Webbed neck syndrome skin#

These patients can often have specific factor deficiency or platelet aggregation anomalies, but none were noted with this patient. Presurgical testing did not reveal any coagulation abnormalities. The patient presented with characteristic features of pterygium colli with a low and laterally displaced nuchal hairline. Case ReportĪ 13-year-old male was evaluated for congenital webbing of the neck secondary to Noonan syndrome. We have termed this technique an M to T rearrangement. In order to address these disadvantages, a novel surgical technique was used on this patient. Previously reported posterior and lateral approaches have notable disadvantages with regard to hairline displacement and recurrence. Since its description, various surgical approaches have been implemented to correct the deformity.

Webbed neck syndrome skin#

Excess hair-bearing skin is also present and extends down the cervical region well beyond the normal hairline. Pterygium colli involves an ectopic fibrotic facial band superficial to the trapezius muscle. Kobylinski initially reported this deformity in 1883 and the name “pterygium colli” was coined by Funke in 1902. įor the Noonan syndrome patient, the most concerning physical defect is often congenital webbing of the neck or pterygium colli. An autosomal dominant pattern of inheritance has been determined and attributed to missense mutations in the PTPN11 gene on chromosome 12 in 50% of cases. The syndrome is fairly common, as the incidence is estimated to be approximately 1 : 1,000–2,500 live births. Other findings can include broad or webbed neck, pectus deformity, cryptorchidism, characteristic facies, varied coagulation defects, lymphatic dysplasias, and ocular abnormalities. Noonan syndrome is characterized by short stature, congenital heart defect, and developmental delay of variable degree. IntroductionĪ syndrome involving valvular pulmonary stenosis, small stature, hypertelorism, mild mental retardation, undescended testes, and skeletal abnormalities was first described by Noonan and Ehmke in 1963. M to T rearrangement is an effective technique for the correction of webbed neck deformities seen in Noonan and Turner syndromes.

An important effect of this technique is the prevention of anterior displacement of hair bearing skin. The excision of excess skin along with the zig-zag closure also prevents postoperative scar contraction and recurrence. Inferolateral displacement of hair-bearing skin can be removed with resection of the superior intervening triangle and improves the appearance of the low nuchal hairline. This prevents the recurrence seen with the use of posterior techniques. Using a lateral approach, the M and T incisions are made and the trapezial fascial web is directly visualized and able to be completely excised. In order to address these disadvantages, a new surgical technique was used on this patient. We present a patient with pterygium colli and a low and laterally displaced nuchal hairline. For the Noonan syndrome patient, the most concerning physical defect is often congenital webbing of the neck or pterygium colli.