Cases reported "Micrognathism"

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1/28. Moebius syndrome: the new finding of hypertrophy of the coronoid process.

    The first detailed description of congenital facial paralysis was reported by Moebius in 1888. It is characterized by either unilateral or bilateral paralysis of the facial muscles and an associated abducens palsy. The present report is of two patients with Moebius syndrome, who were also diagnosed with trismus at birth. Each patient also demonstrated bilateral hypertrophy of the coronoid process of the mandible. In effect, the zygoma obstructed the excursion of the mandible because of a "coronoid block." A three-dimensional computed tomography scan demonstrated normal temporomandibular joints but bilateral hypertrophy of the coronoid processes and micrognathia. Both patients demonstrated less than 10 mm of oral excursion. Bilateral coronoidectomies were performed through an intraoral approach. The oral excursions after surgery increased to at least 20 mm. In each of these patients, the coronoid process was enlarged relative to the zygoma, which was of normal size and configuration. The trismus was associated with blocking of the coronoid by the anterior zygoma, preventing open or full excursion of the hypoplastic mandibles. Moebius syndrome can have a variable presentation at birth. In two patients, the authors describe a new finding of hypertrophy of the coronoid process and trismus secondary to obstruction of the coronoid by the hypertrophic zygomas during oral excursions. Each patient is described, and a review of the literature is discussed.
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ranking = 1
keywords = obstruction
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2/28. Mandibular distraction osteogenesis in a neonate.

    Children with craniofacial anomalies are predisposed to airway obstruction and frequently require airway intervention. tracheotomy is performed when the airway obstruction is severe and refractory to other less invasive interventions. tracheotomy is associated with significant morbidity, and there is a trend noted in the literature toward achieving earlier decannulation by the institution of definitive structural changes to the mandible. Mandibular distraction osteogenesis has been shown to alleviate airway obstruction in the pediatric population. We report a case in which mandibular distraction osteogenesis was successfully carried out in a neonate with acute airway obstruction at birth as a result of combined Pierre Robin sequence and klippel-feil syndrome. After 1 year, the patient still had an adequate airway with tolerable scarring and no neurologic sequelae.
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ranking = 251.58221311456
keywords = airway obstruction, airway, obstruction
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3/28. The retrognathic mandible--surgical correction.

    The preceding case reports demonstrate the excellent results available to the patient with a retrognathic mandible. Etiology, adverse effects, and the modalities used in evaluation of the underdeveloped mandible have been discussed. The age at which surgical intervention is undertaken needs additional input by clinicians. Several surgical procedures have been and are employed to advance the mandible. The C osteotomy is recommended since the final result has been excellent and consistent. The surgeon has constant surgical control and vision. The advanced portion of the mandible can be firmly held by intraosseous wires. There is minimak disturbance of muscle position and the inferior alveolar nerve. We have not encountered unusual loss of blood, immediate postoperative problems with the airway, infection, or unfavorable relapse. A postoperative regimen that has been found rewarding is discussed. We have come to the conclusion that most patients can tolerate lengthy surgical procedures with few adverse side effects. Our colleagues in anesthesia and nursing provide superb operative and postoperative care. When the preoperative work-up indicates that more than one procedure is indicated, we recommend that the total surgical treatment plan be carried through at one operation, although occasionally this approach may be contraindicated. We should strive for perfection and can fall short of this goal when a patient will not return for additional surgical procedures which could have been accomplished during the first operation. Although good results have been experienced with both the C osteotomy and the sagittal split osteotomy, we believe that the C osteotomy is a superior procedure. However, this does not imply that the sagittal split osteotomy should not be a part of the armamentarium of the oral surgeon. In conclusion, the C osteotomy, the sagittal split osteotomy, genioplasty, and additional procedures--such as a mandibular alveolar osteotomy to intrude supraerupted teeth--when indicated, can provide very favorable results in cases of retrognathia or micrognathia of the mandible.
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ranking = 9.5455051529935
keywords = airway
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4/28. Anesthetic management for patients with arthrogryposis multiplex congenita and severe micrognathia: case reports.

    arthrogryposis multiplex congenita (AMC) is a spectrum syndrome of multiple persistent limb contractures often accompanied by associated anomalies, including cleft palate, genitourinary defects, gastroschisis, and cardiac defects. Pediatric patients with AMC frequently present for multiple surgeries requiring general endotracheal anesthesia. We describe our anesthetic experience with the laryngeal mask airway and endotracheal tube in two neonates with AMC and severe micrognathia. We discuss AMC and outline the problems encountered in difficult airway management.
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ranking = 19.091010305987
keywords = airway
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5/28. Complicated airway management in a child with prune-belly syndrome.

    We describe a 15-month-old boy with prune-belly syndrome (PBS) in whom airway management was complicated. Following an inhalation induction using sevoflurane, tracheal intubation by direct laryngoscopy proved impossible after repeated attempts. A laryngeal mask airway (LMAtrade mark) was inserted and the child had an uneventful anaesthetic course.
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ranking = 57.273030917961
keywords = airway
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6/28. Early treatment of severe mandibular hypoplasia with distraction mesenchymogenesis and bilateral free fibula flaps.

    The technique of distraction has revolutionized the treatment of mandibular hypoplasia; however, presently large mandibular defects still require bone grafts. Microvascular grafting is commonly used in adults. Conversely, in pediatric reconstruction, nonvascularized rib grafts remain standard. Unfortunately, resorption of nonvascularized bone remains a major issue, particularly when soft tissue is hypoplastic. This case study represents a combination of techniques in the treatment of severe mandibular deficiency, and introduces the concept of distraction mesenchymogenesis. The patient was a 2 1/2-year-old boy with severe bilateral Pruzansky class III mandibular hypoplasia. He had a permanent open mouth posture, an overjet of 23 mm, and was unable to move the lower mandibular segment. His oropharyngeal airway diameter was 2.2 mm and he was tracheostomy dependent. The patient was treated with distraction of the lower jaw mesenchyme followed by bilateral functional free fibular microvascular flaps containing reinnervated muscle. This created a well-vascularized body, ramus, and condyle bilaterally within an adequate soft-tissue envelope. Postoperatively, the overjet was reduced to 5 mm. The patient can now actively move his mandible. Airway diameter increased to 10 mm, and the patient is able to tolerate intermittent tracheostomy plugging. This innovative combination of techniques allows early intervention, limits graft resorption, and improves airway control.
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ranking = 19.091010305987
keywords = airway
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7/28. A technique for maintenance of airway access in infants with a difficult airway following tracheal extubation.

    Tracheal extubation of patients with a difficult airway represents a challenge to anaesthesiologists and intensive care physicians. While a variety of techniques designed to maintain access to the airway in case of the need for tracheal reintubation have been described in adults, no reports have been published in infants and young children. We describe an approach to this issue in a young child with severe micrognathia.
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ranking = 95.455051529935
keywords = airway
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8/28. airway obstruction following palatoplasty: analysis of 247 consecutive operations.

    OBJECTIVE AND methods: Between February 1987 and September 1997, 247 patients underwent primary repair of a cleft of the secondary palate by one surgeon, using the double-opposing Z-plasty (Furlow) technique. This retrospective study reviews perioperative and postoperative airway compromise among these patients. The purposes of this study were to identify factors associated with airway obstruction following palatoplasty and to analyze the management of those patients. Although infants experiencing airway problems following Wardill-Kilner and Von Langenbeck palatoplasty have been described, airway complications in a group of Furlow repair patients has not been previously reported. RESULTS: Fourteen patients (5.7%) had airway problems. The average age of these patients was 18 months, which was not significantly different from those without airway problems. airway obstruction occurred as late as 48 hours after the completion of surgery. Twelve of the 14 patients had severe airway compromise requiring continued postoperative intubation, reintubation, or tracheostomy (one). There were no deaths. Thirteen of the 14 patients with postoperative airway problems (93%) had other congenital anomalies in addition to clefting, a named congenital disorder, or both. Seven of those 13 had Pierre Robin sequence. In contrast, only 40 of the 233 patients without airway problems (17%) had additional congenital anomalies or named disorders. Presence of other congenital anomalies was associated with a significantly increased risk of airway obstruction (p =.005). CONCLUSION: patients with cleft palate with the Pierre Robin sequence or other additional congenital anomalies had an increased risk of airway problems following palatoplasty. awareness of this risk permits identifying those patients prior to surgery so that they can be monitored and managed appropriately, minimizing the likelihood of major complications or death.
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ranking = 238.87334481399
keywords = airway obstruction, airway, obstruction
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9/28. prenatal diagnosis of Pierre-Robin sequence as part of Stickler syndrome.

    Stickler syndrome or hereditary progressive arthro-ophthalmopathy, is an autosomal dominant condition characterized by ocular manifestations, arthritic changes, orofacial features and deafness, in variable degrees.We report the first case of prenatal diagnosis of Stickler syndrome in a child with a Pierre-Robin sequence (PRS) causing a polyhydramnios. When isolated polyhydramnios is not explained by immunological, metabolic or infectious causes, swallowing difficulty due to PRS must be considered. As PRS is aetiologically heterogeneous, the prognosis depends on the cause. Genetic investigations and familial history must be taken into account. Here, in a context of familial Stickler syndrome, making the prenatal diagnosis of PRS as part of Stickler syndrome allowed us to reassure the parents and to anticipate airway trouble at the child's birth.
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ranking = 9.5455051529935
keywords = airway
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10/28. Extreme microglossia.

    The congenital anomaly of extreme microglossia is uncommon and fewer than 50 cases have been described. The microglossia has often occurred in association with limb abnormalities and, therefore, these cases have been grouped together as the hypoglossia-hypodactylia syndrome within the oromandibular-limb hypogenesis syndromes. We present five cases seen at our referral centre. Surprisingly for this number none had limb anomalies but all had marked micrognathia-Gorlin-Hall classification type 5-two requiring tracheostomy for upper airway obstruction. All required tube feeding for between 4 and 17 months. Long term follow-up is not yet available.
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ranking = 73.981899218525
keywords = airway obstruction, airway, obstruction
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