corrections were 61% for growing rods and 73% for fusions. Associated blood loss for these procedures was 400 mL and 1293 mL, respectively, and normalized blood loss for fusion was 2.34 mL/kg/level. Fifteen of 24 surgical procedures involved complications (63%), including cerebrospinal fluid leaks (n=7) and blood loss >20% of estimated total blood volume (n=11). CONCLUSIONS:Scoliosis was present in 62% of our sample of LDS patients. Bracing did not halt curves in 11 of 15 patients, whose curves progressed >5 degrees or to >50 degrees by completion of bracing. At latest follow-up, 47% of the braced patients had undergone surgery after prior bracing attempts. The high blood loss associated with these operations is believed to be related to vascular fragility in patients with LDS. LEVEL OF EVIDENCE: Level IV-retrospective cohort study.
Authors: Francesco Baldo; Laura Morra; Agnese Feresin; Flavio Faletra; Yasmin Al Naber; Luigi Memo; Laura Travan Journal: Ital J Pediatr Date: 2022-06-06 Impact factor: 3.288