Möbius 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 Möbius 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 Möbius syndrome.
Speech and communication are major problems for children with Moebius syndrome, a congenital anomaly that includes facial and abducens nerve paralysis and, often, other cranial nerve deficits. In addition, these children frequently have severe functional problems such as drooling as well as poor self-esteem. The purpose of this study was to investigate the outcome of speech with bilateral gracilis muscle transplants innervated by the masseteric nerve in children with Moebius syndrome. The outcome of this two-stage procedure was investigated in 12 patients. Assessments were done before and after surgery, and additional data were collected from video records. After surgery, the children showed improved intelligibility of speech with a significantly lower frequency of all compensatory phonemes, including the sounds of /p/, /b/, /m/, /w/, "sh", /f/, and /v/. This procedure had evident positive impact in all problematic areas and is the procedure of choice for these unfortunate children.
The cyclic stresses in the cranium caused by pulsation of the brain play an important role in the design of materials for cranioplasty, as well as craniofacial development. However, these stresses have never been quantified. In this study, the force in the epidural space against the cranium was measured intraoperatively in 10 patients using a miniature force probe. Heart and ventilatory rates computed from the force tracing correlated closely with the corresponding measured values in the patients, confirming that the forces measured were indeed a result of brain pulsation. The mean outward systolic normal and tangential stresses were 54.2 kilo-Pascals (kPa) and 345.4 kPa, respectively. The systolic shear stress was 199.8 kPa. Through mechanotransduction, these stresses play a role in cranial development. The calculated yield stress of a cranioplasty repair was 0.4 MPa, which is within one order of magnitude of the known strength of common calcium-phosphate cements. This indicates a possible relation of these pulsatile forces and occult failure of calcium-phosphate cement cranioplasties through material fatigue.
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