Distal Femoral Extension Osteotomy and Patellar Tendon Advancement in Management of Fixed Knee Flexion Deformity and Crouch Gait in Cerebral Palsy

Background Cerebral palsy (CP) is generally associated with musculoskeletal deformities that occur during body growth. Fixed knee flexion deformity (FKFD) results from long standing knee flexion that is due to progressive contracture of spastic hamstrings combined with a quadriceps weakness. Fixed knee flexion compromises the passive mechanisms of joint stabilization at mid and terminal stance in children with CP. The aim of this study is to highlight the results of distal femoral extension osteotomy and patellar tendon advancement (DFEO + PTA) for management of FKFD in crouching CP patients with spastic or mixed tone diplegic ambulatory cerebral palsy children. Patients and Methods A prospective study was conducted involving twenty CP patients with fixed knee flexion deformity (14 males and 6 females). The 20 cases with fixed knee flexion deformity were GMFCS level II (4 cases), and III (16 cases). All patients were clinically and radiologically assessed according to knee flexion angle, extension lag, koshino index and the Gross motor function measure pre- and post-operative. All patients underwent DFEO ± PTA. Eighteen limbs had DFEO on the RT side, and twenty on the LT side. Results The mean age of the patients was 11.18±3.23 (6-16) years. The period of follow up ranged from 12 to 24 months (The mean follow up was 16.20 ± 2.46). At last follow up, the median knee flexion angle improved from 25 to 0. The median extension lag improved from 17.5 to 0. The mean koshino index improved from 1.59 ± 0.32 to 1.05 ± 0.10. the GMFM improved from 52.86 ± 7.36 to 68.15 ± 5. 82. Conclusion The combined procedure (DFEO + PTA) is effective in increasing knee extension in the stance phase, reducing knee pain and improving knee extension strength.

Undiagnosed Case of Klippel-Trenaunnay Syndrome Presenting as Extensive Heterotrophic Ossification and Flexion Deformity of Right Lower Limb Requiring Amputation : A Case Report

Klippel-Trenaunnay Syndrome is a rare disease characterized by a clinical triad of capillary malformation, soft tissue and bony hypertrophy, and atypical varicosity. This syndrome ranges from asymptomatic disease to life-threatening bleeding, embolism, and deformities. Management includes early diagnosis, prevention, and treatment of complications. We present a case of a 43-year-old male presenting with pain, swelling and deformity of the right leg for 30 years. On examination, diffusely enlarged tender right limb with several dark patchy discolorations, multiple tortuous vessels were found. Right leg X-ray showed heterotrophic ossification and distortion of ankle joint. Due to chronic severe pain, recurrent infection, contracture and flexion deformity of right leg, the patient underwent above knee amputation. This case focuses on the variable presentation and multiple problems faced by patients with Klippel-Trenaununay Syndrome as they get diagnosed late and shows the importance of high index of suspicion for early diagnosis and prevention of complications.

Growth modulation with tension-band plates for the correction of paediatric lower limb angular deformity: current concepts and indications for a rational use

Growth modulation (GM) with tension-band plates (TBPs) by tethering part of the growth plate is an established technique for the correction of angular deformities in children, and it has increasingly supplanted more invasive osteotomies. Growth modulation with TBPs is a safe and effective method to correct a variety of deformities in skeletally immature patients with idiopathic and pathological physes. The most common indication is a persistent deformity in the coronal plane of the knee exceeding 10°, with anterior and/or lateral joint pain, patellofemoral instability, gait disturbance, or cosmetic concerns. GM has also shown good results in patients with fixed flexion deformity of the knee and ankle valgus. This paper reviews the history of the procedure, current indications, and recent advances underlying physeal manipulation with TBPs. Cite this article: EFORT Open Rev 2021;6:658-668. DOI: 10.1302/2058-5241.6.200098

Characteristics of Congenital Clasped Thumb: A Case Report and Literature Review

Congenital clasped thumb is a progressive flexion and adduction deformity presenting with heterogeneous congenital abnormalities and syndromes. This deformity is usually accompanied by first web space narrowing and metacarpophalangeal joint (MPJ) laxity. Understanding the various features of the clasped thumb and making an accurate diagnosis is essential for treatment. Depending on the classification, treatment can vary from conservative to surgical. We describe the case of a bilateral clasped thumb with various characteristics, which were treated differently according to the disease type. The deformity of the clasped thumb was bilateral, and the patient had MPJ flexion deformity, flexor pollicis longus shortening, first web space narrowing, and MPJ instability, which were confirmed through a stress test. The left thumb was a complex type and was surgically treated, whereas, the right thumb was a flexible type, which was treated with splinting; the treatment showed promising results at 2 years post surgery. Diagnosis of the clasped thumb through thorough history taking, physical examination and considering its characteristics, and appropriate classification of the disease is essential for treatment. Furthermore, a stress test can identify MPJ instability in the congenital clasped thumb.

Subcutaneous Calcification and Fixed Flexion Deformity of the Right Elbow Joint in a Child with a GNAS Mutation, the First Case Report

Introduction: The art of medicine glorifies when a clinician listens carefully to the patient’s story, gives a thorough examination, performs appropriate investigations, and finally links findings together to reach a definite diagnosis. An interesting case was reported here, highlighting the integration of different symptoms and manifestations with some relevant biochemical investigations to reach a final diagnosis. To the best of our knowledge, fixed flexion deformity, as a complication of subcutaneous calcification, has not been previously reported in a child with Albright hereditary osteodystrophy (AHO). Case Presentation: A 2.5-year-old boy was born at term with a birth weight of 3.5 kg (-0.49 SDS). The child was referred to a general pediatrician with a history of right elbow joint swelling noticed initially at six months of age. He then developed the limitation of right upper arm movement, which slowly progressed afterward. The patient had no history of trauma. At nine months of age, he was diagnosed with hypothyroidism, preceded by cold skin, dry hair, and constipation. At nine years of age, he presented with a fixed flexion deformity of the right elbow, associated with markedly limited joint movement and symmetrical hands with hyperpigmented knuckles of right metacarpal bones. Subcutaneous masses were felt along the right forearm, showing tenderness on palpation. Investigations revealed elevated serum parathyroid hormone and normal calcium, indicating parathyroid hormone resistance. Further genetic testing revealed GNAS mutation. The child was obese throughout his childhood. Conclusions: This case report describes an obese child with subcutaneous calcification that led to fixed flexion deformity of the elbow, starting at an incredibly early age. Hypothyroidism and pseudohypoparathyroidism raised the suspicion of AHO, which was later confirmed by genetic testing. This is the first case report on fixed flexion deformity in a patient with GNAS mutation (c.719-1G > A Chr20: 57484737) in West Asia.

Functional and Clinical Outcome With Modified Lateral Approach Total Hip Arthroplasty in Stiff Hips With Ankylosing Spondylitis

Background Ankylosing spondylitis at THA have significant hip stiffness with flexion deformity and restricted function. ROM (range of movement) improvement with good functional outcome is seen following THA with modified lateral approach in these hips.Methods 69hips who underwent THA with a modified Hardinge approach in 40patients were evaluated at a mean follow up of 38.33 months (0 to 83 months). Modified Harris hip score, ROM (range of movement), and SF 36 scores were used to assess clinical and functional improvement at review. Results 69 hips with significant decrease in ROM preoperatively with 32 clinically fused hips showed significant improvement in the range of flexion. The mean flexion in 69 hips improved from 29.35 (SD 31.38) to 102.17 degrees (SD10.48). The mean difference of 72.82 with p Value <0.0001 was found to be significant. 45 /69 hips had fixed flexion deformity with 13 hips having a deformity above 30 degrees. The flexion at review was below 90 degrees in 3 hips. Eleven hips had flexion of 90 degrees at review while the remaining 55 hips had flexion above 100 degrees. Modified HHS improved from 17.03(SD 6.02) to 90.66 (SD7.23). SF 36 at review indicated health status in 40 patients as excellent in 11, very good in 20, fair in 3 and poor in 1. Mean mental health score was 84.10(SD11.58)Conclusion Our study suggests that modified lateral approach THA in AS with stiff hips achieves significant improvement in ROM, HHS and quality of life indicated by SF12 and SF36 scores. Modified lateral approach is useful especially in fused AS hips with flexion deformity.