gms | German Medical Science

Turner syndrome affects about 1/2500 female births worldwide. This syndrome is caused by complete or partial loss of one X chromosome. In some girls, a number of features are apparent such as: webbed neck or pterygium colli, low hairline at the back of the head, lymphedema of hands and feet, broad chest with widely spaced nipples, heart defects, kidney problems, ovarian dysgenesis, and poor growth [1].

Because of the aesthetic and psychological harm, these girls ask for surgical correction of the webbed neck at school age. Many techniques have been described to correct this deformity. We expose our surgical method with a literature review.

Five girls between 17 and 19 years old came for surgical correction of their pterygium colli with low and laterally displaced nuchal hairline. For all these girls, the surgical method was the same. They underwent it under general anesthesia. The girl was placed in a prone position for bilateral repair (Figure 1 [Fig. 1]). The incision was made at the junction of the hairless skin and the hairy skin from the mastoid until the lower ending of the webbing skin. An undermining of the subcutaneous skin was done on the antero-lateral direction exposing the fibrous fascial band which must be excised to prevent recurrence. Obtaining the hedged cutaneous surface, the harmful skin having a triangular shape was excised with respect to the future hairline. Skin closure was done after postero-superior translation of the lateral cervical flap associated to a single Z-plasty on the ending of the incision regarding to the acromion (Figure 2 [Fig. 2]). The follow-up was done with an average of 24 months. The hyperthrophic scars observed in three patients were treated by corticotherapy injection and silicone sheet application. No functional deficit was found and the range of motion of the head and shoulders was completely free in all these girls. In addition, the morphological appearance of the neck and the placement of the hairline were correct allowing these girls a better social integration (Figure 3 [Fig. 3], Figure 4 [Fig. 4]).

The first description of the webbed neck was reported by Kobylinski in 1883 [2] and the name “pterygium colli” was coined by Funke in 1902 [3]. It corresponds to bilateral dermal webbing that can be identified and rolled between the fingers. It extends from the mastoid to the acromion. The excess of the skin is associated with a thickening of the superficial cervical fascia sometimes entangled by fibers of the platysma muscle. The etiopathogeny of this deformity is discussed. Some authors attribute it to the spontaneous resolution of a cervical cystic hygroma before 16 weeks gestation. This hygroma is secondary to lymphatic malformation, particularly dilated jugular lymphatic sacs that result from a blockage in the venous drainage system. Von Kaisenberg revealed the hypoplasia of lymph vessels in the superficial dermis by using immunohistochemical markers [4].

Girls with Turner syndrome present a hairy posterior face of the pterygium colli continued by a low hairline next to the sixth and seventh cervical vertebrae. The aim of the surgical treatment is to create a normal neck contour with a symmetrical postero-lateral hairline by excising a triangular cutaneous band as done in our patients [5].

Chandler in 1937 was the first to describe the surgical correction of pterygium colli using Z-plasties. However, the skin of the anterior basicervical region becomes hairy by the transposition of the posterior skin of the pterygium colli [6].

Foucar (1948) described a posterior approach. Through a midline incision in T, two lateral flaps are detached and then translated into a posterior-superior direction to erase cervical webbing. The excess of scalp is excised [7]. To avoid visible scars, Shearin and Defranzo [8] used a posterior butterfly incision in which the loose skin and subcutaneous tissue was pulled to a midpoint on the back of the neck. The fibrous band was never excised and recurrences were usually seen [9]. Rather than the posterior approach, Menick in 1984 had the idea of using a lateral approach to create a cervical advancement flap and closure with a shape of two “Y”s, without associated Z-plasty [10].

Niranjian [11] and Miller [12] conducted skin expansion for patients respectively 16 and 30 years old. At this age, the biomechanical properties of skin differ from those in children.

S. Murthy and M. McGraw recently described a lateral approach called M to T rearrangement. By this technique, the trapezial fascial web is directly exposed and completely excised [13].

We believe that the posterior approach doesn’t allow for complete excision of the trapezial fascial web giving more recurrence. In addition, this approach makes it difficult to control the anatomical components, particularly the external jugular vein, cervical plexus, VII and XI nerve, unlike the lateral approach.

The author declares that he has no competing interests.