Combined Surgical Treatments Of Facial Contour Deformities Resulting From Severe Progressive Hemifacial Atrophy

Progressive hemifacial atrophy(PHA),also known as Parry-Romberg syndrome,was first described by Parry in 1825 and later by Romberg in 1846.It's a rare disorder characterized by a slow and progressive hemifacial atrophy involving the skin,subcutaneous tissue,muscles,and osteocartilaginous framework later.To date,the etiology of PHA is still unknown.The principal goal of plastic surgical treatment is to restore a harmonious and symmetrical appearance to facial deformities.A number of procedures have been developed to reconstruct by using autologous fat grafts,dermal grafts,bone and cartilage grafts,distraction osteogenesis,tissue expansion,myocutaneous flaps.Later, microvascular free flap was widely used,including the deltopectoral, anterolateral thigh,groin,dermal-fat,radial forearm,latissimus dorsi, scapular and parascapular flaps,omentum free flap,the transverse rectus abdominis muscle(TRAM) flap,the deep inferior epigastric perforator(DIEP) flap.Free flaps have been introduced as the gold standard for correcting moderate and severe deformities.We prefer anterolateral thigh adipofascial flap,because it has the same advantages as anterolateral thigh flap.With minimal donorsite morbidity,it can provide abundant tissue with reliable blood supply.Other methods,such as bone augmenation,orthognathic surgery,or genioplast was performed at least 6 months after the first procedure.Objective To evaluate the outcome of combined treatments of facial contour deformities resulting from progressive hemifacial atrophy.Material and methods From 2004.3～2009.3,there were 23 patients with moderate or severe progressive hemifacial atrophy. The patients aged from 13～32 years old with a median 21.3 years. The duration of the disease was from 4 to 15 year with a median of 7.4 years between the onset and the first consultation in the hospital.there were male and 17 female patients. Among them,9 patients received free anterolateral thigh adipofascial flap while 1 patient received latissimus dorsi flap at the first stage;3 patients received orthognathic surgeries, such as Le Fort I osteotomy, mandibular anterior subapical osteotomy, mandibular distration osteogenesis;18 patients received other procedures to change the facial contour,such as separated the outer mandibular cortex while using contralateral mandibular cortex, malar expansion with artificial bone, correction of prominent malar complex by L-type osteotomy, bone augmentation by using Medpor, genioplasty; 13 patients received dermal grafting;8 patients received double "z" plasty, such as skin flap grafting with musculus orbicularis oculipedicle, pedicled orbicularis musculocutaneous flap;Minor deformities were performed at the third stage or even Iater.All the cases were followed up postoperatively from 6 months to 8 years. The longitudinal outcome of facial contour were observed and assessed postoperatively.Results 10 flaps survived with one flap partially survied; isolation of eyelid from eyeball occurred in 1 patient, which was corrected by debulking and facial sling;1 patient received retroauricular full-thickness skin graft for correction of lower eyelid ectropion;2 patient received facial sling to correct sagging of the flap;5 patients received debulking procedure 6 months postoperatively; femoral seroma occurred in 1 patient while cured by change of dressing; hypertrophic scar occurred in 1 patients because of poor healing; partial auricular composite graft necrosis occurred in 1 patient. All patients received satisfactory results. No obvious complications and malformation were found in the donor sites.Conclusions Free flaps, especially anterolateral thigh adipofascial flap,is the best choice for patients with moderate and severe deformities.Some patients with moderate deformities need reconstructive surgery of maxillofacial bones at the first stage.Combined and individualized treatments could provide a better cosmetically good result.