Acrodysplasia Scoliosis

Last update 01 Nov 2024

Basic Info

Synonyms

Acrodysplasia scoliosis, Acrodysplasia scoliosis (disorder), Brachydactyly, scoliosis, carpal fusion syndrome

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Introduction

A rare genetic dysostosis disorder with characteristics of brachydactyly and other finger/toe anomalies (short and/or wide metacarpals, abnormal or absent metatarsals, broad halluces), carpal synostosis, fused cervical vertebrae, scoliosis and spina bifida occulta. There have been no further descriptions in the literature since 1984.

100 Clinical Results associated with Acrodysplasia Scoliosis

100 Translational Medicine associated with Acrodysplasia Scoliosis

0 Patents (Medical) associated with Acrodysplasia Scoliosis

Literatures (Medical) associated with Acrodysplasia Scoliosis

25 Feb 2020·Zhongguo gu shang = China journal of orthopaedics and traumatology

[Halo-pelvic traction combined with stageⅡsurgical correction for the treatment of severe and rigid scoliosis].

Article

Author: Xue, Wen ; Liu, Lin ; Wang, Zhong-Hua ; Wang, Zeng-Ping ; Qian, Yao-Wen

OBJECTIVETo investigate the clinical effects of the Halo-pelvic traction combined with posterior instrumentation correction and internal fixation for the treatment of severe and rigid scoliosis.METHODSFrom January 2015 to 2019 May, 16 patients with severe and rigid scoliosis were treated by the Halo-pelvic traction combined with posterior instrumentation correction. There were 7 males and 9 females, aged 14 to 28 years with an average of 17.6 years, Cobb angle was 90° to 140° with an average of (108.84±17.93) °. Including congenital scoliosis in 10 cases (6 cases of dyssegmented scoliosis, 3 cases of dysplasia, 1 case of mixed scoliosis), idiopathic scoliosis in 4 cases and neurofibromatosis in 2 cases. All patients in group had scoliosis corrected by Halo-pelvic distraction combined with posterior instrumentation correction and internal fixation. Among them, 7 cases were treated with self-made guide device for iliac bone puncture, and the other cases with traditional bare hands. The height, scoliosis Cobb angle, kyphosis Cobb angle and correction rate of 16 patients before and after surgery were observed.RESULTSThe body height was corrected from (144.88±6.32) cm to (154.56±7.87) cm ; Cobb angle of scoliosis was corrected from (108.84±17.93) ° to (42.12±7.29) °, Cobb angle of kyphosis was corrected from (64.18±16.39) ° to (33.81±6.06) °. After the Halo pelvic traction, the body height increase, Cobb angle of scoliosis and kyphosis were changed with the mean of (7.06± 5.65) cm, (41.50±9.09)%, (30.52±9.66)%, respectively. After the stage Ⅱ surgical correction, the body height increase, Cobb angle of scoliosis and kyphosis were changed with the mean of (2.63±1.78) cm, (32.47±10.80)%, (15.55±9.47)% (The baseline Cobb angle referenced by the correction rate was the Cobb angle of the previous treatment). The body height increase and correction rate of Cobb angle of scoliosis and kyphosis with staged traction combined with surgery was (9.69±5.42) cm, (61.09 ± 5.03)%, (46.07±8.60)%. There was significant difference in body height increase, correction rate of Cobb angle of scoliosis and kyphosis between before and after each period of treatment (P<0.05).CONCLUSIONTreatment of severe and rigid scoliosis with Halo-pelvic traction combined with stage Ⅱ surgical correction can significantly correct deformities and improve appearance, and has fewer surgical complications and strong clinical operability.

01 Oct 2016·European Journal of Orthopaedic Surgery & Traumatology

Lumbar scoliosis associated with spinal stenosis in idiopathic and degenerative cases

Review

Author: Faundez, A ; Le Huec, J C ; Cogniet, A ; Mazas, S

Degenerative de novo scoliosis is commonly present in older adult patients. The degenerative process including disc bulging, facet arthritis, and ligamentum flavum hypertrophy contributes to the appearance of symptoms of spinal stenosis. Idiopathic scoliosis has also degenerative changes that can lead to spinal stenosis.PURPOSEThe aetiology, prevalence, biomechanics, classification, symptomatology, and treatment of idiopathic and degenerative lumbar scoliosis in association with spinal stenosis are reviewed.STUDY DESIGNReview study is based on a review of pertinent but non-exhaustive literature of the last 20 years in PubMed in English language.METHODSRetrospective analysis of studies focused on all parameters concerning scoliosis associated with stenosis.RESULTSVery few publications have focused specifically on idiopathic scoliosis and stenosis, and this was before the advent of modern segmental instrumentation. On the other hand, many papers were found for degenerative scoliosis and stenosis with treatment methods based on aetiology of spinal canal stenosis and analysis of global sagittal and frontal parameters. Satisfactory clinical results after operative treatment range from 83 to 96 % but with increased percentage of complications. Recent literature analysed the importance of stabilizing or not the spine after decompression in such situation knowing the increasing risk of instability after facet resection. No prospective randomized studies were found to support short instrumentation. Long instrumentation and fusion to prevent distabilization after decompression were always associated with higher complication rates. Imbalance patients with unsatisfactory compensation capacities were at risk of complications.CONCLUSIONSOperative treatment using newly proposed classification system of lumbar scoliosis with associated canal stenosis is useful. Sagittal balance and rotatory dislocation are the main parameters to analyse to determine the length of fusion.

01 Sep 2016·Der OrthopädeQ4 · MEDICINE

Operative Therapie der degenerativen Lumbalskoliose

Q4 · MEDICINE

Review

Author: Franke, J ; Pumberger, M ; Zahn, R K ; Putzier, M ; Halm, H

BACKGROUNDDe-novo scoliosis is most commonly associated with chronic back pain and in 70 % of cases with neurological symptoms of the lower extremities. In recent literature, the occurrence and severity of segmental lateral listhesis has been discussed as being an important prognostic factor of sagittal and frontal deformity progression. In general, operative interventions in patients with de-novo scoliosis are associated with a high rate of complications. Therefore, conservative treatment modalities are recommended at early stages of the disease. If conservative management fails and a sufficient reduction of the patient's symptoms cannot be achieved, depending on the symptoms, a selective decompression, short-segment fusion or long-instrumented reduction and fusion are indicated. Additionally to the patient's symptoms, specific imaging diagnostics are necessary to develop an adequate surgical treatment strategy.TREATMENTSelective decompression without fusion is indicated in patients with a fixed deformity and primarily neurologic pain or deficits. In conditions of a focal pathology as cause of significant low back pain and/or neurologic symptoms at early stages of deformity, a short segment fusion is the treatment of choice. However, short-segment fusion as a less-invasive procedure must not be performed in biplanar unbalanced patients and/or advanced de-novo scoliosis. In advanced degenerative de-novo scoliosis a long-segment reposition and fusion following an alignment correction are needed. Standardized pre-operative planning and perioperative management are highly critical to the post-operative success.CONCLUSIONAll operative treatment strategies in patients with de-novo scoliosis can be successful but they require sophisticated and individual surgical indication.

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