Moebius syndrome with oral involvement

Summary. The oral findings of 12 patients with Moebius syndrome are described. Facial weakness, hypoplastic upper lip, microstomia, mouth-angle drooping, hypoplasia of mandible, gothic palate, tongue weakness, fissured tongue, tongue atrophy and open bite were found. In addition to describing the oral findings, this report discusses some implications for dental treatment of patients with this syndrome.     

Self-inflicted oral trauma: report of case

Self-inflicted oral trauma is often encountered in patients who lack cerebral control over the masticatory cycle. Many factors must be considered in treatment, and a variety of appliances may be fabricated to prevent injury to oral structures. This case report documents the use of a soft acrylic appliance in treatment of a comatose patient who showed ruminatory reflex chewing.
This case report demonstrated successful use of a soft acrylic mouthguard material as an intraoral appliance. The appliance was designed and fabricated in accordance with previously mentioned guidelines (Table). Fabrication, insertion, and maintenance was uncomplicated and noninvasive. Periodic examinations over a 6-month period have demonstrated resolution of the oral lesion and a high level of tolerance of the appliance.     

Self-inflicted oral trauma: report of case

An easily made, reusable mouth- guard suitable for comatose patients with neuropathologic chewing is presented. Its use is described for a comatose child with self-inflicted tongue trauma.     

Self-inflicted oral injury in an infant with transverse myelitis

Acute transverse myelitis (ATM) is an inflammatory disorder that affects the spinal cord. It is characterized by signs and symptoms of neurologic dysfunction in the motor and sensory tracts of the spinal cord bilaterally, resulting in weakness, sensory loss, and autonomic dysfunction with acute or subacute onset. We report on a child who had ATM and respiratory distress requiring ventilatory assistance who presented with severe self-mutilation involving his lips. Other findings included bruxism that had caused mobility of the primary teeth. Comprehensive dental care was carried out under sedation. The management of the lip trauma was achieved by the use of a soft, plastic mouth guard, and corticosteroid ointment.     

Soft tissue facial morphometry in subjects with Moebius syndrome

Moebius syndrome is a congenital facial palsy associated with the impairment of ocular abduction. The three‐dimensional characteristics of the facial soft tissues of 12 male and 14 female subjects [3–52 yr of age (mean age + standard deviation: 17 + 14 yr)] were measured using a non‐invasive, computerized system; facial volumes, areas, angles, and distances were computed and compared with those obtained in reference subjects of the same age and gender. When compared with reference subjects, patients with Moebius syndrome had a more prominent and hyperdivergent face in the sagittal plane, a smaller and more prominent upper facial third; a smaller middle facial width; a smaller nose; smaller mandibular volume, depth, corpus length, and ramus height; and a more posterior positioned mandible, with a less prominent chin. In conclusion, patients with Moebius syndrome had a tendency towards a skeletal Class II pattern. These morphological variations may be the combined effect of a general alteration of the motor and sensitive facial nerves, including the trigeminal nerve, and of a maldevelopment of the brainstem.     

Resiliency and success in adults with Moebius syndrome.

OBJECTIVE: This study asked selected mature individuals with Moebius syndrome to discuss the sources of strength and resiliency that allowed them to achieve professional and personal success. DESIGN: Adults with Moebius syndrome were selected in a nonrandom manner based primarily on their affiliation with the Moebius Syndrome Foundation. Following a letter from the author describing the objective, the subjects were interviewed by telephone and were encouraged to respond at length. PARTICIPANTS: Eighteen adults (aged 29 to 70 years) responded. All were or had been gainfully employed in a variety of professional or vocational positions. RESULTS: The respondents reported the following as major sources of resiliency and success: family support, faith, humor, sense of self, special skills, determination, and networking. CONCLUSIONS: The recognition and reinforcement of strengths and resiliences in younger patients may help maximize their professional and personal success as adults.     

"Moebius syndrome": a case report

Moebius syndrome is an extremely rare disorder characterized by a lifetime facial paralysis, involving sixth and seventh cranial nerves with malformations of orofacial structures and the limbs. In this case, an 8 year old girl with Moebius syndrome is presented, clinical findings are described and management aspects are discussed. Early dental evaluation and parental counselling as a part of preventive dental regimen can go a long way in providing complete psychosocial rehabilitation for such physically disabled children.     

An interesting case of systemic lupus erythematosus in a patient with Moebius syndrome

Moebius’ syndrome (MS) is characterized by a nonprogressive facial palsy associated with impairment in eye abduction, which can be uni‐ or bilateral. Some authors raise the possibility that patients with MS may suffer from social stigmatism due to their facial dysmorphism and that constant teasing and bullying perpetrated by people in the same social circle are adjuvants in the development of low self‐esteem, behavioral problems, and even psychiatric disorders. Psychological stress, anxiety, and depression are factors contributing to both development and impairment of autoimmune diseases, such as systemic lupus erythematosus (SLE). The objective of this work is to report the case of a patient with MS who developed SLE. In the present case report, we have emphasized the importance of both clinical dental examination and surgeon–dentist in the early diagnosis of systemic diseases by considering that these conditions can affect both syndromic and normoreactive patients.     

Long-term results of facial animation surgery in patients with Moebius syndrome

Gracilis neuromuscular transplant is considered the gold standard for facial animation in Moebius syndrome patients. However, long-term evaluation of the results has not been critically examined in the international literature. Thus, it remains unknown how the transplanted flap changes with facial growth, and whether contraction (smiling) is maintained.Pediatric patients with Moebius syndrome who underwent facial animation surgery with at least 5 years of follow-up were retrospectively examined. Photographs taken at the 1-year and most recent follow-up visits were analyzed and compared using Emotrics software. Analyses focused on the rest position, and on gentle and maximum smiles.Eighteen patients were enrolled. Seven patients had bilateral and 11 unilateral Moebius syndrome; therefore, 25 gracilis transplants were analyzed. The latest follow-ups ranged from 5 to 13.2 years (mean 7.6 years).The three principal facial expressions that were examined did not differ significantly between 1 year and a mean of 7.6 years after surgery, but tended to improve in most patients. Commissure excursion and smile angle for the maximum smile did improve significantly (p = 0.002 and 0.029, respectively).The series examined in this study supports the limited literature regarding the long-term stability of gracilis transplantation to animate the faces of Moebius syndrome children.     

Self-inflicted orodental injury in a child with Leigh disease

Leigh disease is an inherited progressive mitochondrial neurodegenerative disease that affects the neurological, respiratory and cardiovascular systems and is associated with retardation of the intellectual and physical development. This report describes the case of a 4-year-old boy with Leigh disease who presented with self-inflicted traumatic injury to the teeth, alveolar bone, lips and tongue during repeated episodes of intense orofacial spasms. Conservative management of the injury included repositioning the fractured alveolar bone, splinting the traumatized teeth and planning for a mouthguard. However, after a second incident of severe self-induced injury to the teeth and alveolar bone, extraction of the anterior teeth became inevitable to protect the child from further self-mutilation and to allow healing of the injured tissues.     

The effects of gracilis muscle transplantation on speech in children with Moebius 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.     

Poland-Moebius syndrome: a case with oral anomalies

The combination of Moebius and Poland anomalies is rarely described in the literature. While some authors believe this association is an independent syndrome, others think that Poland, Moebius and Poland-Moebius syndromes are variations of the same condition. We report a case of Poland-Moebius syndrome in a 6-year-old girl who presented with bilateral convergent strabismus, the inability to abduct her eyes beyond the midline and brachydactyly of her right hand. Oral manifestations included incompetent lips and an abnormal tongue. Other facial features included hypoplasia of her mandible and her left ear at a slightly lower level than her right ear. Panoramic and lateral skull radiographs confirmed the absence of certain teeth and the hypoplastic mandible. The diagnosis of Poland-Moebius syndrome was made on the basis of cranial nerve involvement and oro-facial manifestations.     

Prevalence of oral trauma in children with bilateral clefts

Abstract –  The main object of this study is to analyze the prevalence of oral trauma in subjects with complete bilateral clefts, with anterior projection of the premaxilla. A total of 106 children aging 6 months to 9 years were analyzed. The caretakers answered a specific questionnaire, in order to report the presence or absence of trauma to the soft and/or hard tissues of the child's mouth. Whenever there was a history of trauma, the patients were submitted to clinical examination. The prevalence of oral trauma was 53%, being 91% of soft tissue lesions, 8.9% of avulsion, 7% of luxation and 1.8% of intrusion. For the males, the prevalence was 56% and for the females it was 47%, with no statistical significance. Regarding the following aspects:period of time spent with the parents and at school, and presence or absence of siblings, no statistical difference could be found. Among the traumatized individuals, 80% aged less than 3 years by the moment of the trauma, 89% suffered the trauma at home, 75% presented lesions in the soft tissue at the premaxilla, 16% in the maxillary incisors, and 8.9% presented lesions in both structures. It was noticed that 45% of the permanent incisors that succeeded the traumatized deciduous teeth presented alterations, being 48% of structure and 52% of structure and position. The prevalence of trauma in this sample was superior to that observed in the literature, without any positive associations between the evaluated aspects. These results suggest that the projection of the premaxilla brings about a higher risk of oral trauma around this area.