Introduction
Material and methods
Results
Retrospective cohort analyses
Patients | Number of patients (%) | Female | Male | Median age of presentation in days (IQR) | Gestational age in days | Mean birth weight in grams (SD) | Presence of CP (%) | CP typeb (%) |
---|---|---|---|---|---|---|---|---|
Isolated RS | 32 (43) | 20 | 12 | 10.0 (5–17.75) | 275(median), 270(p25), 282 (p75) | 3135 (789) | 97 | I (0); II (16); III (58); IV (26) |
Nonisolated RS | 43 (57) | 19 | 24 | 8.0 (1.25–32.75) | 277(median), 273 (p25), 282 (p75) | 3237 (553) | 98 | I (3); II (24); III (56); IV (17) |
Syndromic RS | 23 | 10 | 13 | 7.5 (1–17.75) | 279 (median), 273 (p25), 281 (p75) | 3314 (512) | 100 | I (4); II (13); III (61); IV (22) |
Stickler syndrome | 11 | |||||||
Treacher Collins syndrome | 2 | |||||||
Spondyloepiphyseal dysplasia | 2 | |||||||
4q deletion syndrome | 1 | |||||||
Van der Woude syndrome | 1 | |||||||
Osteopathia striata with cranial sclerosis | 1 | |||||||
EEC syndromea
| 1 | |||||||
Goldberg–Shprintzen syndrome | 1 | |||||||
Yunis–Varon syndrome | 1 | |||||||
Auriculo–Condylar syndrome | 1 | |||||||
Hemifacial microsomia | 1 | |||||||
RS with other associated anomalies or chromosomal defects | 20 | 9 | 11 | 10.5 (2–62.75) | 275 (median), 272 (p25), 282 (p75) | 3149 (597) | 95 | I (0); II (37); III (53); IV (10) |
Total study group | Isolated RS | Nonisolated RS |
p value* | |
---|---|---|---|---|
Number of patients | 75 | 32 (43 %) | 43 (57 %) | |
Conservative treatmenta
| 44 (59 %) | 24 (75 %) | 20 (47 %) | 0.014 |
Surgical treatmentb
| 31 (41 %) | 8 (25 %) | 23 (53 %) | 0.014 |
MDO | 18 | 6 | 12 | |
TLA | 6 | 1 | 5 | |
Tracheotomy | 7 | 1 | 6 | |
Mean age at surgical intervention in days (SD) | 50 (55) | 57 (42) | 47 (60) | 0.620 |
Mean duration of admission in days (SD)c
| 48 (43) | 33 (35) | 58 (45) | 0.018 |
Conservatively treated group (SD) | 30 (30) | 24 (32) | 35 (27) | 0.285 |
Surgically treated group (SD) | 73 (46) | 55 (35) | 80 (48) | 0.163 |
Nasogastric tube | 58 | 20 (63 %) | 38 (88 %) | 0.009 |
Literature review
Study | Populationa
| Performed examinationb
| Indication for (surgical) intervention | Type of interventionc (%) | |
---|---|---|---|---|---|
Abel et al. (2012) [10] |
N: 104 MG, Gl, CP iRS, sRS | Overnight sleep study Microlaryngobronchoscopy when Tr was considered | Moderate ( ≥3 clusters, ≥3 sPO2, 80–85 %) or severe ORD (≥3clusters, ≥3 sPO2, <80 %) not responding on positioning and NPA | Tr | 19 % |
Augarten et al. (1990) [43] |
N: 8 MG, Gl, CP | Monitoring of vital parameters, blood gases and weight gains Lateral neck radiographs | Respiratory rates ≥60/min, requirement of ≥60 % O2, PaO2 ≤ 65 mmHg and PaCO2 ≥ 60 mmHg or acidemia, despite positioning | TLA Tr if no improvement after TLA | 38 % |
Benjamin et al. (1991) [19] |
N: 26 MG/RG, Gl iRS, sRS | Pulse oximetry Laryngoscopy before endotracheal intubation | Oxygen saturation <90 % for >10 % of the time not improving by position or NPA | Endotracheal intubation Tr if this fails, to bypass obstruction | 23 % |
Bull et al. (1990) [30] |
N: 21 RS (not specified) iRS, sRS | Modified PSG during 2 h When indicated: nasoendoscopy, airway fluoroscopy, upper GI radiographs and scintiscan and head CT Suggestive/gastroesophageal reflux but normal radiographic studies: pH probe during PSG | ↑ End tidal CO2 or uncorrectable desaturation (<90 % in >5 % of the sleep time or <80 % in 1 % of the sleep time) with 2 L nasal O2
Continued failure to thrive despite nutritional and oxygen supplementation | TLA or Tr | 48 % |
de Buys Roessingh et al. (2007) [29] |
N: 48 MG/RG, Gl, ORD, CP iRS, sRS | Pulse oximetry Serial blood gas (every 2 days) Nasoendoscopy, bronchoscopy, pH probe PSG if monitoring shows bad results | Desaturation < 90 % with clinical evidence of respiratory distress or chronic CO2 retention (BE > 6.5) despite CPAP followed by NPA and palatal plate | TLA, Tr | 0 % |
Caoutte Laberge et al. (1994) [31] |
N: 125 MG/RG, Gl, ORD iRS, sRS | Serial blood gas measurement Oxygen saturation monitoring Modified PSG (according to Freed et al. 1988) | PO2 < 60 mmHg or PCO2 > 50 mmHg | Subperiosteal release of the floor of the mouth musculature or TLA; Tr if no relieve of UAO | 18 % |
Cheng et al. (2010) [32] |
N: 20 MG, Gl, ORD | Continuous oxygen saturation measurement Laryngoscopy and bronchoscopy before MDO Preoperative PSG | Extensive periods of desaturations <90 % not responding on CPAP | MDO + TLA | 30 % |
Cole et al. (2008) [33] |
N: 39 MG, Gl, CP | Weight gain and saturation monitoring | Moderate to severe respiratory distress when nursed side to side or with NPA | No surgical intervention performed | 0 % |
Cruz et al. (1999) [34] |
N: 47 MG, Gl, CP iRS, sRS | PSG, nasoendoscopy Laryngoscopy, and consideration of flexible and or rigid bronchoscopy before invasive treatment Speech/swallow team evaluation using oropharyngeal motility studies | No resolve of the “airway difficulty” with positioning or short-term use of an NPA | TLA Tr in (sub)glottic pathology or other swallowing or neuromuscular difficulties | 43 % |
Dauria et al. (2008) [35] |
N: 9 MG, ORD iRS | Laryngoscopy and bronchoscopy 3D CT before distraction | Failure of positioning or NPA | Tr MDO if no compounding pathology and /or gestational age >39 weeks | 44 % |
Evans et al. (2011) [7] | Literature study | Modified PSG is important in early infancy for CO2 retention in addition to hypoxemia or desaturation, overnight full PSG may have a role when clinical picture is not clear Laryngoscopy and bronchoscopy | No airway stability (abnormal oxygen saturations, carbon dioxide levels, presence of work of breathing and signs of airway obstruction) maintained by positioning or NPA | Temporarily endotracheal intubation TLA/MDO: Single level tongue base obstruction Tr: >1 level of obstruction or not a candidate for TLA/MDO | – |
van den Elzen et al. (2001) [8] | N: 74 MG, CP, Gl iRS, sRS | Continuous pulseoximetry PSG on indication, not routinely performed | Hypoxia (continuous and persistent SpO2 levels <90 %) not responding on positioning or NPA | Endotracheal intubation Tr (if no successful extubation within 4–6 weeks or after repeated intubations) | 15 % |
Freed et al. (1988) [36] |
N: 6 MG, CP, Gl, ORD iRS, sRS | Transcutaneous oxygen and transcutaneous carbon dioxide levels during a minimum of 8 h (range 8–18 h) Modified PSG Studied in lateral, prone and supine position for ≥45 min | Average oxygen levels <60 mmHg and CO2 levels >60 mmHg during ≥8 h Any O2 level <80 % Obstructive episodes on PSG | TLA | 67 % |
Gangopadhyay et al. (2012) [44] | Not mentioned | Continuous pulse oximetry PSG can be a useful tool | Inadequate results on sleep studies and poor weight gain despite positioning, supplemental O2 and NPA | TLA or MDO (both options discussed with parents and team) | Not mentioned |
Gilhooly et al. (1992) [37] |
N: 15 MG, Gl, ORD iRS, sRS | 4-channel PSG including ECG | “Event of obstruction” of ≥15 s during sleep or quiet activity or shorter episodes associated with ↓ HR < 80 BPM or sPO2 < 85 % despite positioning | TLA | 40 % |
Glynn et al. (2011) [20] |
N: 69 MG, Gl, CP iRS, sRS | Nasoendoscopy Continuous oxygen saturation monitoring for 24–36 h Hearing assessment with otoscopy, tympanometry, visual response and pure audiometry Microlaryngobronchoscopy before Tr | SpO2 < 90 % >5 % of the time, despite positioning and NPA | Endotracheal intubation Tr if attempts to extubate fail | 14 % |
Hoffman et al. (2003) [46] |
N: 72 MG, Gl, ORD, CP iRS, sRS | Clinical examination PSG Bronchoscopy | Average transcutaneous O2 < 60 mmHg/CO2 > 50 mmHg, SpO2 < 880 %, and/or obstructive episodes on sleep study despite positioning and supplemental oxygen | TLA Tr for (sub)glottic pathology | 35 % |
Jarrahy et al. (2012) [27] | Literature study | CT scan, manometry, electromyography, 24 h pH monitoring, and nuclear medicine imaging to evaluate presence of reflux Nasoendoscopy pre- and postoperative, “sleep evaluation” | Failure of positioning/NPA orunsuitable airway for a trial of nonsurgical management | Subperiostal floor of mouth release TLA, MDO, Tr | – |
Kochel et al. (2010) [14] |
N: 7 MG, Gl, ORD +/− CP iRS, sRS | Nasoendoscopy Continuous pulse oximetry Blood gas analyses | Clinical signs of respiratory distress (i.e., agitation, dyspnea, tachypnea, intercostal recession, etc.) or oxygen desaturation or respiratory acidosis in blood gas analyses | Orthopedic oral appliance with/without extension (posterior, extra oral or pharyngeal tube) | 100 % |
Van Lieshout et al. (2013) [38] |
N: 59 MG/RG, ORD iRS, sRS | PSG (in all infants with ORD despite prone positioning or with persistent feeding difficulties) Nasoendoscopy on indication | Failure of prone positioning and respiratory support (NPA, CPAP, and/or oxygen supplementation) | Tr and/or MDO | 7 % |
Mackay et al. (2011) [16] | Literature study | Evaluation of desaturation occurring spontaneously, during feeding and sleep Nasoendoscopy, bronchoscopy PSG, pH monitoring, CT scan and cephalometrics | Persistent obstruction despite positioning or NPA | TLA MDO (if TLA fails) Tr (if MDO fails) | – |
Marques et al. (2000) [49] |
N: 62 RG, Gl, ORD iRS, sRS | Nasoendoscopy Continuous pulse oxymetry | SpO2 < 90 %, increasing respiratory effort and/or no removal of NG tube possible despite NPA within 15 days | TLA (type 1 obstruction) Tr (type 3 or 4 obstruction, or no improvement after TLA/NPA) | 35 % |
Poets and Bacher (2011) [9] | Literature study | Clinical observation PSG | Significant UAO during sleep, defined as a mixed-obstructive apnea index (MAOI) > 3 in a sleep study | Pre-epiglottic baton plate | – |
N: 21 max–min. discrepancy of >3 mm, Gl, +/− CP | Pulse oximetry for ≥12 h, PSG (continuous monitoring oxygen saturation, end-tidal CO2 and EEG during sleep), nasoendoscopy and bronchoscopy before invasive intervention | Any single saturation below the 80 % or PO2 < 90 % for >5 % of the monitored time despite positioning | TLA MDO (if TLA fails) Tr if no response to TLA/MDO or (infra)glottic problem present | 57 % | |
Scott et al. (2012) [28] | Literature study | Nasoendoscopy PSG if no life threatening airway compromise is present Serial capillary blood gases (to document a trend of elevated or increasing carbon dioxide levels) Continuous-pulse oximetry and cardiac monitoring | Signs of upper airway obstruction despite prone- or side positioning or NPA | TLA, Tr, MDO | – |
Thouvenin et al. (2013) [48] |
N: 141 RG, Gl, CP iRS, sRS | Continuous monitoring of cardiac and respiratory rhythms, regularly check of transcutaneous PO2 and PCO2 levels. PSG on indication Karyotype assay, echocardiography, skeletal radiography, ophthalmologic examination | Oxygen saturation < 90 % for >5 % of the time or saturations < 80 % not responding on positional changes or NPA | Tr | Not mentioned |
Tomaski et al. (1995) [47] |
N: 90 MG, Gl, CP iRS, sRS | Flexible fiberoptic nasopharyngolaryngoscopy, cardiac and pulmonary evaluation, chest radiogram, electrocardiogram, ophthalmologic and genetics consultation PSG, continuous pulse oximetry, and apnea monitoring Pre-op: lateral X-ray, rigid direct laryngoscopy and bronchoscopy | Positioning and NPA are not successful in relieving airway obstruction | Tr | 12 % |
Wagener et al. (2003) [39] |
N: 22 MG, ORD, Gl, CP iRS, sRS | Continuous oxygen saturation monitoring | Severe UAO (cyanotic attack, transcutaneous oxygenation > 90 %, PCO2 < 50 mmHg) not responding on positioning or NPA | No surgical intervention necessary | 0 % |
Vyas et al. (2008) [40] Kohan et al. (2010) [45] |
N: 149 MG, ORD iRS, sRS | PSG Radionuclide milk scan (severity of gastroesophogeal reflux and gastric emptying) with 24-h pH probe (in indeterminate results) and laryngobronchoscopy | Intubation at birth necessary, failed extubation or failed conservative treatment (prone positioning or NPA) | MDO Tr if: 1. Central apnea 2. Severe gastroesophageal reflux 3. Other airway lesions | 78 % |