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Fulltext Outcome of bilateral hip reconstruction in unilateral hip subluxation in cerebral palsy: Comparison to unilateral hip reconstruction

Kamisan N, Thamkunanon V

J Orthop, 2020 07 08;20:367-373.
PMID: 32699490 DOI: 10.1016/j.jor.2020.06.017

Objective: To evaluate the post-operative outcomes of the hips in CP patients with unilateral hip subluxation treated with bilateral and unilateral hip reconstruction.
Methods: A retrospective review was performed of all diplegic and quadriplegic patients with unilateral hip displacement treated with either bilateral or unilateral hip reconstructive surgery. Radiographic parameters [migration percentage (MP), pelvic obliquity angle (POA) and migration percentage difference (MPD)] and changes in functional ability (sitting, standing and walking) were evaluated and compared between the 2 groups. Failure was defined as post-operative MP>40%, POA>5° and MPD>30%.
Results: Eighteen patients had unilateral hip reconstruction and 42 patients had bilateral hip reconstruction. Mean age of 87 months and 90 months and means follow-up of 38 months and 40 months respectively. Post-operative MP was significant in both groups. However, of 18 patients in unilateral hip reconstruction group, 33.3% of patients had contralateral hip subluxation and 22.2% of patients had hip failure on the operated hip; compared to only one of 42 patients had hip failure and no contralateral hip problem in the other group. For assessment of pelvic symmetry, MPD was significantly improved in both group but POA was only significant in bilateral group. Overall functional improvement was significant in patients with bilateral hip reconstruction compared to unilateral group.
Conclusion: Bilateral hip reconstruction in unilateral displacement had shown to have better outcome in correcting unstable hip and pelvic asymmetry, thus provide good sitting balance and improvement in overall functional outcome.
Fulltext Approach to bone procedure in fixed equinovarus deformity in cerebral palsy

Thamkunanon V, Kamisan N

J Orthop, 2018 Dec;15(4):1008-1012.
PMID: 30302034 DOI: 10.1016/j.jor.2018.09.001

Fixed equinovarus deformity in cerebral palsy requires bone procedures for surgical correction. We reported the outcome of surgical procedure establishing the approach to multiple osteotomy and fusion to this problem. Retrospectively, 55 feet were reviewed. Step of surgical evaluation were applied to all patients by addressing each component of equinovarus deformity. 31 feet achieved correction by multiple osteotomy. Overall 78% had good outcome and maintained plantigrade foot. This study had outlined a simple surgical step-approached procedure to address fixed equinovarus deformity in cerebral palsy with high success rates. We recommended multiple bone osteotomy to preserve joint motion.
Fulltext Overcoming the barriers to diagnosis of Morquio A syndrome

Bhattacharya K, Balasubramaniam S, Choy YS, Fietz M, Fu A, Jin DK, et al.

Orphanet J Rare Dis, 2014;9:192.
PMID: 25433535 DOI: 10.1186/s13023-014-0192-7

Morquio A syndrome is an autosomal recessive lysosomal storage disease often resulting in life-threatening complications. Early recognition and proficient diagnosis is imperative to facilitate prompt treatment and prevention of clinical complications.
Identifying the need for a multidisciplinary approach for early recognition of mucopolysaccharidosis VI (MPS VI)

Choy YS, Bhattacharya K, Balasubramaniam S, Fietz M, Fu A, Inwood A, et al.

Mol Genet Metab, 2015 May;115(1):41-7.
PMID: 25892708 DOI: 10.1016/j.ymgme.2015.03.005

Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is caused by deficient activity of the enzyme, N-acetylgalactosamine-4-sulfatase, resulting in impaired degradation of the glycosaminoglycan dermatan sulfate. Patients experience a range of manifestations including joint contractures, short stature, dysostosis multiplex, coarse facial features, decreased pulmonary function, cardiac abnormalities, corneal clouding and shortened life span. Recently, clinicians from institutions in the Asia-Pacific region met to discuss the occurrence and implications of delayed diagnosis and misdiagnosis of MPS VI in the patients they have managed. Eighteen patients (44% female) were diagnosed. The most common sign presented by the patients was bone deformities in 11 patients (65%). Delays to diagnosis occurred due to the lack of or distance to diagnostic facilities for four patients (31%), alternative diagnoses for two patients (15%), and misleading symptoms experienced by two patients (15%). Several patients experienced manifestations that were subtler than would be expected and were subsequently overlooked. Several cases highlighted the unique challenges associated with diagnosing MPS VI from the perspective of different specialties and provide insights into how these patients initially present, which may help to elucidate strategies to improve the diagnosis of MPS VI.