In Klippel flail syndrome all are seen except:
Ans: A (High...) ",Prominence of the trapezius muscle gives the appearance of webbing at the base of neck. The posterior hairline is much lower than the normal. Associated anomalies includes are common & includes hem iverteh rae, posterior arch defect, cenical meningomyelocele, thoracic defects, scapular elevation & visceral a noma lies''- Apley 8th/161KLIPPEL-FLAIL SYNDROMECCONGENITAL SHORT NECK) CANALE & BEATY: Campbell's Operative Orthopaedics, 11th edThe most common signs of the disorder are a short neck, low hairline at the back of the head, and restricted mobility of the upper spine.# Klippel-Feil syndrome is a congenital fusion of the cervical vertebrae that may involve two segments, a congenital block vertebra, or the entire cervical spine. Congenital cervical fusion is a result of failure of normal segmentation of the cervical somites during the third to eighth week of life.The skeletal system may not be the only system affected during this time; cardiorespiratory, genitourinary, and auditory systems frequently are involved.The classic clinical presentation of Klippel-Feil syndrome is the triad of a low posterior hairline, a short neck, and limited neck motion .This triad indicates almost complete cervical involvement and may be clinically evident at birth; however, fewer than half of patients with Klippel-Feil syndrome have all parts of the triad. Many patients with Klippel-Feil syndrome have a normal appearance, and the syndrome is diagnosed through incidental radiographs. Shortening of the neck and a low posterior hairline are not constant findings and may be overlooked; webbing of the neck (pterygium colli) is seen in severe involvement. The most constant clinical Finding is limitation of neck motion. Rotation and lateral bending are affected more than flexion and extension. If fewer than three vertebrae are fused or if the lower cervical vertebrae are fused, motion is only slightly limited.Occipitalization of the atlas, hemivertebrae, and basilar impression occur frequently in patients with Klippel- Feil syndrome, but their isolated occurrence is not considered part of this syndrome.In patients with Klippel-Feil syndrome, neurological compromise, ranging from radiculopathy to quadriplegia to death, can occur. The neurological symptoms are caused by occipitocervical anomalies, instability, or degenerative joint and disc disease. Instability and degenerative joint disease are common when two fused areas are separated by a single open interspace."Several congenital problems have been associated with congenital fusion of the cervical vertebrae, most commonly scoliosis, renal abnormalities, Sprengel deformity, deafness, synkinesis, and congenital heart defects"Associated abnormalities may include:Scoliosis (side-to-side curvature of the spine), which is abnormal curving of the spine. The spine sometimes appears as a "C" or an "S"spina bifida, when the spinal canal and the back bone do not close completely during birth.anomalies of the kidneys and the ribs,cleft palate (hole in the roof of the mouth), respiratory problems,heart malformations short statureDuane syndromeFig: Clinical (A and B) features of Klippel-Feil syndrome in young boy
High posterior hair line
Associated visceral anomalies present
Restriction of neck mobility'
Short neck
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