RESUMEN
Sprengel's deformity is a conspicuous anomaly, affecting one or both scapulas. The congenital elevation of the scapula is frequently accompanied by additional anomalies, such as rib, vertebral, or muscular deformities, among which are rib fusion or vertebral deformity. Defects in the cervical vertebrae are most likely to result in Klippel-Feil syndrome, which is characterised by a short neck, restrictions on head mobility, and low-growing neck hair. Fewer than half of the instances had scoliosis, which is compensatory due to efforts to straighten the spine. The present case report was the case of Sprengel's deformity reported to our department.
RESUMEN
BACKGROUND: Sprengel's deformity is a congenital abnormality of the shoulder girdle. Because scapular retraction, such as the Green procedure, is usually performed during childhood to improve esthetics and shoulder function, Sprengel's deformity is rarely found in older patients. CASE PRESENTATION: We presented a unique case of a Japanese female cadaver with Sprengel's deformity at the age of 80 years. Anatomical dissection and radiological imaging revealed musculoskeletal anomalies associated with Sprengel's deformity, including Klippel-Feil syndrome, presence of an omovertebral bone, and absence of the trapezius muscle. In addition, bilateral cervical ribs were in contact with the brachial plexus. These anomalies may lead to numbness, pain, and limited range of motion of the neck and upper girdle with aging. CONCLUSIONS: Because most adult patients with Sprengel's deformity experience neck pain and limited movement of the shoulder, the presented case is a rare case of neglected Sprengel's deformity in an 80-year-old cadaver.
Asunto(s)
Cadáver , Escápula , Escápula/anomalías , Articulación del Hombro/anomalías , Humanos , Femenino , Anciano de 80 o más Años , Escápula/diagnóstico por imagen , Síndrome de Klippel-Feil/complicaciones , Anomalías Congénitas/diagnóstico por imagen , Plexo Braquial/anomalías , Plexo Braquial/diagnóstico por imagenRESUMEN
PURPOSE: Klippel-Feil syndrome (KF) is a rare disease defined as single or multi-level cervical vertebra fusion. KF could be accompanied by other spinal anomalies or isolated, and in which case necessity of whole spine screening is not clearly known. KF is investigated in terms of prevalence, gender distribution, fusion types, and frequency of accompanying anomalies according to types of KF. METHODS: Approval from our hospital's ethics committee was received for this single-center, retrospective study. Considering the exclusion criteria among the 40,901 cervical spine MRIs, 40,450 patients were included in the study. It was re-evaluated for KF, fusion level, classification, cervical scoliosis, and other musculoskeletal and spinal anomalies. RESULTS: 125 (0.309%) of 40,450 patients is diagnosed with KF, which is more common in women (P < 0.001). Single fused segment 106 (84.8%), multilevel fused segments 8 (6.4%), contiguous fused segments 11 (8.8%) are observed. Upper level KF is detected in 13 (10.4%) patients. The frequency of additional anomaly is significantly higher in upper level KF compared to other level fusions (P < 0.001, Chi-square t). The cervical scoliosis is diagnosed 34 (27%). In KF patients with scoliosis, the frequency of additional anomalies was significantly higher (P < 0.001, Chi-square t). CONCLUSION: Klippel-Feil prevalence is 0.309%, it is frequently observed in women, and at C2-C3 level. Additional anomalies are especially associated with 'contiguous fused segments' and 'upper level' types. Klippel-Feil with scoliosis is an indicator of increased risk for associated anomalies, and examination of the whole spine is recommended.
Asunto(s)
Vértebras Cervicales , Síndrome de Klippel-Feil , Síndrome de Klippel-Feil/diagnóstico por imagen , Síndrome de Klippel-Feil/complicaciones , Síndrome de Klippel-Feil/epidemiología , Humanos , Femenino , Masculino , Adulto , Estudios Retrospectivos , Persona de Mediana Edad , Vértebras Cervicales/anomalías , Vértebras Cervicales/diagnóstico por imagen , Adolescente , Niño , Adulto Joven , Anciano , Escoliosis/epidemiología , Escoliosis/diagnóstico por imagen , Prevalencia , Preescolar , Imagen por Resonancia MagnéticaRESUMEN
PURPOSE: To report the functional and cosmetic results of cases with Sprengel deformity (SD) who underwent a novel technical modification in Woodward procedure for severe SD comprising of lowering of the scapula followed by stabilization of scapula with application of anchoring sutures. MATERIALS AND METHODS: Twenty-three children were operated at a mean age of 6.6 ± 2.5 years (range 3.4-11 years) and followed up for a mean period of 5.51 ± 1.98 years. RESULTS: The mean Cavendish score of the patients before procedure of 3.2 ± 0.45 was reduced to 1.7 ± 0.56 (p < 0.001) after the surgery. All patients improved in global shoulder abduction. The mean preoperative abduction of 97° ± 15.2° increased to a mean of 149° ± 14.1° post-operatively (p < 0.001). Significant improvement was also observed in mean Rigault grades after surgical intervention using anchoring suture modification of Woodward procedure. The mean Rigault grade of 2.7 ± 0.47 prior to surgery was reduced to 1.3 ± 0.47 (p < 0.001) at final evaluation. Age of the patients negatively correlated with the functional outcome (r = - 0.48; p < 0.02). The presence of an omovertebral bar or associated congenital anomalies did not affect the functional outcome. CONCLUSION: The modification of Woodward procedure for correction of SD by application of anchoring sutures was a reliable method for correction of SD particularly at younger age. The procedure was associated with significant improvement in shoulder abduction, cosmetic appearance and radiological grades over 5 year follow-up.
RESUMEN
We present a report of two patients having the association of omovertebra, Sprengel's deformity of the shoulder and Klippel-Feil abnormality with craniovertebral junctional instability. Our literature survey did not locate any report of such association. Significance of bone alterations is analyzed. Two young patients presented with neck pain, torticollis, webbed neck, and spastic quadriparesis. In both patients, the investigations revealed basilar invagination, Klippel-Feil abnormality and Sprengel's deformity of the shoulder. Apart from these relatively common associations, both the patients had omovertebral bone that extended from the transverse process of C5 vertebra to scapula. Following atlantoaxial stabilization surgery, the patients rapidly recovered from all symptoms. Musculoskeletal abnormalities at the craniovertebral junction that include Klippel-Feil abnormality, Sprengel's shoulder, and omovertebra are secondary alterations to primary atlantoaxial instability.
RESUMEN
The presence of an omovertebral bone with Sprengel's deformity and Klippel-Feil syndrome is a complex congenital anomaly that is not well understood. It most commonly manifests as cosmetic deformity, limited range of motion, and functional disability, although there are reports of the insidious development of cervical myelopathy. In this paper, the authors present the case of a 49-year-old man with acute neurological deficits after a low-energy mechanism of traumatic spinal cord compression, resulting from an impinging omovertebral bone through a traumatic laminar defect. The patient underwent resection of the omovertebral bone, laminectomy decompression of the spinal canal, and anterior stabilization. This case highlights a rarely discussed complication of undiagnosed Sprengel's deformity and its associated conditions following even low-energy traumatic mechanisms.
RESUMEN
Coexistence of Klippel-Feil syndrome with Sprengel deformity and omovertebral bone is a rare complex bone abnormality with unknown incidence and etiology. Herein, we report a case of a 6-year-old girl with coexistence of these congenital abnormalities evaluated by three-dimensional computed tomography. We also make a brief review and discuss in details the role of this imaging modality in the evaluation of such complex cases.
Asunto(s)
Anomalías Congénitas/diagnóstico por imagen , Síndrome de Klippel-Feil/diagnóstico por imagen , Escápula/anomalías , Articulación del Hombro/anomalías , Niño , Femenino , Humanos , Imagenología Tridimensional , Tomografía Computarizada Multidetector/métodos , Enfermedades Raras , Escápula/diagnóstico por imagen , Articulación del Hombro/diagnóstico por imagenRESUMEN
Klippel-Feil syndrome is a rare condition characterized by the congenital fusion of two of the seventh cervical vertebrae. A 50-year-old woman presented with a 2-year history of neck pain and ataxia for 1 year. She had not urinary incontinence. She was referred to a neurosurgeon by a neurologist because of her progressive gait ataxia. Risk for brachial plexus injury because of compression or stretching by the clavicle accelerate with age. Therefore, the surgical approach of adults' patients with Sprengel's deformity can intend suitable surgical conclusions.
RESUMEN
Congenital undescended scapula is congenital structural abnormality which affects only one side usually. Scapula located higher than the usual and rotating deformity that inferior angle to medial side, superior angle to lateral side is common. This report presents one case of the surgical therapy of a sprengel deformity patient who passed an optimal operation period with age 3~7years old, and includes brief review of the literature. 7 years old boy whose chief complaint was the limitation of left scapular-thoracic movement and he had an omovertebral bone bridge and periscapular muscle atrophy. There was improvement of motion ranges and cosmetic problems after surgical treatment.