Sprengel deformity: morphometric assessment and surgical treatment by the modified green procedure.

BACKGROUND: We evaluated the pathologic anatomy of the Sprengel deformity using radiographs, 3-dimensional computed tomography (3D-CT), and magnetic resonance imaging (MRI), and reviewed our results of the modified Green procedure. METHODS: Between 2002 and 2009, 23 scapulae in 22 patients were treated. The average age at the time of surgery was 3.4 years (range, 1.9 to 7.1 y). The outcome of surgery was assessed on the basis of changes in shoulder abduction and the radiographic findings using Cavendish classification, Rigault classification, scapular elevation, and medialization. Preoperative appearance was classified as grade III in 13 cases and grade IV in 10 cases according to the Cavendish classification, and as grade II in 4 cases and grade III in 19 cases according to the Rigault classification. Using 3D-CT, we assessed the height to width ratio on the scapular posterior view, the superior displacement ratio and rotational difference on the trunk posterior view, and the anterior curvature of the supraspinous portion on the scapular medial view. RESULTS: The average follow-up postoperative period was 4.4 years (range, 2.2 to 8.7 y). Postoperatively, the shoulder abduction improved well, with a mean improvement of 63 degrees. Improvement of at least 1 Cavendish grade and 1 Rigault grade was attained in all cases. On preoperative 3D-CT, the mean height to width ratio of the affected scapula was significantly smaller than that of the contralateral scapula. There was an inverse relationship between the superior displacement ratio and the rotational difference. Anterior curvature of the supraspinous portion was seen in all cases. Omovertebral bone was found in 20 cases. MRI identified unossified omovertebral cartilage that was not evident by radiographs or CT. Transient brachial plexus palsy occurred in 2 patients, both of whom recovered within 4 months. Postoperative scapula winging and unsightly scarring were seen in 3 and 8 cases, respectively. CONCLUSIONS: 3D-CT and MRI were helpful in evaluating the pathologic anatomy of the deformity and in planning the surgery. The modified Green procedure provided successful functional and cosmetic results. LEVEL OF EVIDENCE: Level IV.

Foot centralization for tibial hemimelia.

Nineteen foot centralizations were performed in 14 patients with Jones type I and II tibial hemimelia. All feet showed equinovarus deformity and were treated by foot centralization by means of calcaneofibular arthrodesis. The average age of patients at the time of surgery was 1.3 years (range 0.4-3.8 years). The average follow-up postoperative period was 10.2 years (range 2.2-22.9). At the time of the final follow-up, four of the operated feet were plantigrade without secondary surgery. The remaining 15 limbs, however, required secondary surgery to treat postoperative early loss of correction and/or recurrent foot deformities such as equinus, varus and adduction, in addition to talipes calcaneal deformities, and fibular angular deformity at the fibular shortening osteotomy site. The deformities were treated either by repeat foot centralization, or fibular or calcaneal osteotomy. Careful observation for recurrence of the deformity is necessary until the distal fibular epiphysis closes, and the cartilagenous distal fibular end and calcaneus finally achieve ankyloses.

Limb salvage treatment for Gollop-Wolfgang complex (femoral bifurcation, complete tibial hemimelia, and hand ectrodactyly).

We reported the findings from three patients with Gollop-Wolfgang complex and demonstrated the results of five limb salvage treatments for this condition. All three femoral bifurcations were accompanied by ipsilateral complete tibial hemimelia. Two patients showed contralateral complete or partial tibial hemimelia, and one patient had hand ectrodactyly. The five limb salvage treatments included resection of the anteromedial bifurcated femur in three limbs, foot centralization in five limbs, tibiofibular fusion in one limb with partial tibial hemimelia, fibular transfer (Brown's procedure) in three limbs with complete tibial hemimelia, and callus distraction lengthening in one limb. The duration from the first operation to the final follow-up ranged from 3.5 to 5.4 years. None of the three knees treated by fibular transfer achieved a successful functional result, but all of the knees were ultimately able to withstand weight bearing. Early knee disarticulation and resection of the protruded bifurcated femur, followed by fitting of a modern prosthesis is likely to be the best treatment for patients with Gollop-Wolfgang syndrome. We note that limb salvage treatment is an alternative in patients who opt to retain their feet and refuse amputation.

Surgical treatment of hip dislocation in amyoplasia-type arthrogryposis.

We reviewed our clinical and radiographic results in the operative treatment of five unilateral and six bilateral hip dislocations in 11 patients with amyoplasia-type arthrogryposis. The mean age at surgery was 1.8 (range, 1.1-3.2) years. Femoral and/or pelvic osteotomies were combined with an open reduction. The mean follow-up period was 12.7 (range, 5.2-23.2) years. Nine patients were community walkers and two were household walkers. The range of motion and the St Clair hip score of the bilateral hips were not significantly different from those of the unilateral hips. Successful reduction increases the ambulation potential in both unilateral and bilateral hip dislocations.

Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency.

PURPOSE: Kabuki syndrome is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, postnatal growth deficiency and mental retardation. This report reviews our results in the operative treatment of hip dislocations in patients with Kabuki syndrome. METHODS: Between 2001 and 2009, seven dislocated hips (three unilateral and two bilateral hips) in five patients (all girls) were operatively treated at our institution. The operative treatment consists of open reduction, femoral derotation varus osteotomy, pelvic osteotomy (Salter in one and incomplete periacetabular osteotomy in six hips) and capsular plication. The age of the patients at the time of surgery ranged from 2.4 to 5.7 years, with an average of 3.6 years. RESULTS: The follow-up postoperative period ranged from 3.2 to 6.3 years, with an average of 5.0 years. At the final follow-up, all patients reported no click and no pain, and showed well-contained hips by radiographs. All seven hips were graded as Severin class I-II. One patient presented as having habitual dislocation of the hip 4.4 years after surgery. Computed tomographic (CT) scans revealed posterior acetabular wall deficiency, which was not corrected by the anterolaterally directed Salter osteotomy. The incomplete periacetabular osteotomy provided sufficient posterolateral coverage of the acetabulum. CONCLUSION: Operative treatment combining open reduction, femoral derotation varus and incomplete periacetabular osteotomies, and capsular plication provided successful results in patients with Kabuki syndrome who had the characteristics of hip instability such as ligamentous laxity, muscular hypotonia and posterior acetabular wall deficiency.