Möbius 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 "Möbius syndrome" or "Möbius-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 Möbius 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 Möbius 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 Möbius 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. (Plast. Reconstr. Surg. 111: 40, 2003.)
Background: The aim of this study was to present the authors' experience with facial reanimation in adult patients following tumor extirpation and to analyze the functional outcomes. Methods: From 1978 to 2006, 60 adult patients underwent facial reanimation for facial paralysis following tumor extirpation. There was one patient with bilateral facial paralysis. Thus, evaluation was carried out in 61 hemifaces. Three independent assessors evaluated the preoperative and postoperative videos using the Terzis grading scale for eye closure, smile, depressor, and overall aesthetic and functional outcomes. Preoperative and postoperative electromyographic interpretations and the effect of demographic variables were also evaluated. Results: There was significant improvement regarding preoperative versus postoperative outcomes for overall aesthesis and function. Good and excellent results were observed in 72.14 percent of the hemifaces ( n = 44). The difference between preoperative and postoperative electromyographic results was of statistical significance ( p < 0.0001) for each target reinnervated. Better results were observed in younger patients (≤35 years) ( p = 0.023) and in early cases ( p = 0.019). Conclusions: The results of this present series illustrate that age and denervation time correlate with the final functional outcome. Cross-facial nerve grafts should ideally be used in patients with denervation time less than 6 months or more than 2 years combined with muscle transfers. In patients with a denervation time between 6 months and 2 years, the use of the babysitter procedure can yield a superior outcome. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.
Background: The purpose of this study was to evaluate retrospectively the patients who had undergone radial nerve reconstruction. Methods: The medical charts of 35 patients with radial nerve lesions and 13 patients with superficial radial nerve lesions who underwent surgical exploration and repair in the authors' center were reviewed. The outcomes were analyzed in relation to various factors, such as age at injury, denervation time, level of injury, length of nerve graft, and type of reconstruction. Results: With regard to the radial nerve, good and excellent motor results were seen in 27 of 35 cases (77.14 percent). Postoperative lateral pinch and grip strength of the affected side were equal to 75.49 percent and 76.42 percent of those of the nonaffected side, respectively. Patient age, level of injury, denervation time, associated nerve injuries, length of nerve graft, and type of surgical reconstruction significantly influenced the functional outcome. For the superficial radial nerve, good and excellent sensory results were seen in 10 of 13 cases (76.92 percent). Postoperatively, pain scores were graded as excellent in 69.23 percent and as good in 23.07 percent of patients. Conclusions: Better functional results were achieved in younger patients, with denervation time of 3 months or less, in lesions in continuity, in patients without associated nerve injuries, in distal lesions, with neurolysis, and with nerve grafts less than or equal to 5 cm long. Functional outcomes following microsurgical repair of radial nerve injuries are encouraging and should be pursued.
Background: In children with global obstetric brachial plexus palsy, prioritization should be first focused on hand reinnervation and then directed to shoulder and elbow function. In this study, the surgical strategy for restoration of hand function and the methods and outcomes are analyzed. Methods: Between 1979 and 2005, 59 patients (61 extremities) underwent reconstruction for hand reanimation. The mean follow-up was 7.7 years (range, 2 to 22 years). Of these, 16 cases underwent primary reconstruction alone, 35 underwent both primary and secondary procedures, and 10 late cases underwent palliative surgery. Hand function was evaluated with a modified Gilbert- Raimondi hand scale (grades 4 to 6 were considered useful outcomes). Results: Grade 4 or better functional recovery was observed in six of six cases (100 percent) that underwent primary reconstruction within the first 3 months of life. These patients did not require any secondary procedures. Multiple secondary procedures were necessitated to maximize the functional outcome in late cases or in patients with incomplete recovery following primary reconstruction. Overall, 46 of 61 cases (75.4 percent) achieved grade 4 or greater. The long-term results were better; 23 of 26 cases (88 percent) with a follow-up of more than 8 years achieved grade 4 or greater. Conclusions: When primary reconstruction was performed within 3 months, functional return to the hand was the greatest and the need for palliative surgery was dramatically reduced. For older patients (≥4 months), secondary procedures can significantly enhance hand function. The best results were seen when a combination of tendon transfers and free muscles transfers was performed.
One of the most unsettling sequela of facial paralysis (FP) is the loss of the blink reflex, leading to both a functional and aesthetic deformity. A successful method of treating FP and, in particular, loss of eye-sphincter function, is the use of the cross-facial nerve graft (CFNG) to reinnervate the previously denervated orbicularis oculi muscle. The present study examined the histomorphometric aspects of the entire CFNG, with respect to axon diameter and myelin area. The axon profile of the CFNG had a positive correlation with motor end-plate counts and electrophysiologic recordings. These results should help in further understanding the number of motor axons needed to restore adequate function to the paralyzed eye sphincter, and establish more rational reconstructive procedures.
The reinnervation of full-thickness skin grafts has been studied with single mechanosensory unit recordings. Shortly after transplantation of the skin, most of the incoming fibers conduct impulses at slow velocities, which correspond to the unmyelinated group of sensory fibers. The older skin grafts demonstrated an increasing amount of myelinization as shown by the shorter latencies and faster conduction velocities. However, the critical balance between myelinated and unmyelinated fibers that characterizes normal cutaneous innervation was still disrupted in the grafted groups at 15 months. Whether this imbalance persists indefinitely is uncertain. The introduction of electrophysiological techniques into the study of peripheral nerve injuries, in experimental animals and in man, has given us some insight into the unexplored nature of this field of experimental and clinical investigation. The future promises to be exciting.
Background: In lower root avulsion plexopathies, free muscle transfers for hand reanimation provide the only hope for the paralyzed hand, as the outcomes of hand functional restoration after primary brachial plexus reconstruction are uniformly poor. The purpose of this study was to analyze the outcomes of free gracilis muscle transfers for hand reanimation in severe brachial plexus injuries in relation to the respective motor donors. Methods: Since 1981, 71 free gracilis muscles have been transplanted for hand reanimation. Thirty-eight were for finger flexion and 33 were for finger extension. Neurotizations included motor donors such as intercostal nerves (n = 29), contralateral C7 root (n = 28), spinal accessory nerve (n = 7), or upper roots of the ipsilateral plexus (n = 5). Results: Preoperative and postoperative muscle grading and range of motion were found to be significantly different. The strongest motor donor for finger extension was the distal spinal accessory. The medial antebrachial cutaneous nerve as a conduit nerve carrying motor axons yielded worse results than other motor donors. Intercostals were useful for finger flexion and the contralateral C7 root was useful for finger extension. Scar formation in the volar wrist area was frequently a problem. Conclusions: After securing the stability and function of the proximal joints of the upper limb, attention should be shifted to the hand, and in compliant patients with supple finger joints, hand reanimation should be attempted. It is only through these efforts that the future of the paralytic limb can be upgraded to a useful assist extremity.
Sir: My article in the March of 2009 issue of Plastic and Reconstructive Surgery entitled “The ‘Babysitter’ Procedure: Minihypoglossal to Facial Nerve Transfer and Cross-Facial Nerve Grafting”1 refers to T. Drobnik as somebody who was associated with the accessory facial nerve transfer. To be exact, the statement goes as follows: “When the proximal part of the facial nerve is unusable, different motor donor nerves can be used (accessory, hypoglossal, trigeminal, and phrenic) ….” Whether Drobnik performed his procedure in 1879 or in 1899, as the authors of this letter claim, is a matter of conjecture. The origin of “ideas” that led to pioneering procedures is something on which I spent a substantial amount of time when I was compiling the book on the history of microsurgery. It is a challenging journey to pinpoint the influences to which each pioneer was exposed that eventually led to the introduction of a new technique or a new surgical procedure. Robert C. van de Graaf and Jean-Philippe A. Nicolai took this journey in 2003, when they attempted to explore who was the first surgeon to operate on the facial nerve.2 In 1902 to 1903, L’ etat actual de la chirurgie nerveuse was published, edited by Professor Antoine Chipault of Paris. Chapter 14 of the second volume was written by Bronislas Sawicki, a well-known neurologic surgeon in Warsaw, who described the most important neurosurgical operations performed by Polish doctors. On page 189 of this chapter, Sawicki refers to the facial nerve procedure performed by T. Drobnik in 1879, which was not previously published. As the original description of Drobnik’s surgery has never been found, the suggestion by van de Graaf and Nicolai that the 1879 date was a typographical error (they claim it should be 1899) remains an unresolved issue, and it is left up to the individual reader to decide. Julia K. Terzis, M.D., Ph.D. Department of Surgery Division of Plastic Surgery Microsurgical Research Center Eastern Virginia Medical School 700 Olney Road Lewis Hall, Room 2055 Norfolk, Va. 23501 [email protected]
Background: The cross-facial nerve grafting/free-muscle transfer strategy for smile restoration is superior to static reconstruction or regional muscle transposition. The purpose of this study was to evaluate the long-term outcomes of this technique in adult patients. Methods: Eighty-one adult patients received a free-muscle transfer for midface reanimation in the authors' center. Of this group, the authors identified 24 cases with follow-up of 5 years or longer. Smile symmetry and function were evaluated at three points: preoperatively, early postoperatively, and at long-term follow-up. To better evaluate the effect of time, patients were divided into groups according to the length of follow-up: group A, 5 to 6 years; group B, 7 to 10 years; group C, 11 to 15 years; and group D, more than 15 years. Four independent observers rated each patient's smile using a five-category scale ranging from poor to excellent. Panelists were asked to comment on whether the patient's smile weakened over time. Results: All patients obtained higher scores at 2 years from free-muscle transfer in comparison with their preoperative rates (p < 0.0001). Late outcomes demonstrated that muscle regeneration continues beyond the initial 2 years, with a further increase of the scores and motor units on electromyography at the late follow-up (p < 0.0001, p = 0.0313). No significance was found when comparing both variables among the four groups, indicating that time does not have a differential effect on muscle function. In 80 percent of the evaluations, the four observers agreed on maintained smile symmetry over time. Conclusions: Cross-facial nerve grafting/free-muscle transfer is an effective technique for smile restoration in late facial paralysis. These data indicate maintenance of effective muscle function and progressive improvement with time.
