Pterygium colli is mainly observed in association with 3 major malformative syndromes: the Turner, Klippel–Feil, and Noonan syndromes.
- Turner syndrome is a 45X0 karyotype abnormality; the classic triad of manifestations was first described in 1938 as pterygium colli, cubitus valgus, and infantilism syndrome.
- Klippel–Feil syndrome, described in 1912, consists of a cervical vertebral deformity resulting in a short neck, combined with a cleft lip.
- Noonan syndrome mimics Turner syndrome but occurs in boys with normal karyotypes.
Pterygium colli can also be associated with Freeman–Sheldon syndrome (craniocarpotarsal dysplasia), fetal alcohol syndrome, and trisomies 18 and 21.7
The etiopathogenesis of this condition remains unknown, but it could result from in utero cervical lymphatic developmental defects2 or in utero cystic hygroma of the posterior surface of the neck, which would create 2 excess lateral skin folds during resorption.9 In 1984, Clark3 reported that pterygium colli was associated with heart malformation in patients with Turner syndrome. He argued that increased lymphatic pressure and jugular lymphatic sac obstruction resulting in pterygium colli would distend the thoracic duct, compressing the ascending aorta. Blood flow would thus be altered, favoring aortic coarctation and repercussion in the left heart.
Heredity must be taken into account, as Leandris and Ricbourg7 described 3 cases of pterygium colli in the same family (a mother and 2 sons), although they did not specify the associated syndrome. Graham and Smith10 also described the entity of “dominantly inherited pterygium colli.”
Patients with Turner syndrome rarely have mental retardation and generally seek consultation due to the negative cosmetic and social effects of pterygium colli. The literature on the surgical correction of pterygium colli is poor; most authors have described the use of a given technique in 1 or 2 cases, with little follow-up data. The achievement of a harmonious cervical profile and normal hairline implantation are the 2 main objectives of management, but analysis of the results presented in the literature is complex, in part, because the prominence of the malformation can vary.10
For plastic surgeons, the ideal technique provides the best cosmetic results with the fewest complications and recurrences and, especially in the cases described here, the least scarring.
We oppose lateral techniques for pterygium colli management7,8,11 and favor posterior techniques,7,9,10,12–17 following Thomson et al.1 Techniques involving balloon skin expansion have also been described and seem to have yielded good results in isolated cases,7,18,19 but we do not use them. Expansion could aid posterior techniques by providing vertical laxity, which is especially lacking in pterygium colli. Expansion has been found to provide superior skin lifting while minimizing the risk of recurrence. However, it requires several sessions, which pediatric patients may find difficult to tolerate.
Lateral techniques are mainly Z-plasties or modified Z-plasties, which provide good fold reduction but less efficient hairline implantation, with a risk of unattractive anterior hair transposition. These techniques also cause significant scarring, which is readily visible on the lateral surfaces of the neck.
Posterior techniques also provide good fold reduction, but the sutures are often tightened, which could favor recurrence or scar enlargement and hypertrophic scarring.20 Nevertheless, they enable the complete resection of abnormal hairline implantation and the scars can be hidden in the hair.
We prefer posterior techniques because they can provide a harmonious cervical profile. The “posterior cervical lift” is thus named because it involves posterior retention of the superficial lamina of the cervical fascia, such as in cervicofacial rhytidectomy with superficial musculoaponeurotic system suspension. Solid, reliable fixation of the paranuchal connective fibers of the trapezius is possible. All published descriptions of pterygium colli management have focused on how to lift or resect the skin. We suggest a different approach using the superficial lamina of the cervical fascia, with deep suturing that reduces the tightness of the skin sutures.
Another important implication of the surgical management of this condition is the danger to the spinal nerve posed by complete and deep resection of the fold, with removal of fibers from the sternocleidomastoid muscle. Our technique consistently remains above the superficial lamina of the cervical fascia, and so poses no danger to the spinal nerve.
To facilitate the surgical procedure, we systematically operated on young patients (mean age, 10 y) who had laxer and more plastic skin. This surgery should be performed in preschool-aged patients to limit questions from other children; however, patients usually request cosmetic correction in early adolescence if their parents had not requested it earlier.
Recurrence is possible, but we found only 1 case described by Posso et al.9 A 6-year-old girl initially benefited from surgery using a “butterfly” technique, but the result was unsatisfying at 2 years postoperatively, requiring revision surgery with a Z-plasty.
The management of pterygium colli remains a surgical challenge. Through this clinical series, we have described the “posterior cervical lift,” an innovative surgical technique for the management of pterygium colli. It provided satisfactory cosmetic results and stability over time. Patients assessed the outcomes as excellent. We favor posterior techniques, which we believe provide the best compromise between fold reduction and risk of recurrence.
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© 2013 American Society of Plastic Surgeons
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