Hemivertebra Resection in Small Children. A Literature Review.

STUDY DESIGN: Systematic review. OBJECTIVE: To evaluate the lowest possible age to resect an HV in very young patients with a congenital deformity. METHODS: We sought to retrieve all studies reporting age at HV excision in patients with congenital scoliosis. Studies written in English were included. No publication date restrictions were imposed. A search of the PubMed and LiLacs databases was conducted. Additionally, a hand search was performed to supplement the database search. RESULTS: We found 140 articles. Twenty two studies were included into the final assessment. There was considerable heterogeneity in the included studies, both regarding age and the surgical techniques used. There was also a broad spectrum of recommendations regarding suggested age for treatment. The youngest patient undergoing resection and fusion was 3 months of life at the time of surgery. CONCLUSION: How young a patient could and should be submitted to HV resection surgery is still a matter of debate in the literature. Prophylactic surgery might be a proper treatment for young children with congenital scoliosis before malformation becomes a deformity, adding no additional neurological, vascular, or anesthesia-related complications. The defect can be treated early while the deformity is treated late.

Long-term results of hemivertebra excision: How does the spine behave after the peak of puberty?

INTRODUCTION: Early hemivertebra (HV) excision and posterior spinal fusion (PSF) is advocated as the treatment of choice in congenital kyphoscoliosis. We assessed global spinal balance and spinal deformity status once the pubertal peak has taken place, in children with a history of HV excision and PSF at age younger than 5 years. METHODS: Twenty-seven children with congenital kyphoscoliosis without co-existing proximal/distal congenital spinal abnormalities who underwent HV excision and PSF of ≤ 5 levels at age younger than 5 years and who had reached the peak of puberty at the last follow-up visit were evaluated. RESULTS: Twenty-seven HV excision were performed. Mean age at surgery was 3 years and 2 months. A mean of 3.2 segments were fused. Imbalance of the trunk was observed in 80% of thoracic and 75% of thoracolumbar HV excision. No arthrodesis technique (4 cases) presented 100% of spine decompensation. All children younger than 2 years at the time of surgery developed spinal imbalance. Eighteen patients (67%) had global spine imbalance; 81% male population and 63% of the female population. Mean age at surgery was 3 years + 2 months. Mean age of the patients at the time of the study was 15 years + 5 months. Mean follow-up was 12 years + 3 months. CONCLUSION: Many of these patients developed spinal imbalance and scoliosis worsened at the final follow-up. Early age at surgery, preoperative scoliosis severity, HV location, no arthrodesis technique, and the adding-on phenomenon may be involved.

Diagnóstico prenatal ultrasonográfico de hemivértebra / Prenatal Ultrasonographic Diagnosis of Hemivertebrae

Introducción: La hemivértebra es un raro defecto congénito de la columna vertebral fetal en la que solo se desarrolla el cuerpo vertebral de un lado, lo cual provoca su deformidad. Objetivo: Presentar un caso con diagnóstico ecográfico tridimensional prenatal de hemivértebra, como único defecto. Método: Se realizó evaluación ecográfica prenatal y examen anátomo patológico y radiológico posmortem al feto con escoliosis congénita provocado por hemivértebra. Se revisó la literatura sobre este defecto congénito, su diagnóstico prenatal y otros aspectos genéticos que deben tenerse en cuenta para el asesoramiento a la familia. Presentación de caso: Gestante de 28 años remitida a la consulta provincial de Genética Médica en la ciudad de Camagüey, Cuba, el 25 de septiembre del 2018, por sospecha ultrasonográfica de hemivértebra fetal con 20 semanas de gestación. Se confirma diagnóstico a esta instancia, mediante ultrasonografía tridimensional. Con el consentimiento familiar informado se realiza interrupción de la gestación y se comprueba el diagnóstico prenatal realizado por estudios radiológicos y anátomo patológico de la región dorso lumbar. Conclusiones: Se concluye como un defecto congénito múltiple, aislado, de posible etiología multifactorial. Se destacó el valor de la ecografía tridimensional, vista sagital coronal, como método no invasivo más empleado para el diagnóstico prenatal(AU)

Introduction: The hemivertebrae is a rare congenital defect of the fetal spine in which only the vertebral body develops on one side, resulting in deformity. Objective: To present a case with three-dimensional prenatal ultrasound diagnosis of hemivertebrae, as the only defect. Method: Prenatal ultrasound evaluation, postmortem anatomopathological and radiological examination were performed in a fetus with congenital scoliosis caused by hemivertebrae. The literature on this congenital defect, the prenatal diagnosis and other genetic aspects that should be taken into account for family counseling was reviewed. Case report: A 28-year-old pregnant woman referred to the provincial office of Medical Genetics in Camagüey, Cuba, on September 25, 2018, due to ultrasonographic suspicion of fetal hemivertebrae. She was 20 weeks of gestation. Diagnosis is confirmed by three-dimensional ultrasonography. After the informed family consent, the pregnancy was interrupted. The prenatal diagnosis was verified by radiological and pathological studies of the lumbar back region. Conclusions: It is concluded as a multiple congenital defect, isolated, of possible multifactorial etiology. The value of three-dimensional ultrasound, coronal sagittal view, was highlighted as the most commonly used, non-invasive method for prenatal diagnosis(AU)

Resolución quirúrgica de escoliosis congénita: reporte de caso / Surgical resolution of congenital scoliosis: case report

Las deformidades congénitas de la columna vertebral, constituyen uno de los trastornos musculoesqueléticos no traumáticos de difícil manejo clínico quirúrgico, por el alto grado de deformidad que producen y porque su incidencia es menor que las idiopáticas. Se presenta el caso de una paciente de 14 años de edad, quien fue valorada en una consulta externa del Hospital Carlos Andrade Marín de la ciudad de Quito, Ecuador, con una deformidad severa toracolumbar, presente desde el nacimiento, que ha ido progresando hasta causar dolor a la movilidad y limitando sus actividades diarias, impidiendo un desarrollo social adecuado, por lo que, se decide su resolución quirúrgica mediante artrodesis posterior instrumentada, osteotomías correctivas y resección de hemivértebra y barra espinal, posterior a lo cual se realizó fisioterapia y un seguimiento por consulta externa de un año. Su evolución fue favorable, mejorando su sintomatología y movilidad.

Congenital deformities of the spine constitute one of the non-traumatic musculoskeletal disorders of difficult clinical surgical management, due to the high degree of deformity they produce and their incidence is lower than idiopathic ones. We present the case of a 14-year-old patient who was evaluated in the outpatient clinic of the Carlos Andrade Marín Hospital in the city of Quito, Ecuador, with a severe thoracolumbar deformity, present from birth, which has progressed to cause pain to mobility, limiting daily activities, preventing an adequate social development, for which, surgical resolution was decided through instrumented posterior arthrodesis, corrective osteotomies, and resection of hemivertebra and spinal rod, after which physiotherapy was performed and follow-up by external consultation for 1 year. Its evolution was favorable, improving its symptomatology and mobility.

Evaluation of the influence of kyphosis and scoliosis on intervertebral disc extrusion in French bulldogs.

BACKGROUND: Although thoracic vertebral malformations with kyphosis and scoliosis are often considered incidental findings on diagnostic imaging studies of screw-tailed brachycephalic breeds, they have been suggested to interfere with spinal biomechanics and intervertebral disc degeneration. It is however unknown if an abnormal spinal curvature also predisposes dogs to develop clinically relevant intervertebral disc herniations. The aim of this study was to evaluate if the occurrence of thoracic vertebral malformations, kyphosis or scoliosis would be associated with a higher prevalence of cervical or thoracolumbar intervertebral disc extrusion in French bulldogs. RESULTS: French bulldogs that underwent computed tomography for reasons unrelated to spinal disease (n = 101), and French bulldogs with thoracolumbar (n = 47) or cervical intervertebral disc extrusion (n = 30) that underwent magnetic resonance imaging were included. There was a significant association between the presence of kyphosis and the occurrence of intervertebral disc extrusion, particularly in the thoracolumbar region. Dogs with kyphosis were at nearly a two times increased odds of being affected by intervertebral disc extrusion than those without kyphosis [(OR = 1.98 (95% CI: 1.04-3.78)]. There was also an association between the presence of scoliosis and the anatomical distribution of intervertebral disc extrusions, with dogs with scoliosis more likely to have more caudal lumbar intervertebral disc extrusions. Presence of scoliosis was not associated with an increased odds of being affected by intervertebral disc extrusion. CONCLUSIONS: Although thoracic vertebral malformations with kyphosis only rarely cause spinal cord dysfunction in itself, French bulldogs with kyphosis appear to be at higher risk to develop thoracolumbar intervertebral disc extrusion.

Hemivértebra em cães – Revisão de literatura / Hemivertebrae in dogs – Literature review

Hemivértebras são anomalias congênitas encontradas mais frequentemente em cães braquicefálicos com cauda helicoidal. Representam em torno de 7% das alterações em coluna relatadas em literatura. Ocorrem mais comumente na região da coluna tóraco-lombar, causando sintomas de lesão de neurônio motor superior em membros pélvicos. Os cães podem apresentar sintomatologia desde filhotes com progressão durante o crescimento. O diagnóstico é efetuado com base nos sintomas, exame físico e exames de imagem. O tratamento pode ser conservativo em animais sem progressão dos sintomas, porém, em animais com a progressão destes, o mais indicado, atualmente, é a estabilização cirúrgica, com ou sem descompressão medular.

Hemivertebrae are congenital anomalies found most often in brachiocephalic dogs with helical tails. These anomalies represent around 7 % of the spinal alterations reported in the literature. They occur most commonly in the thoracolumbar region of the spine, causing symptoms of upper motor neuron damage in hind limbs. Dogs may display symptoms early in life which progress during growth. Diagnosis is based on symptoms, physical examination, and imaging studies. Treatment may be conservative in animals whose symptoms do not progress, but in animals whose symptoms progress, the treatment currently most indicated is surgical stabilization, with or without spinal decompression.

Hemivértebra em cães Revisão de literatura / Hemivertebrae in dogs Literature review

Hemivértebras são anomalias congênitas encontradas mais frequentemente em cães braquicefálicos com cauda helicoidal. Representam em torno de 7% das alterações em coluna relatadas em literatura. Ocorrem mais comumente na região da coluna tóraco-lombar, causando sintomas de lesão de neurônio motor superior em membros pélvicos. Os cães podem apresentar sintomatologia desde filhotes com progressão durante o crescimento. O diagnóstico é efetuado com base nos sintomas, exame físico e exames de imagem. O tratamento pode ser conservativo em animais sem progressão dos sintomas, porém, em animais com a progressão destes, o mais indicado, atualmente, é a estabilização cirúrgica, com ou sem descompressão medular.(AU)

Hemivertebrae are congenital anomalies found most often in brachiocephalic dogs with helical tails. These anomalies represent around 7 % of the spinal alterations reported in the literature. They occur most commonly in the thoracolumbar region of the spine, causing symptoms of upper motor neuron damage in hind limbs. Dogs may display symptoms early in life which progress during growth. Diagnosis is based on symptoms, physical examination, and imaging studies. Treatment may be conservative in animals whose symptoms do not progress, but in animals whose symptoms progress, the treatment currently most indicated is surgical stabilization, with or without spinal decompression.(AU)