Correction of Spine Deformity in Patients With Melnick-Needles Syndrome

Report of 2 Cases and Literature Review

Abstract

Background: 

Melnick-Needles syndrome (MNS) is a rare X-linked dominant disorder affecting the skeletal system and connective tissue. To date, <70 cases with MNS have been documented. Although few reports in the literature have shown that these patients might develop kyphoscoliosis, there is no report regarding long-term follow-up of patients with MNS undergo spine surgery. The aim of this study is to describe 2 unique cases of MNS with long-term follow-up after instrumented posterior spinal fusion, multiple-level Ponte osteotomies, and pedicle subtraction osteotomies.

Methods: 

A 17-year-old female patient (patient 1) was evaluated for the first time at our institution. She was diagnosed with MNS, severe kyphoscoliosis, and restrictive lung disease. After pulmonary function improvement, she underwent posterior spinal fusion. Halo-femoral traction with gradual weight increase was used a week before spinal surgery. Surgical correction of her spinal deformity was achieved through a hooks-and-wires construct. The second patient was an 18-year-old female (patient 2) who carried the diagnosis of MNS when she was first seen at our institution. She had a significant thoracolumbar junction kyphosis and gibbous. She also suffered from significant pulmonary disease with a stent in her right main stem bronchus. A posterior procedure consisting of multiple-level Ponte osteotomies and pedicle subtraction osteotomy at L2 was carried out.

Results: 

Eight- and 5-year follow-up of patients 1 and 2, respectively, demonstrated solid fusion, a well-maintained correction, and no evidence of implant breakage.

Conclusions: 

The reported cases add to the literature of excellent long-term results of spinal deformity correction with instrumented posterior spinal fusion in patients with MNS. Patients with this condition should be closely monitored from early childhood for the development of spine deformity so that early referral to a spine surgeon can be made followed by appropriate treatment, if necessary.