Presentation
Both patients presented with back and leg pain, urinary hesitancy/incontinence, difficulty sitting and lying down, waddling gait, and restriction of movement. [ncbi.nlm.nih.gov]
Entire Body System
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Developmental Delay
We report on an 8-year-old girl with congenital scoliosis (segmented hemivertebra between the second and third lumbar vertebrae) and psychomotor developmental delay. She has a de novo reciprocal translocation, t(13;17)(q34;p11.2). [ncbi.nlm.nih.gov]
The occurrence of a de novo reciprocal translocation, t(13;17)(q34;p11.2), in a girl with psychomotor developmental delay and congenital scoliosis due to segmented hemivertebra has raised the possibility that 17p11.2 is a candidate region for a hemivertebra [rrnursingschool.biz]
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Intermittent Claudication
A 71-year-old man presented with left lower back pain and intermittent claudication accompanied by severe left buttock pain. [ncbi.nlm.nih.gov]
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Infertility
A 35-year-old infertile man with unilateral renal and deferential agenesis, seminal vesicle cyst and hemivertebra is reported. [ncbi.nlm.nih.gov]
Skin
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Delayed Wound Healing
Unanticipated surgeries were performed on eight patients, including one delayed wound healing, two pedicle fractures, one progressive deformity and four implants removals. [ncbi.nlm.nih.gov]
Two cases had delayed wound healing but the wounds did heal after regular dressing changes. A typical hemivertebra resection case is shown in Figure 1. [publishing.rcseng.ac.uk]
In 2011, Zhang et al. reported a 10.8 % complication rate in 56 cases during a mean follow-up of 32.9 months, including one delayed wound healing, two pedicle fractures, two rod breakages, and one additional surgery for proximal junction kyphosis [ 18 [josr-online.biomedcentral.com]
Musculoskeletal
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Torticollis
We present our experience with two cases of C3 hemivertebra resection and torticollis correction via a combined anterior-posterior-anterior surgical approach and short segment fixation. [ncbi.nlm.nih.gov]
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Buttock Pain
A 71-year-old man presented with left lower back pain and intermittent claudication accompanied by severe left buttock pain. [ncbi.nlm.nih.gov]
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Leg Pain
Both patients presented with back and leg pain, urinary hesitancy/incontinence, difficulty sitting and lying down, waddling gait, and restriction of movement. [ncbi.nlm.nih.gov]
Urogenital
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Urinary Incontinence
incontinence In most cases, signs plateau at age 9 months when the vertebrae stop growing. [marvistavet.com]
Warning: Should abnormal pressure on the spinal cord be caused the following signs can be seen: Weakness of the rear limbs Fecal incontinence Urinary incontinence Warning: It is important to understand that usually the presence of hemivertebrae is of [vet4bulldog.com]
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Urinary Hesitancy
Both patients presented with back and leg pain, urinary hesitancy/incontinence, difficulty sitting and lying down, waddling gait, and restriction of movement. [ncbi.nlm.nih.gov]
Neurologic
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Waddling Gait
Both patients presented with back and leg pain, urinary hesitancy/incontinence, difficulty sitting and lying down, waddling gait, and restriction of movement. [ncbi.nlm.nih.gov]
Workup
Workup An accurate radiologic evaluation is essential. Standard posteroanterior (PA) and lateral views of the entire spine are used for the initial evaluation. [emedicine.medscape.com]
Treatment
Depending on their location and the magnitude of the resultant deformity, they may be asymptomatic or require treatment. In the past, treatment has focused on prevention of deformity progression in growing children. [ncbi.nlm.nih.gov]
Prognosis
Hemivertebrae, which are the most frequent cause of congenital scoliosis, pose a challenge in terms of prognosis and therapy. [ncbi.nlm.nih.gov]
Treatment and prognosis The prognosis can be variable dependent on the type of segmentation anomaly, from being a progressive to a non-progressive deformity. The strongest negative impact is when a hemivertebra occurs at the lumbosacral level 7. [radiopaedia.org]
Prognosis The prognosis can be variable dependant on the type from being a progressive to non progressive deformity. The strongest negative impact is when a hemivertebra occurs at the lumbo-sacral level 7. [operativeneurosurgery.com]
In view of the poor prognosis of surgical intervention he was fitted with an Jewett brace so that further neurological impairment was avoided. [e-arm.org]
Other types of hemivertebra, especially those involving unbalanced congenital scoliosis, usually progress markedly with growth and have a relatively poor prognosis unless early spinal fusion prevents further spinal curvature. [strongbones.in]
Etiology
The etiology of congenital scoliosis remains unclear. Both a genetic basis and environmental influences have been postulated. A few isolated reports of discordant spinal anomalies in twins exist. [ncbi.nlm.nih.gov]
PubMed Central View Article PubMed Google Scholar Hensinger RN: Congenital scoliosis: etiology and associations. Spine (Phila Pa 1976). 2009, 34 (17): 1745-1750. 10.1097/BRS.0b013e3181abf69e. [scoliosisjournal.biomedcentral.com]
Karger AG, Basel References Wynne-Davies R: Congenital vertebral anomalies: Etiology and relationship to spina bifida cystica. J Med Genet 1975;12:280–288. Moore KL: The Developing Human ed 2. Philadelphia, Saunders, 1983, p 217. [karger.com]
Epidemiology
A cervical location is rare and even rarer as a cause of cervical subluxation in flexion and extension (for which only one previous case has been found). see cervical hemivertebra Epidemiology The estimated incidence is at 0.3 per 1000 live births 2. [operativeneurosurgery.com]
Descriptive epidemiology of hemivertebrae, Hawaii, 1986–2002. Congenit Anom (Kyoto) 2006; 46: 172–176. 4. Moore KL. The developing human. Fourth edn. Philadelphia: W. B. Saunders Co., 1988. Copyright 2014 ISUOG. Published by John Wiley & Sons Ltd. [docksci.com]
Descriptive epidemiology of hemivertebrae, Hawaii, 1986–2002. Congenit Anom. 2006:46172–46176. [ PubMed ] [ Google Scholar ] 17. Connor JM, Conner AN, Connor RAC, Tolmie JL, Yeung B, Goudie D. Genetic aspects of early childhood scoliosis. [ncbi.nlm.nih.gov]
Pathophysiology
Stay current on the latest standards in medical care thanks to extensive updates, including new chapters on Spinal Cord Stimulation and Implantable Drug Delivery Systems, Special surgical Techniques for the Growing Spine, Pathophysiology of Degenerative [books.google.ro]
Prevention
In the past, treatment has focused on prevention of deformity progression in growing children. Little has been written about congenital scoliosis presenting in adulthood. [ncbi.nlm.nih.gov]