Auricular reconstruction with modified expanded two-flap method in Goldenhar Syndrome: 7-Year experiences

Abstract

Background

Goldenhar Syndrome (GS) is a systematic polymalformational disease characterized by microtia, hemifacial microsomy, ocular anomalies, abnormal vertebra and other deformities. These congenital malformations brought severe physical and mental stress to the patients. Auricular reconstruction has always been the primary appeal of the patients and their families to achieve a better facial balance and harmony. However, multiple deformities made it a great challenge to perform this technique. In this study, we reported a modified expanded two-flap method of auricular reconstruction, which is more applicable for patients with Goldenhar Syndrome.

Methods

Between January of 2012 and September of 2019, 69 cases of Goldenhar Syndrome underwent auricular reconstruction with the modified expanded two-flap technique. Auricular reconstruction was performed in three surgical stages. In the first stage, a 50 ml kidney-shaped tissue expander was embedded in mastoid region and the retroauricular skin expanded. In the second stage, the autologous rib cartilage was harvested and the ear framework fabricated. The expander was further removed, and then enveloped by the expanded flap, retroauricular fascial flap and a free skin graft. In the third stage, the reconstructed ear was further trimmed, including lobule transposition and tragus reconstruction.

Results

The patients were followed for 6 months to 7 years. There were 63 cases (91.3%) responded satisfied outcomes in size, shape, position and bilateral symmetry. One case suffered expander leakage and tissue expansion accomplished after the expander was replaced. Hematoma was occurred in four cases and removed to complete the reconstruction. Complications of cartilage exposure were observed in one case.

Conclusion

Modified expanded two-flap method is effective and safe in auricular reconstruction for patients of Goldenhar syndrome with long term satisfying results.

Level of Evidence IV.

Introduction

Goldenhar syndrome, also called as Oculo-auriculo-vertebral (OAV) dysplasia, which was first described by Dr. Goldenhar in 1952 [1]. This congenital deformity is arising from the defects of the first and second branchial arches [2]. Exact etiology of this defect is not yet well established now. The incidence of the syndrome was reported to be 1:35,000–1:56,00 and often affects unilateral organs [3]. Goldenhar syndrome encompassed a series of congenital malformations with varying in severity [4,5]. Typical dysplasia of this syndrome included hemifacial microsomia, microtia, preauricular skin tags, ocular deformities, epibulbar dermoids and vertebral column anomalies [[1], [2], [3], [4], [5], [6], [7]]. Cardiovascular and pulmonary hypoplasias were also been reported [1,[8], [9], [10]]. Rib and rib cartilages deformities were always considered to be the associated symptoms of vertebral anomalies in Goldenhar syndrome [[5], [6], [7]] (see Fig. 1, Fig. 2, Fig. 3, Fig. 4, Fig. 5).

Congenital malformations of Goldenhar syndrome brought severe physical and mental stress to the patients. Auricular reconstruction means great significance for the psychological and physiological development of the patients with Goldenhar syndrome. Reconstruct an external ear and achieve a better facial balance and harmony had always been the primary appeal of the patients who resort to the doctors. However, multiple deformities made it a great challenge and increased difficulty to reconstruct the auricle in Goldenhar syndrome. There are several clinical characteristics of patients with Goldenhar syndrome in the application of auricular reconstruction, which are as follows: i) severe facial asymmetry and depressed temporal bone, ii) tight retroauricular skin and stunted retroauricular fascia, iii) skeleton asymmetry and neck movement restriction due to vertebral malformations, iv) low hairline of the affected side, v) low and anterior site of the remnant ear. Due to these features, a particularly modified auricular reconstruction technique is essential for patients with Goldenhar syndrome.

Several surgical techniques of auricular reconstruction have been reported in the literature and autologous rib cartilage is the consensus best choice for ear framework fabrication. The expanded two-flap method was first described by Neumann in 1957. Tissue expansion provided well-vascularized skin coverage with appropriate thickness and area. In this study, the authors introduced a particularly modified expanded two-flap method for auricular reconstruction in details for patients with Goldenhar syndrome.