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Obstructive sleep apnea syndrome in children: a state-of-the-art review.

Snoring and obstructive sleep apnea are a frequent problem not only in adults, but also in children and adolescents, as can be seen from current epidemiological data. The epidemiology, etiology, diagnosis, and management of obstructive sleep apnea syndrome (OSAS) in adults have been adequately established on the basis of evidential data. As a result of this, both physicians and the public are increasingly aware of OSAS in adults. Although there are numerous parallels between pediatric and adult OSAS, the situation in children differs that in adults. There is a greater variety of symptoms in children with OSAS, diagnosis is often more difficult with serious consequences for growth and development of children. Treatment of OSAS in children is also different from that of the adult patient. There are many possible causes for the development of obstructive sleep apnea in children. These include hypertrophy of the tonsils and syndromes such as Down syndrome, Pickwickian syndrome, Prader-Willi syndrome or Marfan syndrome. OSAS can, however, also be the result of obesity, midfacial dysplasia, retro- or micrognathia, allergic rhinitis or muscular dystrophy. Epidemiological data presented in the literature concerning the incidence of OSAS in children is extremely varied. This wide range is probably due to the fact that snoring may be misdiagnosed as OSAS. The diagnosis of OSAS in children may only be made by considering clinical history (such as rate of growth, tendency to fall asleep during the day, sleep disturbances, susceptibility to infection, etc.), polysomnography (if possible during several nights) and accompanying instrumental diagnosis including cephalometry or laryngoscopy. One of the problems of polysomnography in childhood is that performance and interpretation of the results have not yet been standardized or evaluated for different age groups. Treatment depends on the cause of OSAS and require multidisciplinary management involving the pediatrician, pediatric or adolescent psychiatrist, ear, nose, and throat specialist, maxillofacial surgeons, and neurosurgeons. Adenotonsillectomy (ATE) is the therapy generally chosen if the child has adenoidal vegetations and/or tonsillar hypertrophy. Corrective surgery is possible for rare malformation syndromes. Nocturnal masks for continuous positive airway nasal pressure or procedures for mask respiration are effective in children, but are only used in exceptional cases, such as when ATE is contraindicated or when symptoms of OSAS remain after surgery. The success of pharmacological treatment of OSAS in children has not been evaluated in controlled clinical trials.

Adenoids↗

Ultrasonic growth pattern of fetuses with chromosomal aberrations.

A retrospective analysis of the ultrasonic growth patterns of two 13-15 trisomies, two 18-trisomies and eight 21-trisomies is presented. The patterns were examined by serial cephalometry, abdominometry and head-to-abdomen circumference measurements. Early and symmetric growth retardation was revealed by ultrasonography and later confirmed by postnatal pediatric examinations in the 13-15 trisomies and 18-trisomies. In the 21-trisomies, fetal growth was normal.

Chromosomes, Human, 13-15↗

[Automated recognition and identification of soft tissue landmarks in cephalematric analysis]

OBJECTIVE: To establish the automatic x-ray cephalometric analysis system to provide the convenient and reliable method for clinical cephalometric analysis. METHODS The graphics, image processing techniques and artificial intelligence was usedand the computer digital image processing and pattern recognition such as median filtering, histogram equalization, Laplacian and Canny edge detection were introduced. To provide the templates of the variable anatomical structures, which could automatically outline the contour lines of the hard and soft tissues. Thirty five cases were measured and analysied with the system. RESULTS: The computer measurements had the same consistency with hand measurements. The system could calculate more precisely and save more time and energy than other systems. CONCLUSION: The system can supply a more convenient and precise measurement for cephalometry.

Journal Article↗

GAPO syndrome: a new case of this rare syndrome and a review of the relative importance of different phenotypic features in diagnosis.

The case of GAPO syndrome reported here is the 24th recorded case, 23 cases having been published previously. The 29-year-old male under discussion presents all the typical features of the syndrome, having short stature, dysmorphic craniofacial features, total alopecia and pseudoanodontia. Orally, the erupted primary dentition was extremely worn and on radiographic examination, the second mandibular molars were found to be unerupted, together with the entire permanent dentition. Cephalometry revealed the absence of facial pneumatisation, a deficient cranial base with diminished upper face height and maxillary and mandibular hypoplasia with a prognathic skeletal pattern. Histological examination of an extracted primary incisor and its surrounding root bone revealed extensive ankylosis. This paper describes in detail the clinical findings and reviews, and discusses previously published cases in relation to the present one. As with prior cases, parental consanguinity was present in the pedigree.

Abnormalities, Multiple↗

[The influence of edentulism on spirometric values].

BACKGROUND: Aim of the paper is to study whether edentulism, by causing a decrease in size and tone of pharyngeal musculature, may affect spirometric measurements. METHODS: Spirometry was recorded with and without dentures in 58 edentulous subjects, 36 asymptomatic normal subjects (N) and 22 patients with chronic obstructive pulmonary disease (COPD). In 10 subjects retropharyngeal space with and without dentures was assessed by cephalometry. RESULTS: In the N group, removing dentures produced a significant decrease in lung volumes and airflow rates, while in COPD patients it was ineffective. In both groups, retropharyngeal space was significantly decreased by removing dentures. CONCLUSIONS: Edentulism, by decreasing extrathoracic airway caliber, influences significantly spirometric measurements in normal subjects but not in those with COPD.

Aged↗

[Treatment of mandibular condylar hyperplasia in developmental age. Clinical case].

A case of hyperplasia of the mandibular condyle in a growing-up subject, observed at the Department of Maxillo-Facial Surgery of the University of Rome "La Sapienza", is described. Hyperplasia of the mandibular condyle is a facial asymmetry due to the unilateral overdevelopment of the mandibular bone. In this study the authors underline how bone scintigraphy, 3D tomography and electrognatographic analysis, associated with standard radiography and cephalometry, are important methods of diagnosis in order to make an early diagnosis of hyperplasia of the mandibular condyle and differential diagnosis with other pathologies. In particular, bone scintigraphy is a useful screening procedure to detect if the pathology is in an active phase or not. The 3D tomography is used in pre-surgery to evaluate precisely morphological and structural alterations of the craniofacial bones on a tridimentional base. Finally, the electrognatographic test records the mandibular activity both in physiological and pathological conditions. All these instrumental techniques allow to make a diagnosis and lead to a possible therapeutical approach.

Adolescent↗

[Distraction osteogenesis in correction of mandibular micrognathia accompanying obstructive sleep apnea syndrome].

OBJECTIVE: To evaluate the effect of distraction osteogenesis in correction of mandibular micrognathia accompanying obstructive sleep apnea syndrome (OSAS). METHODS: A total of 15 patients, aged 9 to 43 years, 11 males and 4 females, were treated. 7 patients suffered from bilateral ankylosis of temporo manclibular joint inducing severe mandibular micrognathia and accompanying severe OSAS. 3 cases suffered from unilateral TMJ ankylosis with OSAS. 3 patients suffered from congenital developmental micrognathia with OSAS. 2 cases were caused by trauma. Among the 15 patients, 12 were treated by synchronous bilateral mandibular distraction, 3 patients were distracted unilaterally. Every patient had been evaluated pre- and postoperatively with cephalometry and polysomnography. RESULTS: The minimum distance of distraction was 9 mm, the maximum was 30 mm, with a mean of 20.4 mm, in mandible of 15 patients with 27 sides. The distraction process was smooth in all the cases. The osteogenesis was good, without infection and other complications. Complete curative effect was achieved in 4 severe, 3 moderate, and 4 mild cases. The posterior airway space was increased from averaged 4.5 mm preoperatively to 12.4 mm. The increase of SNB angle was from preoperative 66 degrees to 75 degrees on average postoperatively. The vast majority of the patients had their subjective symptoms alleviated gradually or disappeared completely. The results were stable without relapse during a follow-up period of 12 months. CONCLUSION: The distraction osteogenesis can not only be used to effectively correct severe mandibular micrognathia deformity, but also can cure its accompanying OSAS. The procedure is simple, with low risk, high curative rate, low relapse rate, and stable result. Up to now, it is the most effective method in the treatment of this difficult and complicated kind of disorder.

Adolescent↗

[The study on the correlation between the effect of dental appliances on OSAS and the morphology of upper airway and craniofacial structures].

OBJECTIVE: The purpose of this present study was to evaluate the effectiveness of dental appliances on OSAS by combining the use of overnight polysomnography and cephalometry. METHODS: 22 OSAS adult patients wore the dental appliances designed by authors. The association between the improvement of respiratory variables and the cephalometric variables were investigated on the basis of linear correlation analysis. We also classified the subjects by AI of post-treatment to analyze the morphological disparity of upper airway and craniofacial structures among the groups. RESULTS: There was no significant correlation between the improvement of AI and the morphology of the upper airway and its surrounding soft structures, but there existed significant linear correlation between the improvement of AI and the morphology of the craniofacial skeletal structure. The improvement of the lowest SaO2 was only correlated significantly with the body length of the mandible and the anteroposterior position of the base of tongue. However, the improvement of the RDI wasn't associated significantly with any morphological variables. In addition, there were morphological differences among the different groups. CONCLUSION: The above findings suggested that the effectiveness of dental appliance on OSAS is mainly associated with craniofacial skeletal structures, but no correlation existed between the sagittal dimension of upper airway and the effectiveness.

Adult↗

[Combined genioglossus advancement (ACMG): inferior sagittal mandibular osteotomy with genioglossus advancement and stabilization with suture in patients with OSAS. Preliminary clinical results].

Surgical treatment of obstructive sleep apnea syndrome (OSAS) centres on the identification of the level of obstruction of the upper airway and the choice of the most suitable procedure to correct it. Shaping of the retrolingual hypopharyngeal space is among the most difficult to achieve because it stems from an alteration of the soft tissue of the tongue, from the hypopharynx, and is correlated to the contraction pattern of the genioglossus and the pharyngeal constrictors. We propose a surgical technique of combined genioglossus advancement (CGA) in cases of anteroposterior collapse of the retrolingual airway. Four patients affected by OSAS (RDI average = 22 events/hour), evaluated as type III obstruction in the Fujita classification, presenting the indications for surgical management of retrolingual hypopharingeal obstruction, underwent treatment. They were studied by means of a guided medical history, fiberopy endoscopy evaluation and Muller maneuver, cephalometry, endocrine tests, pneumological examinations and polysomnography. The technique proposed consists in the advancement of the genioglossus muscle by means of a bone screw on the mandibular symphysis, according to the method described by Powell, associated with the stabilization of the base of the tongue with a suspension suture, following the technique originally described by the Author and DeRowe, but without using the Repose kit. This technique makes it possible to access the retrolingual site of obstruction more effectively, more economically and with no increase in morbidity when compared with the individual techniques. In all of the patients, the only complaints regarded dysaesthesia in the area of the lower lip innerved by the mental nerve for 2-5 weeks and moderate odynophagia for 2-3 weeks; there were no haemorrhages or infections. Deglutition of fluids and solids was resumed on the 3rd post-operative day. Polysomnography after 6 months documented three positive results and one partial result, on the basis of Sher's criteria. In conclusion, the CGA technique calls for advancement of the genioglossus insertion tubercle and stabilization of the tongue to be carried out at the same time, without using the Repose kit. The CGA technique is minimally invasive and does not involve cutaneous incisions, making it a therapeutic strategy which may be inserted in a multilevel protocol excluding transcutaneous access. It is therefore proposed for type III or type IIb cases in the Fujita classification.

Female↗

[Diagram for the prognosis of cephalo-pelvic disproportions. Application in 300 cases of pelvic contraction].

The authors using the information obtained by X-ray pelvimetry and ultrasound cephalometry in 300 cases of contracted pelves, suggest a diagram of cephalo-pelvic disproportion that can be used in the great majority of cases of contracted pelves. It is not applicable to those where there is significant deformation of the middle strait or an abnormality of presentation. It is divided into three zones: 1. A superior zone, favourable, into which the majority (60%) of cases of contracted pelvis fit. The risk of a serious dystocia during delivery is so small (less than 2%) that it can be considered scarcely different from those found in women with a normal pelvis. 2. An intermediate zone into which 24% of cases of contracted pelvis fit where the prognosis is uncertain. The labour should be considered as an "at risk" labour and a trial of labour conducted. 3. A lower zone into which 16% of the cases fit and which consists of two categories: --those in which the biparietal diameter is equal to or less than 90 mm. Prophylactic caesarean section should be avoided in these cases where disproportion is rare and where there is a risk of delivering a premature infant: and --those more numerous (9 out of 10) where the biparietal diameter is more than 90 mm and where, unless there is a clinically definitely favourable prognosis, it is justifiable to carry out prophylactic caesarean section. If this line is followed: one can avoid a large number of trials of labour, all of which are inconvenient and some of which are dangerous for the child; few unnecessary caesarean sections are carried out.

Adult↗

[Pneumatization of the frontal sinus].

Through phylogeny and ontogeny, we follow the evolution of the frontal sinus; embryology gives details about the development of it. Then, we proceed to the investigation of factors influencing pneumatization. Afterwards, we analyse the different anomalies regarding pneumatization. In the last part, we mention experiments in progress being a link between cephalometry and the study of the frontal sinus surface in the diagnosis of skeletal Classes III.

Biomechanical Phenomena↗

[Suspicion of Down's syndrome based on patients obstetrical history and clinical data (author's transl)].

At the university women's clinic Zurich between 1963 and 1974, 29 babies in a total of 32255 babies were born with Down's syndrome. 11 of these had a cardiac vitium and 2 an intestinal malformation. 5 babies died during the first year of life. This high morbidity demands an analysis of the criteria in the obstetrical history and clinical picture in order to reach a diagnosis in those cases which would otherwise not be diagnosed if one used the normal criteric for amniocentesis in early pregnancy. Maternal age was the only relevant factor to emerge. In 6 pregnancies a tentative diagnosis for poor intrauterine growth was made in which only one case showed a retarded growth of the bipariental diameter. In all 7 cases in which cephalometry was performed after the 24 week of pregnancy, the concurrent hormonal parameters showed significantly lowered oestriol levels with normal HPL levels. From this information we draw the conclusion that transabdominal amniocentesis should be performed in in pregnancies with permanently low oestriol levels and normal HPL levels; especially in cases which clinically appear "small for dates" and for which there is no other explanation such as anencephaly for the low maternal oestriol levels.

Abortion, Spontaneous↗

[Skeletal and occlusal alterations in the diagnosis of Marfan syndrome].

The Marfan syndrome is an autosomal dominant hereditary connective tissue disorder with variable expressivity. The incidence is estimated to be at least 1 case per 10000 individuals in most populations. The syndrome is caused by mutations in the gene coding for Fibrillin-1 (FBN1), an extracellular matrix glyco-protein. The gene responsible for the mutations was identified in the chromosome 15q21.1 region. Presented for the first time in 1896 by Dr Antoine Bernard Marfan, it was subsequently included among hereditary disorders of the connective tissue. Nowadays the Marfan syndrome is considered to be a heterogeneous pathology, that can derive from many mutations in the gene coding for Fibrillin-1. The ocular, cardiovascular and skeletal manifestations present in the individual affected by Marfan syndrome are consistent with a defect in the gene coding for a structural component of the connective tissues. From the different expressivity derive 4 types or variants of the syndrome: a) asthenia; b) non-asthenia; c) arachnodactyly; e) abnormal joint mobility. In this study the authors show that radiography and cephalometry analysis are important as diagnosis techniques in order to make an early diagnosis of Marfan syndrome, carry out differential diagnosis with other maxillo-facial pathologies and correct the therapeutic approach (orthodontic or surgical) of this syndrome.

Abnormalities, Multiple↗

[Surgical correction of orbital and periorbital deformities using lamella and complex osteotomies].

OBJECTIVE: To investigate surgical correction of orbital and periorbital deformities in orbital and periorbital region. METHODS: In order to reconstruct orbital deformities such as orbital hypertelorism, orbital dystopia, radiated orbit, and secondary deformities of traumatic orbit, lamella osteotomies of either orbital rim and wall or complex osteotomies of both adjacent orbital rims and walls using bicoronal or galea aponeurotica incision were employed. The reconstructed complex was placed in new position with rigid fixation. Clinic evaluations were performed before and after operative osteotomies such as cephalometry (measurement of interorbital distance, IOD), exophthalmos (measurement with Hetel exophthalmometer), the angle (between orbital horizontal level and bilateral tragus linkage, A-OT), and the linear distance (between lateral canthus and tragus, LC-T), then the gap differences were analyzed before and after operation. RESULTS: Nearly normal orbital volumes, better contour of orbit and midface, improved eyelids and visions were achieved in total 44 cases. There were 5 cases of supraorbital osteotomy (intracranial routine), 8 cases of medial orbital osteotomy, 3 cases of inferior orbital osteotomy, and 28 cases of both lateral and inferior orbital osteotomy in these series. No complications were found after surgery. The orbital and midfacial appearances were improved significantly. CONCLUSIONS: Lamella and complex osteotomies of orbital rim and wall are proved to be safe and effective to correct most deformities of orbital and periorbital region and various kinds of orbital malposition.

Adolescent↗

Tongue base suspension technique in obstructive sleep apnea: personal experience.

Tongue suspension with Kit Repose is a surgical mini-invasive end-oral technique used in treatment of rear tongue obstruction. The base of the tongue is anchored with a non-reabsorbable suture, held in place with a titanium screw, to the mandible in correspondence to the geni apophysis of the mandible: this loop should prevent the tongue, during sleep, from dropping backwards, favoured also by gravity and hypotonicity of the genioglossus muscle. Aim of this report is to focus on the results of our experience in 15 patients presenting obstructive sleep apnea submitted to uvulopalatopharyngoplasty associated with tongue suspension, using the Kit Response bone screw system (Influent Inc., San Francisco, CA, USA). Mean age of patients was 50.5 years (range 36-66), with mean RDI (apnoea/hypopnea index) of 44.47 (range 23-63) and mean body mass index of 28.27 (range 22.6-34.4). Scrupulous clinical evaluation, including endoscopy and cephalometry, revealed a pharyngeal obstruction both retro palatal and retro lingual. Clinical and polysonnographic examinations were carried out 4-6 months after surgery. Patients were considered responders if the RDI had decreased by 50% and below 20, with disappearance of subjective symptoms (snoring, daytime sleepiness). Polysonnographic examination showed, overall, good results with mean reduction of RDI from 44.5 to 24.2 (45% reduction); albeit, only 6 cases could be considered surgically successful; 4 cases (26.6%) showed improvement whereas the remaining 5 (33.4%) failed to present any significant change in RDI. Even if the technique was, indeed, mini-invasive, rapidly performed and lacked significant complications, the results were not, in our opinion, encouraging, bearing in mind the high cost of the kit and limited stability of the results over time. Better results can be obtained by advancement of the genioglossus associated with hyoid suspension, whereas, of the mini-invasive techniques, promising outcomes would appear feasible with reduction of volume at the base of the tongue, using radiofrequency.

Adult↗

Genetic study of the craniofacial growth in male rats by factor scores.

The craniofacial growth of F1 male offspring produced by a complete diallel cross of five strains of rats was observed with the use of cephalometry. Factor analysis was applied to several dimensions of dorsoventral and lateral cephalograms to assess the integration of the craniofacial complex with growth. The first three factors were chosen in each cephalogram, resulting in an explanation of 50.7% and 59.3% of the variation in the dorsoventral and lateral cephalograms, respectively, on an average throughout the period of the experiment, and disclosing that Factor I was associated most closely with the change in the craniofacial growth of rats. A further genetic analysis of the factor scores of each offspring lead to the conclusion that the growth changes of craniofacial width and length were more strongly influenced by the genetic effects; while those of upper jaw, nasal cavity, and pharyngeal part were more strongly influenced by the environmental effects.

Animals↗

[Genioglossus advancement and hyoid suspension plus uvulopalatopharyngoplasty for severe OSAHS].

OBJECTIVE: To explore a comprehensive surgical approach of genioglossus advancement and hyoid suspension plus uvulopalatopharyngoplasty on the treatment of severe obstructive sleep apnea hypopnea syndrome. METHOD: Eighteen patients with severe OSAHS (RDI > 40)diagnosed by polysomnography. The obstruction in both the oropharynx and the hypopharynx were determined by preoperative physical examination, fiberoptic pharyngolaryngoscopy, cephalometry, and computed tomography of the upper airway. All the cases were given continuous airway pressure via nose,five to seven days before operation and then were performed genioglossus advancement and hyoid suspension plus uvulopalatopharyngoplasty. The follow up was at least 6 months postoperatively. The Wilcoxon Signed Rank test was used to compare the preoperative and postoperative results by SPSS11.0 for windows. RESULT: Postoperative 6-24 months, there were statistically significance in all but BMI between preoperative and postoperative measurement. Mean RDI was reduced from preoperative (63.83 +/- 16.34) to postoperative (21.43 +/- 20. 34), lowest mean oxygen saturation increased from (72.44 +/- 7.07)% to (81.33 +/- 13.32)%. According to criterion at home, the 6-month rate of responder is 83.33%. The severe complications such as fracture of the mandible and injure of apex of inferior anterior teeth did not occurred. CONCLUSION: GAHM plus UPPP is effective surgical approach for patients with severe OSAHS who suffer from oropharyngeal and hypopharyngeal obstruction.

Adult↗

[Mandibular distraction osteogenesis in the treatment of obstructive sleep apnea syndrome in children with micrognathia].

OBJECTIVE: To evaluate the treatment of obstructive sleep apnea syndrome (OSAS) in children with congenital micrognathia using mandibular distraction osteogenesis and to discuss the advantages and disadvantages of this approach. METHODS: 6 patients (4 males, 2 females) had undergone mandibular distraction osteogenesis, 12 distraction devices were placed through extraoral incision for bilateral distraction. The mean age of treatment was 1 year and 9 months (range 4 months to 9 years). Every patient had been evaluated pre and postoperatively with cephalometry. The period of consolidation was 4 - 11 weeks. The period of follow-up was from 2 to 10 months. RESULTS: The average distraction distance was 19.2 mm (range 15 to 25 mm). The osteotomy and distraction processes were smooth in all the cases, the osteogenesis was good, without infection and other complications. The posterior airway space was increased from averaged 4.5 mm preoperatively to 10. 1mm after surgery. Five children had normal respiration and sleep restored with naso-pharyngeal airway removal or tracheostomy decannulation. One patient is to receive a planned second stage of distraction with a horizontal vector. The results were stable without relapse during a follow-up period of 2 to 10 months. CONCLUSIONS: Application of mandibular distraction osteogenesis is an important component and effective in the treatment of OSAS and permits mandibular advancement in the younger child. As more experience is gained with distraction osteogenesis in the treatment of children with OSAS, the role of distraction will become better defined.

Child↗