Short-term growth hormone treatment in children with Hurler syndrome after hematopoietic cell transplantation.

Children with Hurler syndrome experience progressive growth failure after hematopoietic cell transplantation (HCT). The goal of this study was to review the safety and efficacy of growth hormone (GH) in eight children with Hurler syndrome who were treated at our institution with GH for short stature or GH deficiency between 2005 and 2008. The age at initiation of treatment with GH was 9.6+/-2.3 years and time since HCT was 7.5+/-1.5 years. Mean GH dose was 0.32 mg/kg/week. Baseline growth velocity was 3.5+/-1.5 cm/year (-2.6+/-1.9 s.d.), and it increased to 5.2+/-3.0 cm/year (-0.1+/-3.6 s.d.) after 1 year of treatment. Of the six patients with radiographic data, there was one progression of scoliosis, one progression of kyphosis and one progression of genu valgum. No patient discontinued treatment due to progression of skeletal disease. One patient discontinued GH due to slipped capital femoral epiphysis. Preliminary data suggest that 1-year GH treatment may modestly improve growth velocity in children with Hurler syndrome.

Postoperative deep wound infections in adults after spinal fusion: management with vacuum-assisted wound closure.

OBJECTIVE: Vacuum-assisted wound closure (VAC) exposes the wound bed to negative pressure, resulting in removal of edema fluid, improvement of blood supply, and stimulation of cellular proliferation of reparative granulation tissue. It has been used to treat open wounds in the extremities, open sternal wounds, pressure ulcers, and abdominal wall wounds. This study retrospectively reviewed instrumented spine fusions complicated by surgical wound infection and managed by a protocol including the use of VAC in order to evaluate the efficacy of applying vacuum therapy on patients with deep spine infections and exposed instrumentation. METHODS: Twenty consecutive patients with deep wound infections after undergoing spinal fusion procedures were studied. There were 12 men and 8 women with an average age of 55 years (31-81 years). Eight patients had undergone concomitant anterior and posterior arthrodesis, nine patients had a posterior spinal fusion, and three patients had a transforaminal lumbar interbody fusion. Seven patients had a decompression with exposed dura. Sixteen patients presented with a draining wound within the first 6 weeks postoperatively (average 24 days). There were four patients who presented with back pain and temperature after 1 year postoperatively (average 3 years). All patients were taken to the operating room for irrigation and debridement followed by placement of the VAC with subsequent delayed closure of the wound. RESULTS: There was an average of 1.8 (1-8) irrigation and debridement procedures prior to placement of the VAC. Once the VAC was initiated, there was an average of 2.2 (2-3) procedures until and including closure of the wound. The wound was closed an average of 7 days (5-14 days) after the placement of the initial VAC in the wound. All patients tolerated the VAC without adverse effects. All patients were kept on a 6-week course of intravenous antibiotic therapy. The average follow-up was 10 months (6-24 months). There were no cases of uncontrolled sepsis once the VAC was initiated. All patients achieved a clean closed wound without removal of instrumentation at a minimum follow-up of 6 months. CONCLUSION: VAC therapy is an effective adjunct in closing complex deep spinal wounds with exposed instrumentation.

Coronal plane imbalance in adolescent idiopathic scoliosis with left lumbar curves exceeding 40 degrees: the role of the lumbosacral hemicurve.

STUDY DESIGN: Retrospective radiographic analysis of the potential role the lumbosacral hemicurve has on adolescent idiopathic scoliosis coronal trunk imbalance. OBJECTIVE: To determine if the lumbosacral hemicurve predisposes adolescent idiopathic scoliosis to coronal decompensation preoperatively and postoperatively. SUMMARY OF BACKGROUND DATA: Although coronal decompensation remains a clinical problem in adolescent idiopathic scoliosis, the literature regarding the role of potential intrinsic structural properties of the lumbosacral hemicurve is sparse. METHODS: Fifty patients with adolescent idiopathic scoliosis were used to measure several potential parameters predisposing to coronal decompensation including lumbosacral hemicurve magnitude and flexibility, sacral and iliac obliquity. RESULTS: Overall, 84% (42/50) demonstrated preoperative decompensation. A more rigid lumbosacral hemicurve was significantly related to preoperative coronal decompensation in the combined and the King I groups. Preoperatively, significant correlation with decompensation was observed for sacral and iliac obliquity in the King I group and for iliac obliquity in the combined group. Postoperatively, coronal decompensation remained significantly correlated to sacral obliquity in the combined group and King I groups. Iliac obliquity was significantly related to postoperative decompensation in the combined group. CONCLUSIONS: The lumbosacral hemicurve represents an important structure predisposing to left coronal plane imbalance in adolescent idiopathic scoliosis that includes a large left lumbar curve as a component of the curve pattern. "At-risk" signs for persistent postoperative coronal decompensation include iliac and sacral obliquity noted on the preoperative standing full-length radiographs.