Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case.

Ellis-van Creveld (EVC) syndrome is a rare inherited condition with inheritance, which is autosomal recessive in nature and is also described as skeletal dysplasia (chondroectodermal). The patients present with a grave genu valgum deformity which is a major challenge in orthopedics. The current case report presents a young girl of the juvenile age group who came with deformity over the bilateral lower limb with difficulty in walking and bilateral upper limb polydactyly. The patient underwent relevant investigations and examinations which were suggestive of bilateral genu valgum deformity. Since the deformity was significant, a corrective osteotomy with soft tissue release was planned followed by rehabilitative physiotherapy. Considering the extensive nature of the surgery, a staged procedure was planned. The patient on follow-up presented with a good range of motion and improved gait. Various treatment modalities have been described for the correction of the deformity but few of them are done in patients with EVC syndrome and they state varying results and high incidences of relapse. The present study focuses on corrective osteotomy with soft tissue release as a treatment modality and describes the outcome associated with the modality. Thus, stating that with proper planning and postoperative rehabilitation the patient can achieve a good functional outcome.

Extra-articular distal tibial fractures, is interlocking nailing an option? A prospective study of 147 cases.

PURPOSE: Distal tibia fractures comprise about 7%-10% of lower extremity trauma. Because of the peculiarity of the soft tissue and subcutaneous location of the bone there are many controversies in the ideal treatment of distal tibia fractures especially extra articular pilon fractures. Plating is fraught with complications of wound dehiscence and infection. There are limited studies which document outcomes in such cases using intramedullary interlocking nail. We intend to study the outcome and complications of extra articular distal tibial fractures treated with interlocking nailing. METHODS: This is a prospective study conducted in a tertiary care orthopaedic hospital in southern India. There are 147 patients of distal tibia extra-articular fractures managed by IM nailing with follow up of more than one year were included in this study. Only cases with fresh injury (less than 1 week), fracture below the isthmus, closed and open Gustilo Anderson type 1 and 2 fractures were included in the study. Patients were reviewed at 3, 6, 12 and 24 weeks after surgery and thereafter at one year and were assessed for clinical and radiological signs of healing, any complications, time to union and functional outcome. RESULTS: There were 102 males and 45 females (male/female ratio is 2.3:1) with a mean age of 38.96 (range 23-65) years. According to AO classification, there were 78 cases (53.06%) of 43-A1, 39 cases (26.53%) of 43-A2 and 30 cases of 43-A3 constituting 20.40%. The fracture united in all the patients at an average of 18 weeks (range 16-22 weeks), none of the patient in our series had a delayed or non-union. Two patients (1.47%) had the fracture united in mild valgus but it was well within the acceptable limits (<5°). The functional outcome was assessed in all the patients at final follow up using Olerud and Molander score all the patients fared an excellent to good score, there were no cases with poor score. CONCLUSION: Intramedullary nailing is a viable option to treat distal tibial fractures with excellent outcome. Wound complications related to plating can be avoided but meticulous surgical technique is key to avoid malunion.

Rare Case of Extra-articular Synovial Chondromatosis of Biceps Tendon Sheath in 8 years Male Child.

INTRODUCTION: Primary synovial osteo chondromatosis (PSOC) is chondroid metaplasia with multi¬nodular proliferation of the synovial lining of a diarthrodial joint, bursa, or tendon sheath. It usually occurs in third-fifth decade and shoulder joint involvement is infrequent. It is very rare in children and primary extraarticular PSOC of the shoulder has been reported very rarely in children. CASE REPORT: We present a case of primary PSOC of the long head of biceps in 8year child. It presented as painful swelling in proximal arm. The pain radiograph was showing multiple calcified loose bodies on anteromedial aspect of humerus. MRI scan showed fluid filled cysts with calcified wall. The lesion was excised through deltopectoral approach. He had complete resolution of symptoms without recurrence at 1 year.The diagnosis was confirmed on histopathological examination. CONCLUSION: We want to emphasize that one should keep a differential diagnosis of this rare condition in patients presenting with cystic swelling with calcified wall. We further emphasize the need to follow these patients to detect recurrence or malignant transformation.

Glomus tumor of thumb occurring at unusual location.

Glomus tumour are painful swelling arising from glomus bodies. They are very rare in occurrence. Most of the time the presenting symptoms is severely painful fingertip without palpable swelling. Their most common location is the subungual region of digits. We report the rare case of a glomus tumour located in an uncommon location, i.e., at the tip of right thumb medial aspect near to tendon attachment. The peculiarity of this lesion is that this lesion on magnetic resonance imaging was found to arise from the insertion of flexure pollicis longus tendon and was reported as synovial swelling arising from tendon sheath. Clinical findings were consistent with glomus tumour. Excision biopsy confirmed diagnosis of glomus tumour. Although rare, glomus tumour should be considered as differential diagnosis for fingertip pain and excision of lesion gives complete pain relief.

Hip dislocation during lengthening of congenital short femur.

Hip dislocation is a serious but rarely reported complication of femoral lengthening in congenital short femora. We report a retrospective series of three hip dislocations that occurred during lengthening, analyze the factors contributing to the dislocation, and discuss the treatment of this difficult problem. The average lengthening achieved was 9 cm. We found progressive acetabular dysplasia and decreasing center edge angle. Closed reduction failed. Treatment included soft tissue release, open reduction with femoral shortening, and acetabular procedures. At a mean follow-up of 4.4 years, two patients had a good modified Mckay score and a modified Severin score of 3. Excessive lengthening should be avoided.