Surgical strategies in management of ankylosed bilateral hips and knees in crouched posture.

A 26-year-old wheelchair-bound man was brought to our institute with progressive painful deformity of both lower limbs involving bilateral hip and knee for the past 5 years. The patient was wheelchair-bound for the past 18 months. He was only able to crawl to meet his locomotor demands. On examination, the patient was in a crouched posture with mild kyphosis at the thoracolumbar region and ankylosis of bilateral hip and knee in an unfavourable position. Radiological and serological evaluation diagnosed him to be a case of neglected ankylosing spondylosis. We planned for arthroplasty of the bilateral hip and knee for correcting his posture and to regain his locomotor ability. The severity of the deformity mandated extensive releases and the handling of neurovascular structures. Hence, a holistic treatment plan with various surgical strategies was devised to manage his deformities. The patient made a good recovery and regained his locomotor ability without any major complications at a 2-year follow-up.

Turning the backbone into an ankylosed concrete-like structure: Case report.

RATIONALE: Progressive restriction of the spinal bio-mechanics is not-uncommon deformity encountered in spine clinics. Congenital spinal fusion as seen in Klippel-Feil-anomaly, progressive non-infectious anterior vertebral fusion, and progressive spinal hyperostosis secondary to ossification of the anterior longitudinal spinal ligament are well delineated and recognized. PATIENT CONCERNS: A 24-year-old girl has history of osteoporosis since her early childhood, associated with multiple axial and appendicular fractures and scoliosis. Recently she presented with episodes of severe back pain, spinal rigidity/stiffness with total loss of spine biomechanics. DIAGNOSES: She was provisionally diagnosed as having osteogenesis imperfecta and was investigated for COL1A1/A2 mutations which have been proven to be negative. Autosomal recessive type of osteogenesis imperfecta was proposed as well, no mutations have been encountered. A homozygous for CTSA gene mutation, the gene associated with Galactosialidosis was identified via whole exome sequencing (Next-Generation Sequencing projects) has been identified. INTERVENTIONS: Early in her life she had a history of frequent fractures of the long bones since she was 4 years which was followed by vertebral fractures at the age of 12 years. She manifested lower serum 25OH-D levels and were associated with lower LS-aBMD Z-scores with higher urinary bone turnover indexes (urinary NTX/Cr). OUTCOMES: Lysosomal storage diseases (LSD) have a strong correlation with the development of osteoporosis. LSD causes skeletal abnormalities results from a lack of skeletal remodeling and ossification abnormalities owing to abnormal deposition of GAGs (impaired degradation of glycosaminoglycans ) in bone and cartilage. 3D reconstruction CT scan of the spine showed diffuse hyperostosis of almost the entire spine (begins at the level of T4- extending downwards to involve the whole thoraco-lumbar and upper part of the sacrum) with total diffuse fusion of the pedicles, the transverse and articular processes, the laminae and the spinous processes. LESSONS: This is the first clinical report of adult patient with a history of osteoporosis and fractures with the late diagnosis of Galactosialidosis. Osteogenesis imperfecta (autosomal dominant and recessive) were the first given diagnoses which proven negative. The pathophysiology of the spine ankylosis in our current patient and its correlation with LSD, antiresorptive medications, vitamin D3 and supplemental calcium is not fully understood. Therefore, further studies are needed to elucidate this sort of correlation.

Intraoperative mandibular nerve block with peripheral nerve stimulator for temporomandibular joint ankylosis.

We describe the use of peripheral nerve stimulator for mandibular nerve block intraoperatively in a 4.5year old child with complete temporomandibular joint ankyloses. The block was not possible preoperatively, therefore, it was administered after release of ankyloses. The use of peripheral nerve stimulator increased the chances of a successful block. No intraoperative analgesics and muscle relaxants were required. Postoperative pain relief was excellent. Peripheral nerve stimulator is an easy way of for accurate needle tip placement for mandibular nerve block in patients with distorted anatomy.

What Is the Efficacy of Repeat Manipulations Under Anesthesia to Treat Stiffness Following Primary Total Knee Arthroplasty?

BACKGROUND: Manipulation under anesthesia (MUA) can help post-total knee arthroplasty (TKA) patients who have knee stiffness regain range-of-motion. However, despite undergoing MUA, patients may have persistent knee stiffness. Often, this persistent knee stiffness is treated with a repeat MUA. Therefore, the purpose of this study was to evaluate repeat MUAs by assessing: (1) demographic characteristics, (2) range-of-motion, (3) clinical outcomes, and (4) rate of revision surgery in post-TKA patients with persistent knee stiffness who either underwent a single MUA or repeat MUAs. MATERIALS AND METHODS: One-hundred-and-sixty-seven post-TKA who had undergone an MUA between 2005 and 2011 at two institutions were reviewed. Patients were stratified into those who had a single-MUA (138 knees) and those who had a repeat MUA (29 knees). The mean follow-up period was 63 months (range, 36 to 90 months). The incidence of repeat MUA within this cohort was determined. Demographics and ROM were compared using Student t-test and Chi-square as appropriate. Functional outcomes were assessed using Knee Society scores (KSS) and compared between the two cohorts. RESULTS: Among the 167 patients who underwent a MUA, 29 (17%) required repeat manipulations. The repeat MUA cohort was younger and more likely to have osteonecrosis as the underlying cause of knee disease. For the repeat MUA cohort, 17 patients (59%) had achieved satisfactory mean gains in ROM after their repeat MUAs. These patients had also achieved excellent mean Knee Society objective and functional scores. However, another seven knees (24%) had further persistent knee stiffness requiring arthrolysis of adhesions and five patients (17%) had undergone revision of the polyethylene spacer or patellar component to improve range-of-motion. CONCLUSION: In this study, the majority of patients who had undergone a repeat MUA were able to achieve improvements in flexion range-of-motion and functional outcomes. However, the remaining patients required more invasive procedure to treat persistent knee stiffness. In patients who have persistent knee stiffness after MUAs, a repeat MUA may be helpful to increase range-of-motion and function.