Surgical Correction of a Webbed-Neck Deformity in Turner’S Syndrome
Languages of publication
Turner’s syndrome occurs in approximately 1 out of every 2,000 to 2,500 live-born girls. This genetically determined pathology is characterised by multiple congenital anomalies. A typical form of this syndrome is associated with a lack of one of the sex chromosomes (karyotype 45, X). From the point of view of plastic surgery, one of the most important elements of the therapy is correction of the webbed neck deformity. The aim of the study was to present the possibilities of surgical treatment of a webbed neck of patients with Turner’s syndrome and the evaluation of treatment results. In the years 2000-2012, six children with Turner’s syndrome were treated because of the webbed neck deformity. The age of patients ranged from 9 to 17 years. In the case of all patients, the aim was to distribute the neck skin folds by using Z-plasty in conjunction with a shift to the back of glabrous skin flaps mobilised from the anterolateral surface of the neck. In the case of four operated patients, the folds were completely removed and a correct symmetrical outline of the neck was obtained. One patient was found to have unilateral moderate webbed neck recurrence after about 2 years of treatment. In one case, the correction was insufficient. The performed surgical procedures enabled correction of low hairline only in the lateral parts of the neck. The lower line of the scalp in the central part of the neck has remained unchanged.The lateral approach with a shift of glabrous skin flap to the back, which we performed, allows for effective reduction of the webbed neck, excision of bands of the connective tissue and correction of the low hairline on the side of the neck. Z-plasty enables an adequate extension of scars and improves the contour of the neck.
1 - 3 - 2015
25 - 6 - 2014
3 - 7 - 2015
- 1. Jorde JB, Carem JC , Bamshad MJ , White RL . Genetyka medyczna. Wydawnictwo Czelej, Lublin 2002.
- 2. Gravholt CH : Epidemiological, endocrine and metabolic features in Turner syndrome. Eur J Endocr 2004; 151: 657-87.
- 3. Saenger P, Wikland KA, Conway GS et al.: Recommendations for the diagnosis and management of Turner syndrome. J Clin Endocrinol Metab 2001; 86: 3061-69.
- 4. Łącka K: Zespól Turnera - korelacja między kariotypem i fenotypem. Endokrynol Pol 2005; 6: 986-93.
- 5. Alpman A, Cogulu O, Akgul M et al.: Prenatally diagnosed Turner syndrome and cystic hygroma: incidence and reasons for referrals. Fetal Diagn Ther 2008; 25: 58-61.
- 6. Massa G, Verlinde F, De Schepper J et al.: Trends in age at diagnosis of Turner syndrome. Arch Dis Child 2005; 90: 267-68.
- 7. Gawlik A, Antosz A, Wilk K i wsp.: Opieka medyczna w zespole Turnera -z praktycznego punktu widzenia. Endokrynol Ped 2013; 3: 55-70.
- 8. Reichenberber MA, Goertz O, Lehnhardt M et al.: Surgical correction of pterygium colli. J Ped Surg 2013; 48: 464-69.
- 9. Bekker MN , van den Akker NM , de Mooij YM et al.: Jugular lymphatic maldevelopment in Turner syndrome and trisomy 21: different anomalies leading to nuchal edema. Reprod Sci 2008; 15: 295-304.
- 10. McCauley E, Feuillan P, Kushner H et al.: Psychological development in adolescents with Turner syndrome. J Dev Behav Pediatr 2001; 22: 360-65.
- 11. Chandler FA: Webbed neck (pterygium colli) Am J Dis Child 1937; 53: 798.
- 12. Hikade KR , Bitar GJ , Edgerton MT et al.: Modified Z-plasty repair of webbed neck deformity seen in Turner and Klippel-Feil syndrome. Cleft Palate Craniofacial J 2002; 39: 261-66.
- 13. Menick FJ, Furnas DW , Achauer BM: Lateral cervical advancement flaps for the correction of webbed-neck deformity. Plast Reconstr Surg 1984; 73: 223 29.
- 14. Shearin JC , DeFranzo AJ: Butterfly correction of webbed-neck deformity in Turner’s syndrome. Plast Reconstr Surg 1980; 66: 129-33.
Publication order reference