scholarly journals Treatment of children with Pierre Robin Syndrome

2021 ◽  
Vol 2 (3) ◽  
pp. 35-53
Author(s):  
Dmitry Yu. Komelyagin ◽  
Sergey A. Dubin ◽  
Philipp I. Philipp I. Vladimirov ◽  
Alexey V. Petukhov ◽  
Svetlana V. Yamatina ◽  
...  

Introduction. Incidence of Pierre Robin sequence (PRS) according to different studies varies from 1:8500 to 1:30000 newborns. Swallowing dysfunction may lead to aspiration of food, saliva and gastric content, resulting in severe aspirational pneumonia. Obstructive sleep apnea (OSA) may be a life threatening condition for children with PRS and may cause death by asphyxia. Average mortality rate for PRS according to different studies is approximately 16 % and varies depending on several factors: number of patients in studies, severity of the condition, diagnostic methodologies and presence of associated syndromes in maxillofacial region. Mortality rate in severe PSR (especially associated PSR) is 41,4 %. Aside from high mortality, OSA contributes to the development of cardiovascular disease including pulmonary hypertension, which progresses more rapidly than in adults. OSA exacerbates existing comorbidities; in the absence of timely treatment sleep apnea leads to learning impairment and behavioral problems, developmental delay, failure to thrive. Methods. 80 children with PSR aged between 4 days and 1 year have undergone compression-distraction osteosynthesis (CDO). 48,8 % (39) of children were diagnosed with severe degree of OSAS, 35 % (28) had moderate degree, 16,3 % (13) had mild degree. Pretreatment diagnostics included standard set of examinations and specialist consultations. Special diagnostics included CT of head and neck, fiberoptic nasopharyngoscopy, examination by ENT specialist for the assessment of the degree of upper airways obstruction and exclusion of pathologies and anomalies of nasal cavity, pharynx, larynx and trachea, which also may be the cause for reduced airflow and upper airway obstruction (intubated children haven’t undergone these examinations). Photographs were taken and videos of clinical manifestations of airway obstruction during sleep were recorded. Blood gas tests were regularly taken; blood oxygenation levels were measured with a pulse oximeter. Polysomnography was performed, which allows for accurate and comprehensive evaluation of severity of OSA based on multiple parameters. Extra-cortical and external compression-distraction devices (CONMET, KLS Martin, Synthes) were used. Results. Good outcome was achieved in 90 % (72), satisfactory in 6,3 % (5) and negative in 3,8 % (3) of cases.

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Macario Camacho ◽  
Justin M. Wei ◽  
Lauren K. Reckley ◽  
Sungjin A. Song

Objectives. During anesthesia emergence, patients are extubated and the upper airway can become vulnerable to obstruction. Nasal trumpets can help prevent obstruction. However, we have found no manuscript describing how to place nasal trumpets after nasal surgery (septoplasties or septorhinoplasties), likely because (1) the lack of space with nasal splints in place and (2) surgeons may fear that removing the trumpets could displace the splints. The objective of this manuscript is to describe how to place nasal trumpets even with nasal splints in place. Materials and Methods. The authors describe techniques (Double Barrel Technique and Modified Double Barrel Technique) that were developed over three years ago and have been used in patients with obstructive sleep apnea (OSA) and other patients who had collapsible or narrow upper airways (i.e., morbidly obese patients). Results. The technique described in the manuscript provides a method for placing one long and one short nasal trumpet in a manner that helps prevent postoperative upper airway obstruction. The modified version describes a method for placing nasal trumpets even when there are nasal splints in place. Over one-hundred patients have had nasal trumpets placed without postoperative oxygen desaturations. Conclusions. The Double Barrel Technique allows for a safe emergence from anesthesia in patients predisposed to upper airway obstruction (such as in obstructive sleep apnea and morbidly obese patients). To our knowledge, the Modified Double Barrel Technique is the first description for the use of nasal trumpets in patients who had nasal surgery and who have nasal splints in place.


2017 ◽  
Vol 16 (2) ◽  
Author(s):  
Mohamed Hisham Mohamed Jali @ Yunos ◽  
Shaifulizan Abdul Rahman ◽  
Ramizu Shaari

Introduction: Pierre Robin Sequence (PRS) is a condition consists of set of anomalies, which are cleft palate, micrognathia and glossoptopsis. Management of patients with PRS addresses two main problems, namely airway obstruction and feeding difficulties. Airway obstruction may lead to obstructive sleep apnea (OSA). Treatment modalities for OSA are based on the causes. There are surgical and non-surgical methods. Non-surgical methods such as diet, medication, oral appliances and continuous positive airway pressure (CPAP) can only be employed in moderate cases. Surgical method such as maxillo-mandibular advancement or expansion can be achieved by orthognathic surgery or distraction osteogenesis. We present a case report of successful management of airway in a 23-year old lady who has a PRS features with severe OSA. In our case, the respiratory obstruction that was caused by retrognathic and hypoplastic mandible has been corrected successfully with distraction osteogenesis and the OSA was found to be improved tremendously.


2013 ◽  
Vol 18 (3) ◽  
pp. 143-149 ◽  
Author(s):  
Patrícia Superbi Lemos Maschtakow ◽  
Jefferson Luis Oshiro Tanaka ◽  
João Carlos da Rocha ◽  
Lílian Chrystiane Giannasi ◽  
Mari Eli Leonelli de Moraes ◽  
...  

OBJECTIVE: To verify if the reference values of Sleep Apnea cephalometric analysis of North American individuals are similar to the ones of Brazilian individuals presenting no craniofacial anomalies. The study also aimed to identify craniofacial alterations in Obstructive Sleep Apnea-Hypopnea Syndrome (OSAHS) patients in relation to individuals without clinical characteristics of the disease through this cephalometric analysis. METHOD: It were used 55 lateral cephalograms consisting of 29 for the control group of adult individuals without clinical characteristics of OSAHS and 26 apneic adults. All radiographs were submitted to Sleep Apnea cephalometric analysis through Radiocef Studio 2.0. The standard values of this analysis were compared, by means of z test, to the ones obtained from the control group and these were compared to values from apneic group through Student's t test. RESULTS: There were no significant differences between values obtained from control group and standard values. On the group of OSAHS patients it was observed a decrease on the dimensions of upper airways and an increase on the soft palate length. CONCLUSIONS: The standard values of Sleep Apnea analysis can be used as reference in Brazilian individuals. Besides, through lateral cephalograms it was possible to identify craniofacial alterations in OSAHS patients.


2012 ◽  
Vol 112 (3) ◽  
pp. 403-410 ◽  
Author(s):  
Chien-Hung Chin ◽  
Jason P. Kirkness ◽  
Susheel P. Patil ◽  
Brian M. McGinley ◽  
Philip L. Smith ◽  
...  

Defective structural and neural upper airway properties both play a pivotal role in the pathogenesis of obstructive sleep apnea. A more favorable structural upper airway property [pharyngeal critical pressure under hypotonic conditions (passive Pcrit)] has been documented for women. However, the role of sex-related modulation in compensatory responses to upper airway obstruction (UAO), independent of the passive Pcrit, remains unclear. Obese apneic men and women underwent a standard polysomnography and physiological sleep studies to determine sleep apnea severity, passive Pcrit, and compensatory airflow and respiratory timing responses to prolonged periods of UAO. Sixty-two apneic men and women, pairwise matched by passive Pcrit, exhibited similar sleep apnea disease severity during rapid eye movement (REM) sleep, but women had markedly less severe disease during non-REM (NREM) sleep. By further matching men and women by body mass index and age ( n = 24), we found that the lower NREM disease susceptibility in women was associated with an approximately twofold increase in peak inspiratory airflow ( P = 0.003) and inspiratory duty cycle ( P = 0.017) in response to prolonged periods of UAO and an ∼20% lower minute ventilation during baseline unobstructed breathing (ventilatory demand) ( P = 0.027). Thus, during UAO, women compared with men had greater upper airway and respiratory timing responses and a lower ventilatory demand that may account for sex differences in sleep-disordered breathing severity during NREM sleep, independent of upper airway structural properties and sleep apnea severity during REM sleep.


2011 ◽  
Vol 48 (3) ◽  
pp. 331-336 ◽  
Author(s):  
Margit Bacher ◽  
Judit Sautermeister ◽  
Michael S. Urschitz ◽  
Wolfgang Buchenau ◽  
Joerg Arand ◽  
...  

2011 ◽  
Vol 2 (4) ◽  
pp. 209
Author(s):  
Alessandra Giordano ◽  
Alessandro Cicolin ◽  
Roberto Mutani

Obstructive Sleep Apnea Syndrome (OSAS) is a sleep disorder characterised by repetitive episodes of upper airway obstruction (apnea) or reduced airflow (hypopnoea) despite persistent respiratory effort. Apnea is defined as the cessation of breathing for at least 10 seconds during sleep, while hypopnoea is defined as at least 30% reduction in airflow for 10 seconds associated with oxygen desaturation and sleep fragmentation. The presence in the general population is about 4%. The principal symptoms are: excessive daytime sleepiness (EDS), snoring, dry throat, morning headache, night sweats, gastro-esophageal reflux, and increased blood pressure.Long term complications can be: increased cardio-cerebrovascular risk and cognitive impairment such as deficiency in attention, vigilance, visual abilities, thought, speech, perception and short term memory.Continuous Positive Airway Pressure (CPAP) is currently the best non-invasive therapy for OSAS.CPAP guarantees the opening of upper airways using pulmonary reflexive mechanisms increasing lung volume during exhalation and resistance reduction, decreasing electromyografical muscular activity around airways.The causes of cognitive impairments and their possible reversibility after CPAP treatment have been analysed in numerous studies. The findings, albeit controversial, show that memory, attention and executive functions are the most compromised cognitive functions.The necessity of increasing the patient compliance with ventilotherapy is evident, in order to prevent cognitive deterioration and, when possible, rehabilitate the compromised functions, a difficult task for executive functions.


PEDIATRICS ◽  
1983 ◽  
Vol 71 (5) ◽  
pp. 737-742 ◽  
Author(s):  
Yitzchak Frank ◽  
Richard E. Kravath ◽  
Charles P. Pollak ◽  
Elliot D. Weitzman

Obstructive sleep apnea syndrome was studied in 32 children, aged 2 to 14 years, in the sleep-wake disorders center at Montefiore Hospital and Medical Center during the years 1977 to 1980. All children under-went all-night polysomnograms; 17 of these children had surgery to relieve airway obstruction and seven had a repeat polysomnographic study 4 to 6 weeks following the surgery. There was a significant improvement in the number of obstructive apneas and in other apnea indices following surgery. There was no significant effect on the durations and the proportions of the various sleep stages, on sleep efficiency, or on the number of awakenings.


SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A474-A474
Author(s):  
Nishant Chaudhary ◽  
Mirna Ayache ◽  
John Carter

Abstract Introduction Positive airway pressure-induced upper airway obstruction has been reported with the treatment of obstructive sleep apnea (OSA) using continuous positive airway pressure (CPAP) along with an oronasal interface. Here we describe a case of persistent treatment emergent central sleep apnea (TECSA) inadequately treated with adaptive servo ventilation (ASV), with an airflow pattern suggestive of ASV-induced upper airway obstruction. Report of Case A 32-year-old male, with severe OSA (apnea hypopnea index: 52.4) and no other significant past medical history, was treated with CPAP and required higher pressures during titration sleep studies to alleviate obstructive events, despite a Mallampati Class II airway and a normal body mass index. Drug-Induced Sleep Endoscopy (DISE) showed a complete velopharynx and oropharynx anterior posterior (AP) collapse, long soft palate, which improved with neck extension. CPAP therapy, however, did not result in any symptomatic benefit and compliance reports revealed high residual AHI and persistent TECSA. He underwent an ASV titration sleep study up to a final setting of expiratory positive airway pressure 9 cm H2O, pressure support 6-15 cm H2O (auto-rate), with a full-face mask due to high oral leak associated with the nasal interface. The ASV device detected central apneas and provided mandatory breaths, but did not capture the thorax or abdomen, despite normal mask pressure tracings. Several such apneas occurred, with significant oxyhemoglobin desaturation. Conclusion We postulate that the ASV failure to correct central sleep apnea as evidenced by the absence of thoracoabdominal inspiratory effort, occurred due to ASV-induced upper airway obstruction. Further treatment options for this ASV phenomenon are to pursue an ASV-assisted DISE and determine the effectiveness of adjunctive therapy including neck extension, nasal mask with a mouth closing device and a mandibular assist device.


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