Webbed Neck Deformity Research Articles

Improving Functional and Aesthetic Outcomes in Syndromic Patients With Webbed Neck Deformity

Webbed neck deformity (WND) can have significant functional and psychosocial impact on the developing child. Surgical correction can be challenging depending on the extent of the deformity, and patients often also have low posterior hairlines requiring simultaneous correction. Current surgical techniques include various methods of single-stage radical excision that often result in visible scar burden and residual deformity. There is currently no general consensus of which technique provides the best outcomes. A modified approach to WND was designed by the senior author aimed to decrease scar burden. Endoscopic-assisted fasciectomy was performed with simultaneous posterior hairline reconstruction with local tissue rearrangement camouflaged within the hair-bearing scalp. Staged surgical correction was planned rather than correction in a single operation. A retrospective review was performed to evaluate all patients who underwent this approach over a 2-year period. Two patients underwent the modified approach, a 17-year-old female with Noonan syndrome and a 2-year-old female with Turner syndrome. Both patients showed postoperative improvement in range of motion, contour of the jaw and neckline, and posterior hairline definition. Patients were found to have decreased scar burden compared with traditional techniques. A staged, combination approach of endoscopic-assisted fasciectomy and strategic local tissue reconstruction of the posterior hairline to correct WND achieves good functional and aesthetic results and good patient satisfaction. This modification should be considered when managing WND.

Surgical Correction of a Webbed-Neck Deformity in Turner'S Syndrome.

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.

M to T Rearrangement: An Approach to Correct Webbed Neck Deformity

For the Noonan syndrome patient, the most concerning physical defect is often congenital webbing of the neck or pterygium colli. We present a patient with pterygium colli and a low and laterally displaced nuchal hairline. Since its description, various surgical approaches have been implemented to correct the deformity. Previously reported posterior and lateral approaches have notable disadvantages with regard to hairline displacement and recurrence. In order to address these disadvantages, a new surgical technique was used on this patient. We have termed this technique an M to T rearrangement. 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. This prevents the recurrence seen with the use of posterior techniques. 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. The excision of excess skin along with the zig-zag closure also prevents postoperative scar contraction and recurrence. An important effect of this technique is the prevention of anterior displacement of hair bearing skin. M to T rearrangement is an effective technique for the correction of webbed neck deformities seen in Noonan and Turner syndromes.

Noonan syndrome and correction of the webbed neck

We report on a 16-year-old boy with Noonan syndrome who had short stature, hypertelorism, mild hearing loss, webbed neck, pectus deformities, hypertrophic cardiomyopathy, low posterior hairline, redundant skin, café au lait spot, curled hairs, foetal pads, and undescended testes. The father and a sister of the boy were also found with mild webbed neck, being possibly mildly affected individuals. The significant webbed-neck deformity of the boy was completely corrected by subcutaneous resection of the proximal half of the hypertrophic fascia bands, through small incisions hidden within the hairline. The advantage of this procedure is simple, safe and effective, with no visible scar on the surface of the neck.

Correction of the Webbed Neck Deformity in Noonan Syndrome-A Case Report and Review of Literature

The Noonan syndrome is a genetic disorder resulting in developmental anomaly of multiple parts of the body. Many features of the syndrome are reminiscent of Turner syndrome, except the later is caused by 45×0 chromosome anomaly, whereas the former has normal chromosome number. Accordingly, the Noonan syndrome is also called ”Turner phenotype”. Among the multiple anomalies of Noonan syndrome, the webbed neck deformity is the most prominent one. The deformity is characterized by a subcutaneous band of thickened fascia in the lateral aspects of neck and a low-neck hairline. The goal of surgical management is to correct the low-lying hairline and to restore the normal contour of the neck. The issue of abolishing the webbing band of the neck without residual obvious scarring is a challenge to the surgeons. We report a female patient of Noonan syndrome and describe the technique of correction. We also discuss a number of major procedures described in the literatures.

Modified Z-Plasty Repair of Webbed Neck Deformity Seen in Turner and Klippel-Feil Syndrome

Abstract Objective: Webbed neck deformity exists in many syndromes including Turner's or Klippel-Feil syndrome. Multiple problems are encountered with existing techniques to correct a webbed neck deformity. In Turner's syndrome, a subcutaneous band of thickened fascia and a low neck hairline present a challenge to the surgeon when designing a repair. The authors propose the following new technique that addresses both issues. Material and Methods: Five patients with webbed neck underwent this new procedure. A Z-plasty is performed with the midline arm down the length of the web. The subcutaneous fibrous band is excised, the shortened trapezius is released, and the hair-bearing flap is excised. The anterior flap is rotated and advanced to join its mate flap from the contralateral neck at the posterior midline. A resultant dog-ear near the acromion is corrected with an additional Z-plasty. Results: In all five patients, the functional and aesthetic results were very satisfactory to both patient and surgeon. ...

Modified Z-plasty repair of webbed neck deformity seen in Turner and Klippel-Feil syndrome.

Webbed neck deformity exists in many syndromes including Turner's or Klippel-Feil syndrome. Multiple problems are encountered with existing techniques to correct a webbed neck deformity. In Turner's syndrome, a subcutaneous band of thickened fascia and a low neck hairline present a challenge to the surgeon when designing a repair. The authors propose the following new technique that addresses both issues. Five patients with webbed neck underwent this new procedure. A Z-plasty is performed with the midline arm down the length of the web. The subcutaneous fibrous band is excised, the shortened trapezius is released, and the hair-bearing flap is excised. The anterior flap is rotated and advanced to join its mate flap from the contralateral neck at the posterior midline. A resultant dog-ear near the acromion is corrected with an additional Z-plasty. In all five patients, the functional and aesthetic results were very satisfactory to both patient and surgeon. An 11-year follow-up is presented with excellent correction of the webbing. Both limited range of motion and the cosmetic deformity are addressed by this technique. The results obtained by using the simplified modified Z-plasty technique for repair of webbed neck deformity are very satisfactory. We propose the use of this technique for correction of webbed neck deformities whenever the posterior surface of the neck web contains a significant amount of hair.

Modified lateral cervical advancement flaps for the correction of webbed neck deformity

Modified lateral cervical advancement flaps for the correction of webbed neck deformity

Ｎｏｏｎａｎ症候群を合併した肩関節機能不全に対する治療経験

We report a case of insufficiency of bilateral shoulder function associated with Noonan's syndrome. Noonan's syndrome is characterized by multiple congenital abonormalities similar to those of Turner's syndrome and by normal karyotype.A 4-year-old male infant was presented to Nagasaki University Hospital for deformities around neck and shoulders. These deformities had been noted since birth but no treatment had been sought. His father was noticed to have similar deformities of neck and both shoulders but is not alive now.Physical examination revealed obvious superior migration of both scapulae, and active abduction of bilateral shoulders were only 80°. And he also had webbed neck, hypertelorism, antimongolid slant, low hair line, low set ears, high arched palate and shield like chest. Though there were no signs of cardiac abnormalities. Chromosomal analysis demonstrated the normal male karyotype; Noonan syndrome was compatible. Roentgenograms revealed bilateral elevated scapulae with each shape of equilateral triangle and no omovertebral bone. Abduction of bilateral scaplae on A-P view were under 6° in active aboduction of shoulders.We resected scaplae 1cm in width along the vertebral margin, released rhomboid muscles and lower part of trapezius muscles and corrected the webbed neck deformity in the first and second operation. Although the mobilitiy of scaplae increased, active shoulder abduction decreased to 70° after these operation. Defect of scapular abductor muscles was suspected. Then we transferred pectoralis major muscles to both scaplae. Abduction of the right scapula was improved to 28° after 2 years and abduction of right shoulder increased to 150°. Abduction of left scapula was 4° after 3 months. The obtainment of scapular abducting power and stability of scapulae may have contributed the improvement of shoulder abduction.

Treatment of Webbed Neck in Turnerʼs Syndrome with Tissue Expansion

We describe a successful approach to the management of the webbed neck deformity in Turner's syndrome using skin expansion. This case report demonstrates the advantages of this method over previously described techniques.

Correction of pterygium colli deformity in Ullrich-Noonan Syndrome

A case of Ullrich-Noonan syndrome with the typical marked webbed neck deformity is presented. The posterior approach is preferred for the correction of the webbed neck deformity. Bilaterally localized excess skin and the underlying fibrotic bands were excised in an elliptical shape. The webbed neck was corrected by a large classical z-plasty. After the transposition of z-plasty flaps, the final appearance of the neck was cosmetically satisfactory.

Correction of webbed neck deformity in Turnerʼs syndrome

Correction of webbed neck deformity in Turnerʼs syndrome

Correction of webbed-neck deformity in Turner's syndrome

A case of Turner's syndrome with the typical marked webbing of the neck is presented. A posterior approach is preferred for correction of this webbed neck deformity. A butterfly shaped portion of excess skin is excised and the margins of the defect mobilised as four flaps. The flaps are sutured with a very short midline vertical component, resulting in an almost X-shaped scar. The first procedure resulted in only a partial correction of the deformity and an excess of hypertrophic scar tissue. This was treated with a repeat of the procedure but the midline wounds was repaired with mulitple small Z-plasties. The final appearance of the neck was satisfactory in the lateral profile, but there was still some excess tissue posteriorly.

Lateral cervical advancement flaps for the correction of webbed-neck deformity.

In the webbed-neck deformity, a horizontal excess of cervical skin creates bilateral and often asymmetrical skin webs from the mastoid to the acromion. Hair extends laterally to the free edge on the posterior web surface, creating a wide nuchal hairline. A technique of correction is presented. Through an incision along or within the hairline, the glabrous anterior web surface is undermined with the platysma muscle into the anterior cervical triangle until posterosuperior traction will obliterate the web. The posterior hair-bearing web surface is also elevated, and an excess of scalp is excised anterior to the new hairline position determined by the surgeon. The anterior glabrous flap is advanced posteriorly to resurface the scalp defect and recreate a normal neck contour and symmetrical hairline. A Szymanowski triangle of scalp is excised to equalize wound margins creating two "lazy" Y incisions that join in the scalp midline on completion of the opposite neck web. All scars lie within or along the hairline or extend onto the posterolateral shoulder. The method allows precise control of bilateral neck contour and hairline position without intraoperative repositioning and avoids scars on the exposed anterolateral cervical surface. There has been no recurrence of the neck deformity after 2 years.

Lateral Cervical Advancement Flaps for the Correction of Webbed-Neck Deformity

Lateral Cervical Advancement Flaps for the Correction of Webbed-Neck Deformity

Butterfly correction of webbed-neck deformity in Turner's syndrome: J. C. Shearin and A. J. DeFranzo. Plast Reconstr Surg 66:129–133, (July), 1980

Butterfly correction of webbed-neck deformity in Turner's syndrome: J. C. Shearin and A. J. DeFranzo. Plast Reconstr Surg 66:129–133, (July), 1980