Congenital Webbing of Neck
Conditions
Keywords
Congenital abnormalities, Webbed neck, Surgical technique, Turner syndrome, Fetal cystic hygroma, Nuchal fold
Brief summary
The webbed neck is one of the conspicuous deformities of Turner syndrome that presents a surgical challenge. Despite the availability of surgical techniques, surgical outcomes are not always acceptable and recurrence occurs. Mehri Turki performed a surgical technique based on a latéral cervical approach allowing direct visual control to manage the fibrotic band. It provides a high level of visual control so that vulnerable anatomic structures are easily protected.
Detailed description
Five girls between 17 and 19 years old, with Turner syndrome, had a webbed neck (pterygium colli) with low and laterally displaced nuchal hairline. The surgical technique aims to correct neck contour and hairline placement while concealing cervical scars. Thus, Preoperative drawing delimitated the harmful triangular hairy skin next to the fibrous band extending from the mastoid to the acromion. Besides, the predefined design drawings provide the exact placement of the future hairline. Choosing a prone position for bilateral and symmetrical repair, the surgical technique was as follows: The incision was made at the junction of the hairless skin and the hairy skin from the mastoid up to the lower end of the webbing skin, in front of the acromion and completed by Z plasty to avoid scar contracture. The subcutaneous skin was undermined in the anterolateral direction, exposing the fibrous fascial band which must be excised to prevent recurrence. the harmful skin having a triangular shape was excised considering the future hairline. Skin closure was done after the superior traction of the posterior cervical advancement.
Interventions
The incision was made at the junction of the hairless skin and the hairy skin from the mastoid up to the acromion. A subcutaneous undermining allows direct visual control to excise the fibrotic band. Then the hairy skin with a triangular shape was excised. The closure of the defect was done after undermining an anterior cervical flap which was translated in a posterosuperior direction. Z plasty was performed in front of the acromion to prevent scar contracture. Surgical scars are concealed in the hairline which was well placed. The same procedure is done at the same surgical field on the other side to achieve perfect symmetry.
Sponsors
Study design
Intervention model description
Girls with congenital webbed neck deformity underwent the same surgical technique. Outcomes were analysed with long-term follow-up.
Eligibility
Inclusion criteria
* webbed neck with abnormal hairline
Exclusion criteria
* Surgical contraindication for any health concern * Puberty age
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Neck contour | Just at the postoperative outcome up to 24 months of follow-up | Number of patient obtaining a normal neck contour without lateral fold |
| Posterior hairline placement | Just at the postoperative outcome up to 24 months of follow-up | Number of patients with posterior hairline assent |
| Scars placement | Just at the postoperative outcome up to 24 months of follow-up | Number of patients with a placement of scars at the level of the hairline |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Scars quality | from 8 months up to 24 months | Scars evaluation according to Vancoover scare scale (VSS) for all patients The quality of post-operative scar was evaluated according to VSS as follows: combinations of 4 criteria (1) height (Normal/ 0; 0-2mm/ 1; 2-5mm/ 2 ; \> 5mm / 3) and (2) pliability (Normal 0/ Supple 1/ Yielding 2/ Firm :3 Banding/4, Contracture/5); (3) Pigmentation (Normal : 0/ Hypopigmentation/ 1 Hyperpigmentation/ 2), and (4) Vascularity (Normal/0 Pink/ 1 Red/ 2 Purple/3). scars quality was judged as follows : Excellent result VSS \<1 / Acceptable 1\< VSS \<5 ; Poor scar \>5 |
| Recurrece of the fold | 24 months | Number of patients in whom the fold of the neck recurred |