Dynamic restoration in Möbius and Möbius-like patients

M?bius syndrome is classically characterized by bilateral facial nerve and abducens nerve paralysis in combination with limb defects. In the past 110 years, physicians diagnosed children as having the syndrome on the basis of heterogeneity of symptoms and used the term "M?bius syndrome" or "M?bius-like syndrome" for patients with multiple cranial nerve involvement. The cause and the exact pathogenesis of the syndrome still elude understanding. Genetic work-ups, radiological findings, and data from autopsies differ in their approaches and their findings of the basic causes of M?bius syndrome. In the international literature, about 301 case reports are found scattered through the past century. The appearance of the facial deformity is easy to recognize, because the M?bius patient is impaired in his or her ability to communicate nonverbally. Despite ophthalmologic problems, it is the search for a smile that brings these patients to the reconstructive surgeon. Over the past 100 years, surgical efforts attempted to improve the mask-like appearance by static and dynamic procedures, usually local muscle transpositions. Today, combinations of microsurgical procedures and aesthetic techniques are being used to restore some movement to the expressionless face of these patients by nerve and muscle transplantation. This article discusses the heterogeneity of M?bius syndrome, advocates a new classification system, presents the clinical findings of 42 patients who were seen and examined in consultation, and discusses the surgical management of 20 patients who underwent dynamic restorative microsurgery. Exemplary cases illustrating the preoperative work-up regimen and possible outcomes are reported.     

Facial animation in children with Möbius syndrome after segmental gracilis muscle transplant

M?bius syndrome is a complex congenital anomaly involving multiple cranial nerves, including the abducens (VI) and facial (II) nerves, and often associated with limb anomalies. Muscle transplantation has been used to address the lack of facial animation, lack of lower lip support, and speech difficulties these patients experience. The purpose of this study was to investigate the results of bilateral, segmental gracilis muscle transplantation to the face using the facial vessels for revascularization and the motor nerve to the masseter for reinnervation. The outcome of the two-stage procedure was assessed in 10 consecutive children with M?bius syndrome by direct interview, speech assessment, and oral commissure movement. Preoperative data were collected from direct questioning, viewing of preoperative videotapes, notes from prior medical evaluations, and rehabilitation medicine and speech pathology assessments. All of the patients developed reinnervation and muscle movement. The children who described self-esteem to be an issue preoperatively reported a significant posttransplant improvement. The muscle transplants produced a smile with an average commissure excursion of 1.37 cm. The frequency and severity of drooling and drinking difficulties decreased postoperatively in the seven symptomatic children. Speech difficulties improved in all children. Specifically, of the six children with bilabial incompetence, three received complete correction and three had significant improvement. Despite the length and complexity of these procedures, complications were minimal. Muscle transplantation had positive effects in all problematic areas, with a high degree of patient satisfaction and improvement in drooling, drinking, speech, and facial animation. The surgical technique is described in detail and the advantages over regional muscle transfers are outlined. Segmental gracilis muscle transplantation innervated by the motor nerve to the masseter is an effective method of treating patients with M?bius syndrome.     

Rehabilitation of the face in patients with Down's syndrome

Fifty patients with Down's syndrome underwent surgery for improvement of the facial stigmata. Partial glossectomy, lateral canthoplasty, and nose, cheek, and chin augmentation were the common procedures. With a follow-up of 18 to 24 months, the results were recorded by a multidisciplinary team with similar judgments on the glossectomy, the most satisfactory procedure, and some discrepancy on the other procedures, canthoplasty, and cheek augmentation. There were no infections or extrusion of prostheses, and a rather high incidence of extrusion of prostheses, and a rather high incidence of bone resorption was noted in the mandibular area. The facial changes were satisfactory in the majority of the cases in both medical and nonmedical evaluation and improved self-confidence, especially in the older patients. The satisfactory results here presented advocate certain procedures for attenuation of the Down's syndrome stigmata and improvement of some functions by diminishing the size of the tongue.     

Neurovascular free-muscle transfer for the treatment of established facial paralysis following ablative surgery in the parotid region

Neurovascular free-muscle transfer for facial reanimation was performed as a secondary reconstructive procedure for 45 patients with facial paralysis resulting from ablative surgery in the parotid region. This intervention differs from neurovascular free-muscle transfer for treatment of established facial paralysis resulting from conditions such as congenital dysfunction, unresolved Bell palsy, Hunt syndrome, or intracranial morbidity, with difficulties including selection of recipient vessels and nerves, and requirements for soft-tissue augmentation. This article describes the authors' operative procedure for neurovascular free-muscle transfer after ablative surgery in the parotid region. Gracilis muscle (n = 24) or latissimus dorsi muscle (n = 21) was used for transfer. With gracilis transfer, recipient vessels comprised the superficial temporal vessels in 12 patients and the facial vessels in 12. For latissimus dorsi transfer, recipient vessels comprised the facial vessels in 16 patients and the superior thyroid artery and superior thyroid or internal jugular vein in four. Facial vessels on the contralateral side were used with interpositional graft of radial vessels in the remaining patient with latissimus dorsi transfer. Cross-face nerve grafting was performed before muscle transfer in 22 patients undergoing gracilis transfer. In the remaining two gracilis patients, the ipsilateral facial nerve stump was used as the primary recipient nerve. Dermal fat flap overlying the gracilis muscle was used for cheek augmentation in one patient. In the other 23 patients, only the gracilis muscle was used. With latissimus dorsi transfer, the ipsilateral facial nerve stump was used as the recipient nerve in three patients, and a cross-face nerve graft was selected as the recipient nerve in six. The contralateral facial nerve was selected as the recipient nerve in 12 patients, and a thoracodorsal nerve from the latissimus dorsi muscle segment was crossed through the upper lip to the primary recipient branches. A soft-tissue flap was transferred simultaneously with the latissimus muscle segment in three patients. Contraction of grafted muscle was not observed in two patients with gracilis transfer and in three patients with latissimus dorsi transfer. In one patient with gracilis transfer and one patient with latissimus dorsi transfer, acquired muscle contraction was excessive, resulting in unnatural smile animation. The recipient nerves for both of these patients were the ipsilateral facial nerve stumps, which were dissected by opening the facial nerve canal in the mastoid process. From the standpoint of operative technique, the one-stage transfer for latissimus dorsi muscle appears superior. Namely, a combined soft-tissue flap can provide sufficient augmentation for depression of the parotid region following wide resection. A long vascular stalk of thoracodorsal vessels is also useful for anastomosis, with recipient vessels available after extensive ablation and neck dissection.     

A second gene for autosomal dominant Möbius syndrome is localized to chromosome 10q, in a Dutch family.

Möbius syndrome (MIM 157900) consists of a congenital paresis or paralysis of the VIIth (facial) cranial nerve, frequently accompanied by dysfunction of other cranial nerves. The abducens nerve is typically affected, and often, also, the hypoglossal nerve. In addition, orofacial and limb malformations, defects of the musculoskeletal system, and mental retardation are seen in patients with Möbius syndrome. Most cases are sporadic, but familial recurrence can occur. Different modes of inheritance are suggested by different pedigrees. Genetic heterogeneity of Möbius syndrome has been suggested by cytogenetic studies and linkage analysis. Previously, we identified a locus on chromosome 3q21-22, in a large Dutch family with Möbius syndrome consisting essentially of autosomal dominant asymmetric bilateral facial paresis. Here we report linkage analysis in a second large Dutch family with autosomal dominant inherited facial paresis. After exclusion of >90% of the genome, we identified the locus on the long arm of chromosome 10 in this family, demonstrating genetic heterogeneity of this condition. The reduced penetrance suggests that at least some of the sporadic cases might be familial.     

Results of management of facial palsy with microvascular free-muscle transfer

This paper reports our experience in facial reanimation using free innervated muscle transfer in 69 patients with long-term facial palsy. The majority of patients were treated in two stages with cross-facial nerve graft as the first stage and microvascular muscle transfer at the second stage. The gracilis muscle was used in 62 patients. A system of grading results has been utilized in the long-term evaluation. The overall final result was excellent or good in 51 percent of 47 patients who were available for follow-up. Although the results are not completely satisfactory, they justify the use of this approach to a difficult clinical problem. The results are improving as technical modifications to the procedure have evolved. The gracilis muscle is a reliable free transfer with internal anatomy conductive to use for reanimation of the paralyzed face. This type of transfer, in our experience, has proved superior to nonmicrosurgical methods for treatment of complete and severe incomplete facial palsy. The seventh cranial nerve is used in the innervation of the transferred muscle, the ipsilateral being preferable if available. The authors believe that use of the same cranial nerve is superior to methods that involve other cranial nerves, where spontaneity is often not achieved.     

Evaluation of a new measurement tool for facial paralysis reconstruction

Evaluation of facial movement, including distance and direction, is essential for anyone interested in facial paralysis reconstruction. The authors' goal was to develop a measurement system that is simple, uses commercially available equipment, takes little time, and provides meaningful and accurate measurements. This technique is called the facial reanimation measurement system. It involves placing dots around the patient's mouth and video recording the patient performing maximal effort smiles. Using a video editing program, one frame showing the patient at rest is overlaid with a second frame showing the patient's smile. This overlaid image is imported into Adobe PhotoShop, where measurements are obtained using tools available in the program. Twenty patients were used to test interrater and intrarater reliability of the facial reanimation measurement system. The accuracy of the measurement process was tested by comparing 10 known distances and angles with those obtained using the facial reanimation measurement system. Both intrarater and interrater reliability of the distance and angle measurements are highly accurate, with intraclass correlations greater than 0.9. The facial reanimation measurement system is accurate to within 0.6 mm and 2.0 degrees when compared with a "known" distance and angle. The facial reanimation measurement system has been used to measure smile movements of more than 200 patients and has been demonstrated to be valuable for detecting changes of facial movements over time. This system is simple and economical and only requires 20 minutes to perform. Although the authors demonstrated evaluation of smile movement, the system may be used to evaluate other movements, such as mouth puckering, eye closure, and forehead elevation.     

Improving aesthetic outcomes after alloplastic chin augmentation

A novel approach to increase chin projection with alloplastic material is presented. Key aspects of the technique include the consideration of anthropometric normal values in preoperative assessment and planning, a submental approach with wide subperiosteal exposure of the area to be augmented, the use of two-piece porous polyethylene implants for augmentation, and screw fixation of the implant to the mandible. Screw fixation improves the predictability and precision of reconstruction by preventing implant displacement, by obliterating gaps between the implant and the facial skeleton, and by facilitating final implant contouring. In a series of 46 patients (24 primary and 22 secondary) operated on over a 6-year period, this approach allowed anatomically correct, stable chin contours to be created. Iatrogenic problems with macrogenia, mentalis dysfunction, and soft-tissue distortion resulting from implant migration and capsular contracture have been avoided. There have been no infections. Two patients who had had multiple previous chin operations requested revisional surgery to refine contour.     

Surgical correction of the vertically deficient chin

Surgical correction of the vertically deficient chin has received relatively little attention. This paucity of information is most likely related to the failure to diagnose vertical microgenia and the questionable stability of its surgical correction utilizing autogenous bone grafts. This paper reports on eight patients who have undergone vertical augmentation genioplasty utilizing a transverse symphyseal osteotomy and interpositional implantation of porous, block hydroxyapatite. All patients had preoperative measured decreases in lower face height. Mean vertical lengthening of the chin was 5.3 mm. Seven of the eight patients had class II occlusions and underwent simultaneous sagittal advancement of the chin. Follow-up at a mean time of 11.1 months revealed complete stability of the vertically repositioned symphyseal segment. Mean ratio of the vertical soft to hard tissue augmentation was 0.89:1. There were no instances of operative complications. Vertical facial aesthetics and their application in the evaluation and treatment of patients with vertically deficient lower faces and chins are reviewed.     

Femoral hypoplasia-unusual facies syndrome in a black African infant

The femoral hypoplasia-unusual facies syndrome is a very rare association of femora and facial abnormalities. The most common features include hypoplasia of the femora and a characteristic facies with a short nose, long philtrum, thin upper lip and micrognathia. Maternal diabetes mellitus has been mainly identified as the causal agent. We reported the first case in a black African and discuss prenatal diagnosis and aetiology.     

Brow suspension,a minimally invasive technique in facial rejuvenation

People tend to prefer noninvasive or minimally invasive methods of facial rejuvenation, especially when it involves their face, which is the hallmark of a person's identity and impossible to hide. It is widely known that brow ptosis gives the face a "tired look" and also accentuates deformities of the upper eyelid. Most people who are interested in facial rejuvenation may not accept even a minor surgery, such as an endoscopic surgery. The senior author has developed a minimally invasive method of suspending the brow at a higher position. In this technique, there is neither surgical dissection nor a surgical incision except for four stab incisions and suture insertion, which is why we refer to it as a nonsurgical brow suspension. It is done under local anesthesia, and the brows are fixed in the position that they assume when the patient is supine. In the past 6 years, we performed 387 brow suspensions on 324 female and 63 male patients. The youngest patient was 19 years old, and the oldest was 74 years old. A retrospective chart review was done. These 387 cases were reviewed by comparison of preoperative and postoperative photographs. This approach was not only used for patients who were not interested in surgical rejuvenation but was also combined with lipofilling, laser resurfacing, and/or upper blepharoplasty. This technique is useful for correcting postsurgical brow asymmetry. We present this technique as an adjunct to the established techniques of facial rejuvenation. Despite the high patient acceptance and technical ease, it is not a replacement for the established techniques of facial rejuvenation.     

Distraction osteogenesis in correction of micrognathia accompanying obstructive sleep apnea syndrome

To evaluate the effect of distraction osteogenesis in correction of micrognathia accompanying obstructive sleep apnea syndrome, a total of 28 patients with different severities of obstructive sleep apnea syndrome underwent mandibular distraction osteogenesis. A total of 51 distraction devices were placed for bilateral distraction in 23 patients and for unilateral distraction in five patients. The mean age of patients was 21.2 years (range, 3 to 60 years). Eleven patients had micrognathia accompanying obstructive sleep apnea syndrome secondary to bilateral temporomandibular joint ankylosis, and 10 patients had micrognathia accompanying obstructive sleep apnea syndrome secondary to unilateral temporomandibular joint ankylosis. Three patients had developmental micrognathia accompanying obstructive sleep apnea syndrome. The other four patients had micrognathia and concomitant obstructive sleep apnea syndrome induced by trauma, infection, or tumor resection. Each patient had been evaluated preoperatively and postoperatively with cephalometry and polysomnography. Mandible advancement ranged from 9 to 30 mm (average, 20.4 mm) and was successfully achieved after distraction. Fine new bone formed in the distraction gap when the distraction devices were removed 3 to 4 months after distraction was completed. No infection or other complications occurred in any patients. Complete curative effects were achieved in nine severe, six moderate, and eight mild obstructive sleep apnea syndrome patients after distraction, and the other five patients had been improved to the mild level. After distraction was completed, the posterior airway space was increased on average from 4.6 mm to 12.5 mm and the sella-nasion-point B angle was increased on average from 66 degrees to 75 degrees on cephalometric studies. The polysomnographic examination showed that the apnea hypopnea index was lowered on average from 58.0 to 3.15, and the lowest oxygen saturation was increased on average from 77 percent to 90.3 percent after distraction was completed. The follow-up period was 3 to 61 months (average, 18.1 months). The curative effect was stable and no relapse occurred. Therefore, the authors conclude that mandibular distraction osteogenesis is an effective method for correcting micrognathia accompanying obstructive sleep apnea syndrome. Compared with other current routine surgical procedures, it has many advantages, such as low risk, simple manipulation, high curative rate, low relapse rate, and stable result. It is presently the most effective method for the treatment of this difficult and complicated disorder.     

Dynamic reconstruction of eye closure by muscle transposition or functional muscle transplantation in facial palsy

For patients with facial palsy, lagophthalmus is often a more serious problem than the inability to smile. Dynamic reconstruction of eye closure by muscle transposition or by free functional muscle transplantation offers a good solution for regaining near-normal eye protection without the need for implants. This is the first quantitative study of three-dimensional preoperative and postoperative lid movements in patients treated for facial paralysis. Between February of 1998 and April of 2002, 44 patients were treated for facial palsy, including reconstruction of eye closure. Temporalis muscle transposition to the eye was used in 34 cases, and a regionally differentiated part of a free gracilis muscle transplant after double cross-face nerve grafting was used in 10 cases. Patients' facial movements were documented by a three-dimensional video analysis system preoperatively and 6, 12, 18, and 24 months postoperatively. For this comparative study, only the data of patients with preoperative and 12-month postoperative measurements were included. In the 27 patients with a final result after temporalis muscle transposition for eye closure, the distance between the upper and lower eyelid points during eye closing (as for sleep) was reduced from 10.33 +/- 2.43 mm (mean +/- SD) preoperatively to 5.84 +/- 4.34 mm postoperatively on the paralyzed side, compared with 0.0 +/- 0.0 mm preoperatively and postoperatively on the contralateral healthy side. In the resting position, preoperative values for the paralyzed side changed from 15.11 +/- 1.92 mm preoperatively to 13.46 +/- 1.94 mm postoperatively, compared with 12.17 +/- 2.02 mm preoperatively and 12.05 +/- 1.95 mm postoperatively on the healthy side. In the nine patients with a final result after surgery using a part of the free gracilis muscle transplant reinnervated by a zygomatic branch of the contralateral healthy side through a cross-face nerve graft, eyelid closure changed from 10.21 +/- 2.72 mm to 1.68 +/- 1.35 mm, compared with 13.70 +/- 1.56 mm to 6.63 +/- 1.51 mm preoperatively. The average closure for the healthy side was from 11.20 +/- 3.11 mm to 0.0 +/- 0.0 mm preoperatively and from 12.70 +/- 1.95 mm to 0.0 +/- 0.0 mm postoperatively. In three cases, the resting tonus of the part of the gracilis muscle transplant around the eye had increased to an extent that muscle weakening became necessary. Temporalis muscle transposition and free functional muscle transplantation for reanimation of the eye and mouth at the same time are reliable methods for reconstructing eye closure, with clinically adequate results. Detailed analysis of the resulting facial movements led to an important improvement of the authors' operative techniques within the last few years. Thus, the number of secondary operative corrections could be significantly reduced. These qualitative and quantitative studies of the reconstructed lid movements by three-dimensional video analysis support the authors' clinical concept of temporalis muscle transposition being the first-choice method in adult patients with facial palsy. In children, free muscle transplantation is preferred for eye closure, so as not to interfere with the growth of the face by transposition of a masticatory muscle. In addition, a higher degree of central plasticity in children might be expected.     

Soft-tissue frontal bossing

An unusual case of frontal bossing due to excess subcutaneous fibroadipose tissue in a 38-year-old woman with M?bius syndrome is presented. A relatively simple transcoronal resection of soft tissue resulted in successful elimination of this type of frontal bossing.     

Facial artery in the upper lip and nose: anatomy and a clinical application

Twenty-five facial arteries were examined radiographically in 19 fresh cadavers that had been injected systemically with a lead oxide-gelatin mixture. Major branches of the facial artery in the upper lip and nose were investigated, and the anatomical variations were classified into three types on the basis of the anatomy of the lateral nasal artery, which was determined as an artery running toward the alar base. In 22 cases (88 percent), the facial artery bifurcated into the lateral nasal artery and superior labial artery at the angle of the mouth. In two cases (8 percent), the facial artery became an angular artery after branching off into the superior labial artery and the lateral nasal artery sequentially. In one case (4 percent), the facial artery became an angular artery after branching off into the superior labial artery, and the lateral nasal artery then branched off from the superior labial artery. Branches from the lateral nasal and superior labial arteries were observed stereographically. Vascular anastomoses between those branches were created in the upper lip, columella base, and nasal tip, and an intimate vascular network was formed. With a vascular network in the mucosa of the upper lip, a bilobed upper-lip flap was created for a clinical case with a full-thickness defect of the ala.     

Free proximal gracilis muscle and its skin paddle compound flap transplantation for complex facial paralysis

Gracilis functioning free-muscle transplantation for the correction of pure facial paralysis has been a preferred method used by many reconstructive microsurgeons. However, for complex facial paralysis, the deficits include facial paralysis along with soft-tissue, mucosa, and/or skin defects. No adequate solution has been proposed. Treatment requests in those patients are not only for facial reanimation but also for correction of the defects. Of 161 patients with facial paralysis treated with gracilis functioning free-muscle transplantation from 1986 to 2002, eight patients (5 percent) presented with complex deficits requiring not only facial reanimation but also aesthetic correction of tissue defects. The tissue defects included an intraoral defect created following contracture release (one patient), infra-auricular radiation dermatitis with contour depression (one patient), temporal depression following a temporalis muscle-fascia transfer (one patient), ear deformity (two patients), and infra-auricular atrophic tissue with contour depression (three patients). A compound flap, consisting of a gracilis muscle with its overlying skin paddle separated into two components, was transferred for simultaneous correction of both problems. The blood supply to the gracilis and to the skin paddle originated from the same source vessel and therefore required the anastomosis of only one set of vessels. The versatility of this compound flap allows for a wide arc of rotation of the skin paddle around the muscle. All flaps were transferred successfully without complications. Satisfactory results of facial reanimation were recorded in five patients after all stages were completed. The remaining three patients are undergoing physical therapy and waiting for revision of the skin paddle.     

The surgical management of Melkersson-Rosenthal syndrome.

A triad of facial palsy, orofacial edema, and furrowed tongue constitutes an uncommon condition known as Melkersson-Rosenthal syndrome (MRS). We report on 14 patients with Melkersson-Rosenthal syndrome. Two patients had facial palsy, 12 had orofacial edema, and 1 patient had a furrowed tongue. Nine patients were treated medically. Intralesional steroid therapy had a 75 percent recurrence rate. Systemic steroid therapy resulted in remission in two of three patients. Surgical excision and reconstruction were carried out in five patients with chronic lip or eyelid edema. This provided relief in all cases. The etiology, clinical presentation, and histologic features are discussed. Three illustrative cases are presented. An algorithm is provided that guides the surgeon with regard to both the medical and surgical treatment of the patient with Melkersson-Rosenthal syndrome.