Asif Nazir Baba

Ellis-van Creveld syndrome with facial hemiatrophy

Ellis-van Creveld (EVC) syndrome is a rare autosomal recessive congenital disorder characterized by chondrodysplasia and polydactyly, ectodermal dysplasia and congenital defects of the heart. We present here a case of a 16-year-old short-limbed dwarf with skeletal deformities and bilateral postaxial polydactyly, dysplastic nails and teeth, also having left-sided facial hemiatrophy. The diagnosis of EVC syndrome was made on the basis of clinical and radiological features. To the best of our knowledge, this is the first report of EVC syndrome with facial hemiatrophy in the medical literature from India.

Gorham's disease of femur

Gorhams disease is a rare condition of bones characterized by spontaneous massive and progressive osteolysis. Less than 200 cases have been reported so far. Femur is an uncommon site of Gorham disease. We report a young female, presenting as pathological fracture of the femur, wherein rapid osteolysis of femur occurred. The clinical, pathological, and radiological picture suggested a diagnosis of Gorhams disease. The patient was subjected to radiotherapy (40 Gys) and put on bisphosphonates. At a follow-up of 2 years, the disease process had stopped and partial recalcification of the bone had occurred.

Osteoblastoma of body of the talus—Report of a rare case with atypical radiological features

Osteoblastoma is a benign bone tumour found commonly in the spine and long tubular bones. Involvement of the talus is uncommon, and when present, is found in the neck of the talus. Osteoblastoma of the body of talus is a very rare entity. We report a young male, presenting as chronic ankle pain, with a radiolucent lesion with a thick periosteal shell in the body of the talus. Analysis of clinical, radiological and histological findings confirmed the diagnosis of osteoblastoma. The case is reported for the rarity of the site and atypical radiological features that osteoblastoma can present with.

Luxatio erecta: Inferior glenohumeral dislocation

Inferior dislocation of the shoulder, also called luxatio erecta, is a rare form of the otherwise common shoulder dislocation. It accounts for less than 0.5% of all shoulder dislocations. A case involving the inferior dislocation of the shoulder is reported. A brief review of the presentation and management of the condition is described.

Familial reactive perforating collagenosis

Background: Reactive perforating collagenosis (RPC) is one of the rare forms of transepidermal elimination in which genetically altered collagen is extruded from the epidermis. This disease usually starts in early childhood as asymptomatic umbilicated papules on extremities, and the lesions become more conspicuous with age. Aims: The objective of our study was to determine the clinico-pathological features of RPC and the response to various treatment modalities. Methods: Ten patients of RPC, belonging to five different families, were studied clinically. Various laboratory investigations were carried out and diagnosis was made by histopathology of the lesions. Patients were given various topical and oral treatments. Results: RPC is familial in most cases without any definite inheritance pattern. It begins in childhood and the lesions are usually recurrent and become profuse and large with age. Systemic diseases have no role in the onset of lesions. Conclusion: Oral and topical retinoids in combination with emollients is the best treatment option.

Mitchell's osteotomy in the management of hallux valgus: An Indian perspective.

Background: Hallux valgus is a common condition that affects the forefoot. A large number of procedures are described for managing this condition. Mitchells osteotomy and its modifications are being widely used for treating hallux valgus. However, most of the studies describe the results from the developed world. We present results of the classic Mitchells osteotomy in hallux valgus in Indian subcontinent. Materials and Methods: Forty eight adult patients (including 12 bilateral ones) in the age range of 18–60 years with hallux valgus were managed with the classic Mitchells osteotomy. Pain over the bunion was the reason for surgery in 53 of 60 feet and cosmesis in the remaining 7 feet. Patients with hallux valgus angle more than 20° and not responding to a trial of conservative treatment were included. Patients having metatarsophalangeal (MTP) joint osteoarthritis (Grade II and higher), hallux rigidus, rheumatoid arthritis, and with subluxation of MTP joint were excluded from the study. Further, patients with first metatarsal more than 3 mm shorter than second metatarsal were also excluded. Results: The average follow-up period is 3 yrs (range 18months – 6yrs). About 55 feet (83%) were painless after surgery. Forty-two (70%) patients were happy with the cosmetic results of the surgery. Metatarsalgia was the reason for dissatisfaction with the procedure in five patients. The average correction of hallux valgus and the intermetatarsal angles achieved was 19.7° and 6.9°, respectively. Using the Broughton and Winson scoring system, 37 (61.7%) feet had excellent results, 18 (30%) had good, and five (8.3%) feet had a poor results. Conclusion: The classic Mitchells procedure is a simple procedure and gives good cosmetic and radiological results.

Is Urgent Treatment of Pediatric Neck Femur Always Needed

Purpose: Pediatric hip fractures are very uncommon and the complications of these fractures are very serious. With this purpose in mind we evaluated retrospectively the treatment of pediatric femur neck fractures admitted during proceeding five years in our hospital. The Aim of study was to evaluate the outcome of our treatment and know preventable complications of pediatric neck fractures in comparison with literature. Method: We evaluated 19 pediatric femur neck fractures with age from 3-12 years including 12 males 7 females. Treatment was by closed reduction and internal fixation at average of 4.5 days after hospital admission. Spica cast was applied for all patients up to age 10. Clinical and radiological evaluation was done by RATLIFF CRITERIA Results: We enrolled 23 cases in study 4 lost to follow up and thus excluded from final study. We had 10 cases of Delbet type II, 7 cases type III and 2 cases of type IV. Patients were treated by closed reduction and internal fixation at average of 4.5 days after admission. Our results were 12 good, 3 fair and 4 poor. Complications encountered included avascular necrosis in 5 patients, 1 coxa vara, 2 cases of superficial infection and 1 septic sequel of sab acute septic arthritis. Conclusion: Fluoroscopic aided reduction and internal fixation is a very good method of treatment for pediatric neck femur fractures. Emphasis should be on measures to reduce avascular necrosis and infection and give maximum smile to the children and their parents. In our under eloped and far-flung area complications are unavoidable but preventable once infrastructure and facilities of emergency and postoperative care are improved. Education regarding prevention of pediatric fractures in general and neck femur in particular and active participation of parents will help in minimizing complication of these fracture thus limiting diability and morbidity of these fractures.