Three-year follow-up after Woodward procedure in children with Sprengel’s deformity

Sprengel deformity is a rare congenital disorder characterized by a congenital high scapula caused by obstruction of the development of the scapula, which accompanies bone, muscle and cartilage during pregnancy. This scapular deformity can be associated with other congenital malformations such as rib abnormalities, scoliosis, and Klippel-Feil syndrome. This is a follow-up of 3-year-old children with Sprengel deformity, cavendish grade 3 who were treated with the Woodward procedure. Authors conclude that the Woodward procedure is an effective procedure for obtaining good functional and cosmetic results.

A rare case of Sprengel deformity associated with spondylocostal dysostosis in a four-year child

Sprengel deformity is a complex congenital anomaly affecting one or both scapulae with mispositioning and dysplasia of scapula. It occurs due to failure of descent of scapula during intrauterine development and is the commonest congenital skeletal deformity of the scapula, often associated with other skeletal deformities. spondylocostal dysostosis is a rare genetic disorder which is characterized by malformation of the bones of the spine and ribs. It occurs in approximately 1 in 2,00,000 people worldwide. In spondylocostal dysostosis, affected children have wedge shaped vertebrae along with ribs that are fused, forked or sometimes missing. Based on the severity of the defect's children may present with short stature, scoliosis or hypoplasia of lungs causing difficulty in breathing and recurrent respiratory tract infections. Management of both these skeletal deformities depends on their severity and the presence of associated deformities. Surgical intervention might be required for cosmetic and functional recovery of the shoulder. Authors had a rare and interesting case of a 4-year-old boy who had features of Sprengel deformity affecting left scapula which was associated with features of spondylocostal dysostosis.

Anesthesia for elective bilateral sagittal slip osteotomy of the mandible and genioplasty in a young man with Klippel-Feil syndrome, Sprengel deformity, and mandibular prognathism

Klippel-Feil syndrome is characterized by congenital fusion of two or more cervical vertebrae, a low hair line at the back of the head, restricted neck mobility, and other congenital anomalies. We report a 16-year-old young man with Klippel-Feil syndrome, Sprengel deformity of the right scapula, thoracic kyphoscoliosis, and mandibular prognathism with an anterior open bite. He was treated with orthodontic treatment and maxillofacial surgery. An anticipated difficult airway due to a short neck with restricted neck movements and extrinsic restrictive lung disease due to severe thoracic kyphoscoliosis increased his anesthesia risk. Due to his deviated nasal septum and contralateral inferior turbinate hypertrophy, we chose awake fiber optic orotracheal intubation followed by submental intubation. Considering the cervical vertebral fusion, he was carefully positioned during surgery to avoid potential spinal injury. He recovered well and his postoperative course was uneventful.

Fanconi anemia: in all its glory.

Fanconi Anemia (FA) is a rare autosomal recessive disorder affecting multiple body systems. The diagnosis is based on morphological abnormalities, hematological abnormalities (pancytopenia, macrocytic anemia & progressive bone marrow failure) and genetic testing. However, genetic testing is complicated for FA because there are often many genes that are associated with its development, and large duplications, deletions or sequence variations are frequently observed in some of these genes. We report a patient with cytogenetically confirmed Fanconi anemia. Although morphological abnormalities were present from birth, diagnosis was suspected and made at 8 years of age when he presented to us. We report this case to create awareness among clinicians to use modern modalities of diagnosis for such cases in addition to the clinical assessment. This would further help these children reach their adulthood with good quality of life.

Sprengel Deformity with Bilateral Huge Omovertebra / 대한정형외과학회잡지

Sprengel's deformity is characterized by the congenital migration of the scapula superiorly in relation to the thoracic cage. Other congenital anomalies, such as Klippel-Feil syndrome, may occur in combination with Sprengel's deformity. We report on a case of Sprengel's deformity with a huge bilateral omovertebrae, which was combined with the clinical features of Klippel-Feil syndrome, including cervical fusion, short neck, low posterior hairline, and limitation of neck motion. However, no other deformities or functional defects were observed.

Deformidade de Sprengel: tratamento cirúrgico pela técnica de green modificada / Sprengel's deformity: surgical correction by a modified green procedure

OBJETIVO: Demonstrar os resultados estéticos e funcionais de pacientes submetidos à correção cirúrgica de escápula alta congênita - deformidade de Sprengel - por uma modificação da técnica de Green, bem como avaliar o grau de satisfação dos pacientes e as complicações da técnica utilizada. MÉTODOS: Foram avaliados nove pacientes operados pela técnica de Green modificada, no período de setembro de 1993 a abril de 2008. Como modificação da técnica original foram realizados descolamento muscular subperiosteal, ressecção apenas da porção súpero-medial da escápula e, em vez da utilização de tração esquelética, optou-se pela fixação com fio de aço subcutâneo da porção medial da espinha da escápula à crista ilíaca posterior contralateral. A idade média dos pacientes foi de sete anos e três meses. O seguimento pós-operatório médio foi de três anos e sete meses. RESULTADOS: Houve incremento médio na elevação de cerca de 39º (variando de 0º a 80º ). Segundo a classificação de Cavendish, obteve-se a melhora estética de dois graus em oito casos e de três graus em um. Todos os pacientes ficaram satisfeitos com o resultado. CONCLUSÕES: Os pacientes com deformidade de Sprengel submetidos a tratamento cirúrgico por meio de uma modificação da técnica de Green, com fixação da escápula na crista ilíaca posterior contralateral em vez de se utilizar tração esquelética, apresentaram melhora tanto funcional como estética; todos os pacientes e/ou familiares ficaram satisfeitos e as complicações relacionadas com a técnica cirúrgica não interferiram no resultado final.

OBJECTIVE: To evaluate the cosmetic and functional results of patients submitted to surgical correction of Congenital High Scapula (Sprengel's Deformity) using modified Green's Procedure, as well as patients' satisfaction and complications. METHODS: Nine patients submitted to surgical treatment from September 1993 to April 2008 have been assessed. The modification from original technique was: subperiosteal muscle detachment, resection of superomedial scapular portion and fixation of medial portion of scapular spine to contralateral posterior iliac crest instead of skeletal traction, with subcutaneous wire. The mean age was 7 years and 3 months. The mean follow-up time was 3 years and 7 months. RESULTS: The mean improvement in forward elevation was 39o (range 0 to 80o). According to the Cavendish Classification, cosmetic improvement of two degrees was achieved in eight cases, and three degrees in one. All patients were satisfied with results. CONCLUSIONS: Surgical correction of Sprengel's Deformity by a modified Green's procedure with contralateral posterior iliac crest fixation instead of skeletal traction, showed both cosmetic and functional improvements; all patients and/or family members were satisfied with the results, and the complications associated to the surgical technique did not interfere on end results.

Surgical therapy of Sprengel deformity by Woodward procedure: A case report

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.

A Case of Klippel-Feil Syndrome

Klippel-Feil syndrome(KFS) consists of short neck, low posterior hairline and restriction of motion of the neck due to fusion of cervical vertebrae. The typical disorder results from a failure of the normal segmentation of mesodermal somites during 3-8 weeks of gestation. In 1912, the first complete clinical description of this syndrome was given by Klippel and Feil. Feil reported additional cases in 1919 and distinguished between three morphologic groups. The incidence of KFS has been estimated to be approximately 1 : 40,000-42,000 births. A slight female predilection has been noted. Although the disorder is sporadic, there are examples of familial occurrence; how ever, no clear mechanism of inheritance has been accepted. Since the disturbance producing a short neck occurs early in embryogenesis, defects in other organ systems may occur at the same time. Common musculoskeletal anomalies that accompany KFS include scoliosis, as well as Sprengel's deformity in as many as one-third of cases. Neurologic, cardiovascular, and urinary tract anomalies are associated with KPS. We report a case of Klippel-Feil syndrome with associates anomalies include Sprengel's deformity.

Assessment of Sprengel Deformity Using Three - Dimensional Computed Tomography / 대한정형외과학회잡지

We evaluated the scapular shape, dispiacement and rotation in 10 cases of Sprengel deformity using three-dimensional computed tomography in order to investigate its clinical usefulness. Standard views, including trunk posterior view, scapular true posterior view and medial view, were taken, and the 3-D image was rotated in three axes to visualize the omovertebral bony connection. In the trunk posterior view, the amount of superior displacement of the affected scapula was measured using the glenoid level as reference, and the rotational deformity by the tilting of the base of scapular spine. Scapular dysplasia was evaluated in the scapular true posterior and medial views. The presence, size, and anchoring points of omovertebral bone were assessed in various view points. There was a tendency of inverse correlation hetween superior displacement and rotational deformity of scapula. In most cases, the affected scapulae were convex at their medial borders and concave at their lateral borders, with increased width/height ratio. The anchoring point of omovertebral connection appeared to determine the scapular shape, level, and amount of rotation. Three-dimensional CT was helpful in preoperative planning.

Congenital High Scapula Associated with Anomaly of the Acromion: A Case Report / 대한정형외과학회잡지

Congenital high scapula, more commonly referred to as Sprengel's deformity, was first described by Eulenberg in 1863. Usually other congenital anomalies are associated such as absent or fused ribs, chest wall asymmetry, Klippel-Feil syndrome, cervical ribs, congenital scoliosis, cervical spina biffida was treated with the excision of the elongated acromion and the resection of prominent elongated acromion to be described is an extremely rare malformation. We experienced one case of congenital high scapula associated with anomaly of the acromion, which was treated with the excision of the elongated acromion and the resection of prominent supermedial border of the scapula for the purpose of the cosmetic and functional correction. After 1 year follow up, abduction was improved by only 10 degrees in the glenohumeral joint with the definite cosmetic improvement.