San Basilio María, Lorca-García Concepción, de la Torre Manuel, Terrados Rocío, Berenguer Beatriz
Department of Pediatric Surgery, La Paz Children's Hospital, Madrid, Spain.
Deppartment of Pediatric Plastic Surgery, Gregorio Marañón Children's Hospital, Madrid, Spain.
Indian J Plast Surg. 2024 Nov 8;58(2):110-114. doi: 10.1055/s-0044-1791744. eCollection 2025 Apr.
Pterygium colli is a rare congenital disorder characterized by excessive skin in the lateral and posterior neck, often associated with genetic syndromes such as Turner's syndrome or Klippel-Feil syndrome. Severe presentations present special challenges for surgeons since its diagnosis is not always evident and its treatment often results in important scaring. Surgical correction of the typical cases mostly involves a lateral approach, but in this "special" case, we used a double, posterior and lateral approach. A 15-month-old girl with prenatal diagnosis of a cystic lesion in the posterior neck presented with massive neck webbing at birth. Genetic studies ruled out chromosomal disorders and mutations. Surgical correction involved a modified posterior approach combined with a lateral approach 1 year later. The cervical tissue redundancy was successfully reduced achieving a good aesthetic outcome with very acceptable scars. At 1 year of follow-up, the patient continues with good neck appearance and mobility. The goal of surgical treatment of pterygium colli is achieving a harmonious neck contour with good function and minimal scarring while minimizing complications and sequelae. No consensus exists on the optimal approach for its correction, emphasizing the need for individualized treatment considering patient-specific characteristics, particularly in the severe cases.
蹼颈是一种罕见的先天性疾病，其特征为颈部外侧和后部皮肤过多，常与特纳综合征或克-费综合征等遗传综合征相关。严重病例给外科医生带来特殊挑战，因为其诊断并不总是很明显，而且治疗往往会导致明显的瘢痕形成。典型病例的手术矫正大多采用外侧入路，但在这个“特殊”病例中，我们采用了后侧和外侧联合入路。一名在产前诊断为颈部后部囊性病变的15个月大女孩出生时出现巨大的颈部蹼状畸形。基因研究排除了染色体疾病和突变。1年后采用改良后侧入路联合外侧入路进行手术矫正。成功减少了颈部组织冗余，获得了良好的美学效果，瘢痕也非常美观。随访1年时，患者颈部外观和活动度良好。蹼颈手术治疗的目标是实现颈部轮廓协调，功能良好，瘢痕最小，同时尽量减少并发症和后遗症。对于其矫正的最佳方法尚无共识，这强调了需要根据患者的具体特征进行个体化治疗，尤其是在严重病例中。
