Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0037315 (sleep apnea)
 8,000 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over the past 3 years, 16 patients (12 males, 4 females) have undergone mandibular distraction osteogenesis in conjunction with soft-tissue procedures to treat medically refractory obstructive sleep apnea. Thirty distraction devices were placed for bilateral distraction in 14 children and unilateral distraction in 2. The mean age of treatment was 4 years and 8 months (range 14 weeks to 12 years and 8 months). Eight of the patients had failed conventional medical and surgical treatment of obstructive sleep apnea and were considered tracheostomy candidates, whereas the remaining eight had tracheostomies placed shortly after birth for upper airway obstruction. These eight children could not be decannulated by standard protocols. The average distraction distance was 25 mm (range 18 to 35 mm). To date, seven of the eight patients with tracheostomies have been decannulated, and one is still in progress. Clinical improvement in the signs and symptoms of sleep apnea and reduction or elimination of preoperative oxygen requirements occurred in seven of the eight children with medically refractory sleep apnea. Twelve-channel polysomnograms were obtained preoperatively and postoperatively in each of the eight patients without tracheostomies. Respiratory disturbance index decreased from a mean of 7.1 to 1.7 after surgery. Lowest oxygen saturation rose from a mean of 0.70 to 0.89 after surgery. Application of mandibular distraction osteogenesis is an important component in the treatment of obstructive sleep apnea and permits mandibular advancement in the younger child. As more experience is gained with distraction osteogenesis in the treatment of children with obstructive sleep apnea, the role of distraction will become better defined.
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PMID:Mandibular distraction osteogenesis in the treatment of upper airway obstruction in children with craniofacial deformities. 946 62

Children with craniofacial malformations are at special risk for the development of peripheral airway obstruction. The problems are magnified in patients with retroposition or hypoplasia of the mandible. In these cases, the base of the tongue is posteriorly displaced, hereby decreasing the airway diameter. By application of distraction osteogenesis the mandible can be lengthened to move the base of the tongue forward and open the airway. Three female patients aged between 7, 11, and 15 months suffering from peripheral airway obstruction caused by mandibular hypoplasia were treated by gradual distraction. All of them had a gastrostomy or a nasogastral tube in place, respectively, due to severe nutrition problems. In the youngest patient tracheostomy was performed shortly after birth and was already planned in the 15-month-old child, who had received a permanent nasopharyngeal tube. The 11-month-old child suffered from severe refractory sleep apnea. Exercises in oral feeding were possible in the youngest patient after 10 days of distraction. In the oldest one, the airway tube was removed on the six day of distraction and, thus, tracheotomy was successfully avoided. In the 11-month-old child apneic events a rapidly decreased. Our experience suggests that distraction osteogenesis after careful preoperative evaluation can be successfully performed for the treatment of peripheral airway obstruction in patients with selected craniofacial anomalies.
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PMID:[Experiences with distraction osteogenesis in therapy of severe peripheral airway obstruction in infancy and early childhood]. 965 5

A new technique of using implantable springs as an adjunct after corrective surgery for craniofacial malformations is presented. A 6-month-old boy with multiple premature craniosynostoses and extreme turricephaly underwent surgery of limited extensiveness but supplemented with a set of indwelling springs for gradual postoperative skull reshaping. At spring removal three months later the skull was normalised both clinically and on cephalogram. A 5-year-old boy with Apert syndrome, severe midface retrusion, exorbitism, and sleep apnoea underwent a monobloc full face disjunction without repositioning, but was fitted with two springs for postoperative facial advancement. Three months postoperatively cephalometric analysis revealed 14 mm advancement at incisor level and at least 16 mm in the frontal region. There was no more exorbitism or clinically noticeable midface retrusion. Sleep studies revealed that the sleep apnoea was significantly improved, meaning complete cure except when sleeping flat on the back. It was concluded from these first clinical applications of spring assisted craniofacial distraction that springs hold significant promise for the future in many respects.
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PMID:Spring mediated dynamic craniofacial reshaping. Case report. 978 39

Twenty consecutive children, ranging in age from 6 days to 18 years, were treated with skeletal expansion, in addition to soft-tissue reduction, for medically refractory obstructive sleep apnea. The underlying diagnoses were craniofacial microsomia (n = 6), Down syndrome (n = 3), Pierre Robin syndrome (n = 3), cerebral palsy (n = 3), Nager's syndrome (n = 1), Treacher Collins syndrome (n = 1), cri du chat syndrome (n = 1), juvenile rheumatoid arthritis (n = 1), and temporomandibular joint ankylosis (n = 1). Fourteen children had severe medically refractory sleep apnea and were tracheostomy candidates; in the remaining six, tracheostomies were placed shortly after birth and could not be decannulated. Overnight, 12-channel polysomnography was obtained before and after surgery. The mean apnea index improved from 7.42 to 1.26, the mean respiratory disturbance index improved from 25.24 to 1.72, and the mean lowest apnea-related oxygen saturation improved from 68% to 88%. Of the 14 children with medically refractory obstructive sleep apnea, two required tracheostomies. Of the six patients with tracheostomies, five have been decannulated at the time of this writing. Skeletal expansion in conjunction with soft-tissue reduction in the pediatric population permits substantial increases in the volume of both the nasopharynx and oropharynx. Creative use of conventional osteotomies and the application of distraction osteogenesis have enabled surgeons to apply maxillofacial and craniofacial techniques in treating children with obstructive sleep apnea.
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PMID:Skeletal expansion combined with soft-tissue reduction in the treatment of obstructive sleep apnea in children: physiologic results. 980 73

Treatment of the craniofacial dysostoses (e.g., Crouzon, Apert, Pfeiffer, Saethre-Chotzen syndromes) is critically dependent on the successful advancement of the midface with a Le Fort III procedure. The purpose of this retrospective clinical outcome study was to evaluate a new technique for distracting the Le Fort III procedure and to compare its results in growing children with those of the standard Le Fort III osteotomy. The records of 22 children were reviewed; 10 patients (mean age, 6.5 years) underwent a standard Le Fort III procedure, and 12 patients (mean age, 7.5 years) underwent a Le Fort III distraction procedure. The distraction group included two separate techniques, bilateral buried distraction (n = 2) and halo distraction (n = 10). Preoperative and 2- to 3-month postoperative cephalograms were analyzed. The average horizontal advancement achieved in the standard Le Fort III group was 6 mm, compared with 19 mm of advancement in the distraction group (p </= 0.005). Complications were evenly distributed between the two groups (one infection and one tracheostomy in each group), and the lengths of hospitalization were similar. No documented improvement in sleep apnea was identified in the standard Le Fort III group. However, in the distraction group two patients experienced normalization of sleep studies postoperatively as measured by respiratory disturbance index, and two patients underwent successful decannulation of tracheotomies. For aesthetic reasons, halo distraction was preferred over bilateral buried distraction. With halo distraction the vector of traction is focused in the facial midline, which helps to reposition the concave midface and to provide a more convex facial profile. In growing children, the ideal vector for distraction is determined by the malar position and not by dental occlusion. The amount of overcorrection can be calculated from tables of normal anthropologic data. On this preliminary review, it was concluded that the use of halo distraction, in combination with a modified Le Fort III osteotomy, provided a significantly further forward-positioned midface and seemed to offer a better correction of sleep apnea than did the standard Le Fort III osteotomy.
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PMID:The Le Fort III osteotomy: to distract or not to distract? 1137 47

Paediatric obstructive sleep apnoea (OSA) associated with craniofacial anomalies frequently is refractory to traditional treatment modalities. Skeletal expansion using distraction osteogenesis to correct facial deformities has proven to be an excellent treatment modality for the resolution of OSA. This paper discusses the use of distraction osteogenesis from infancy through adolescence and will show objective improvement in not only facial norms but also in OSA indices.
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PMID:Paediatric sleep apnoea and treatment with distraction osteogenesis. 1170 30

We report an unusual problem with fibreoptic bronchoscopy in an 8-year-old girl with Negar syndrome. She had a history of difficult airway since birth, and had undergone mandibular distraction for severe obstructive sleep apnoea when she was aged 2 years. Nagar syndrome is a Treacher-Collins like syndrome with normal intelligence, conductive bone deafness and problems with articulation. The patients have malar hypoplasia with down slanting palpebral fissures, high nasal bridge, micrognathia, absence of lower eyelashes, low set posteriorly rotated ears, preauricular tags, atresia of external ear canal, cleft palate, hypoplasia of thumb, with or without radius, and limited elbow extension. Protracted attempts with a fibreoptic bronchoscope failed to visualize the glottis, and this was only possible when the tube was guided to the larynx by blind nasal intubation. Apparently, the healing of the wounds for the mandibular distraction in the mandibular space on the inside of the rami of the mandible had caused differential fibrosis on either side of the hyoid, leading to a triplane distortion of the larynx with a left shift, clockwise rotation to a 2-8 o'clock direction and a slight tilt towards the left pharyngeal wall. The large epiglottis overlying this had precluded a view of the larynx. Finally, the older technique of breathguided intubation facilitated fibreoptic bronchoscopy to achieve tracheal intubation.
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PMID:Difficult paediatric intubation when fibreoptic laryngoscopy fails. 1251 41

A 31-year-old man who had been diagnosed with obstructive sleep apnea syndrome (OSAS) due to retrognathia was referred to our hospital. Before treatment, the angle of the sella-nasion-point B (SNB) was 67 degrees. The lowest oxygen saturation (SpO2) during sleep was 77%, the longest-running episode of sleep apnea was 128 seconds, and his apnea index (AI) was 29.9. He underwent bilateral horizontal mandibular distraction osteogenesis using intraoral distraction devices. After mandibular distraction, SNB increased to 72 degrees. The lowest SpO2 during sleep increased to 85%, the longest-running episode of sleep apnea decreased to 40 seconds, and his AI decreased to 4.1 (within normal range). No marked recurrence of OSAS was observed even at one year after mandibular distraction. This report suggests that mandibular lengthening by distraction osteogenesis is an effectual method for the treatment of OSAS due to retrognathia.
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PMID:Mandibular lengthening by distraction osteogenesis for treatment of obstructive sleep apnea syndrome: a case report. 1255 33

We present a 26-year-old patient with juvenile-onset arthritis, Alagille's syndrome, micrognathia, and progressive sleep apnea. Despite the presence of significant temporomandibular joint pathology, mandibular distraction was indicated to correct life-threatening sleep apnea. Before distraction, the patient had only 10 mm of maximal interincisal opening and bilateral temporomandibular joint symptomatology. After distraction, the patient's sleep apnea resolved. There was slight improvement in her maximal incisal opening (12 mm) with neither exacerbation nor improvement of her temporomandibular joint symptomatology.
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PMID:Distraction osteogenesis in a patient with juvenile arthritis. 1663 96

The 22q11.2 deletion syndrome is characterized by wide phenotypic variability, frequently involving characteristic craniofacial features, cardiac malformations, and learning difficulties. Skeletal anomalies are also common and include an obtuse angle of the cranial base, retrognathia, and cervical spine abnormalities. Despite these anomalies, sleep-disturbed breathing is not reported frequently in patients with 22q11.2 deletion syndrome. We describe a patient with an obstructive sleep disturbance that was successfully treated with a tonsillectomy followed by mandibular distraction osteogenesis. She also had central sleep apnea, initially attributed to spinal cord impingement from cervical instability. Posterior cervical fusion was associated with a decrease in the number of central apneic events.
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PMID:Sleep disturbances in 22q11.2 deletion syndrome: a case with obstructive and central sleep apnea. 1747 50


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