Influence of Maxillary Constriction on Nasal Resistance and Sleep Apnea Severity in Patients With Marfan's Syndrome

doi:10.1378/chest.110.5.1184

Chest

Volume 110, Issue 5, November 1996, Pages 1184-1188

https://doi.org/10.1378/chest.110.5.1184 Get rights and content

Background

Marfan's syndrome is associated with a high prevalence of obstructive sleep apnea (OSA). As this syndrome is associated with a characteristic constricted maxilla and high-arched palate, we reasoned that nasal airway constriction and resultant high nasal airway resistance (NAR) may contribute to the development of OSA. Therefore, the aim of this study was to measure NAR in patients with Marfan's syndrome. In addition, we aimed to examine the influence of maxillary morphology on both NAR and the severity of OSA.

Method

We measured NAR in 13 consecutive patients with Marfan's syndrome and 13 control subjects. NAR was measured by posterior rhinomanometry, and expressed as the inspiratory resistance at a flow of 0.5 L/s. Dental impressions were taken to evaluate maxillary arch morphology, allowing measurement of the following distances: intercuspid (ICD), interpremolar (IPD), intermolar (IMD), and maximum hard palate height (MPH). Ten of the patients and four of the control subjects had previously undergone nocturnal polysomnography.

Results

Mean NAR for the Marfan group was more than twice that in the control group (7.7±1.2 vs 2.9±0.4 cm H₂O/L/s; p<0.005). The patients also had marked constriction of the maxillary arch compared with control subjects. Two of the lateral maxillary measurements were significantly inversely correlated with NAR. There were significant correlations between various maxillary arch measurements (MPH/ICD, MPH/IPD, MPH/IMD) and the apnea/hypopnea index.

Conclusion

These data suggest that high NAR is a common feature of Marfan's syndrome. Maxillary constriction with a relatively high hard palate appears to be a major reason for the high NAR. The significant correlations between indexes of maxillary constriction and sleep apnea severity suggest that maxillary morphology may play an important role in the pathophysiology of OSA in Marfan's syndrome.

Section snippets

Subjects

Thirteen consecutive patients with Marfan's syndrome presenting for routine follow-up at the Marfan Clinic at our institution were recruited for the study. They were compared with an equivalent number of normal control subjects who were recruited from the hospital staff. None of the patients or control subjects had evidence of overt acute nasal abnormalities (eg, infection, allergic rhinitis) or marked septal deviations; nor did they use nasal decongestants on a regular basis. Informed consent

RESULTS

The anthropometric features of patients and control subjects are summarized in Table 1. The study population was relatively young, in keeping with the epidemiology of Marfan's syndrome. There were no significant differences in age, height, or weight between patients and control subjects. There was a difference in sex ratio between the two groups; 4 of the 13 Marfan patients were male, in contrast to 8 of the control subjects. However, this difference was not statistically significant (χ²=2.4;

DISCUSSION

The main aim of this study was to examine whether nasal resistance is elevated in patients with Marfan's syndrome. A group of 13 consecutive patients with Marfan's syndrome were evaluated, and they were indeed found to have significantly higher NAR than matched control subjects. The normal range for NAR is thought to be between 1.0 and 3.5 cm H₂O/L/s (at a flow of 0.5 L/s), with a mean of approximately 2.5 cm H₂O/L/s.11, 12 The subjects used as control subjects in this study had similar NAR to

ACKNOWLEDGMENTS

The authors thank the patients and control subjects who participated in this study. The support of the Marfan Clinic, and Dr. R. Jeremy in particular, was greatly appreciated.

REFERENCES (24)

HirstAE et al.
Marfan's syndrome: a review
Prog Cardiovasc Dis
(1973)
CistulliPA et al.
Sleep disorders in Marfan's syndrome [letter]
Lancet
(1991)
CistulliPA et al.
Sleep apnea in Marfan's syndrome: increased upper airway collapsibility during sleep
Chest
(1995)
WarrenDW et al.
The relationship between nasal airway cross-sectional area and nasal resistance
Am J Orthod Dentofacial Orthop
(1987)
HersheyHG et al.
Changes in nasal airway resistance associated with rapid maxillary expansion
Am J Orthod
(1976)
PartinenM et al.
Obstructive sleep apnea and cephalometric roentgenograms: the role of anatomic upper airway abnormalities in the definition of abnormal breathing during sleep
Chest
(1988)
HarvoldEP et al.
Primate experiments on oral sensation and dental malocclusion
Am J Orthod
(1973)
GuilleminaultC et al.
Familial aggregates in obstructive sleep apnea syndrome
Chest
(1995)
PyeritzRE et al.
The Marfan syndrome: diagnosis and management
N Engl J Med
(1979)
PyeritzRE.
The Marfan syndrome
Am Fam Physician
(1986)

CistulliPA et al.

Sleep-disordered breathing in Marfan's syndrome

Am Rev Respir Dis

(1993)

GrunsteinRR et al.

Snoring and sleep apnoea in men: association with central obesity and hypertension

Int J Obes

(1993)

Cited by (63)

Distraction Osteogenesis Maxillary Expansion (DOME) for adult obstructive sleep apnea patients with narrow maxilla and nasal floor
2020, Sleep Medicine
This study correlates objective and subjective measurements associated with obstructive sleep apnea (OSA) to define the efficacy of Distraction Osteogenesis Maxillary Expansion (DOME) to treat adult OSA patients with narrow maxilla and nasal floor.
This is a retrospective study reviewing cases from September 2014 through April 2018 with 75 eligible subjects. Inclusion criteria required OSA confirmed by attended polysomnography (PSG). Pre- and Post-operative clinical data were measured at the Stanford Sleep Medicine and Stanford Sleep Surgery Clinics. DOME is a two-step process starting with insertion of custom-fabricated maxillary expanders anchored to the hard palate by mini-implants followed by minimally invasive osteotomies. After maxillary expansion was complete, orthodontic treatment to restore normal occlusion was initiated. Perioperative Apnea-Hypopnea Index (AHI), Epworth Sleepiness Scale (ESS), Nasal Obstruction Symptom Evaluation (NOSE), and Oxygen Desaturation Index (ODI) were measured for 43, 72, 72, and 34 subjects respectively. Statistical analysis was performed using paired T-test with significance set at p-value < 0.05.
The mean age of test subjects was 30.5 ± 8.5 years with a gender distribution of 57 males and 18 females. There was a significant reduction in pre and post-operative NOSE score (10.94 ± 5.51 to 3.28 ± 2.89, p < 0.0001), mean ESS score (10.48 ± 5.4 to 6.69 ± 4.75, p < 0.0001), and AHI (17.65 ± 19.30 to 8.17 ± 8.47, p < 0.0001) with an increased percentage of REM sleep (14.4 ± 8.3% to 22.7 ± 6.6%, p = 0.0014). No significant adverse effects were identified.
DOME treatment reduced the severity of OSA, refractory nasal obstruction, daytime somnolence, and increased the percentage of REM sleep in this selected cohort of adults OSA patients with narrow maxilla and nasal floor.
Distraction osteogenesis maxillary expansion (DOME) for adult obstructive sleep apnea patients
2019, Sleep Apnea and Snoring: Surgical and Non-Surgical Therapy
Obstructive sleep apnea in children with Marfan syndrome: Relationships between three-dimensional palatal morphology and apnea-hypopnea index
2018, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
They found that the shorter intermolar distance and the greater palatal height in adults with Marfan's syndrome, when compared with control subjects, showed no correlation with OSA severity, assessed by AHI. On the contrary Cistulli et al. suggested that maxillary morphology may play an important role in the pathophysiology of OSA in Marfan's syndrome [9,16]. They always evaluated a group of adults with Marfan's syndrome but they measured the nasal airflow resistance (NAR) and no AHI.
To evaluate the relationship between the severity degree of OSA (apnea/hypopnea index AHI>1) and palatal area and volume, measured by 3D analysis of digital casts in Marfan children.
Twenty children with a clinical diagnosis of MS were recruited from a tertiary medical center. All the subjects underwent standard nocturnal polygraphy testing. Sixteen Marfan patients (7F,9 M; mean age 8.8yy ± 1.5yy) with AHI>1 were enrolled. Marfan Group (MG) was compared with a control group (CG) of 17 children without Marfan syndrome (9F,8 M; mean age 8.5yy ± 1.7yy) presenting with nose-breathing pattern. For each subject maxillary digital casts were taken and palatal area and volume were measured. Unpaired t-test was used to test significant differences between MG and CG for area and volume measurements. Pearson correlation coefficient (PCC) was used to measure the linear correlation between the degree of OSA (AHI index) and palatal volume and palatal area.
80% of Marfan children presented an AHI>1 and a diagnosis of OSA. MG presented statistically significant lower values of palatal surface area (662.68 mm²; P < 0.0001) and palatal volume (2578.1 mm³; P < 0.0001) with respect to CG (923.0 mm² and 3756.6 mm³, respectively). Correlation analysis showed that AHI index had no linear correlation with palatal area (r = - 0,07) and with palatal volume (r = − 0,11).
OSA is highly prevalent in children with Marfan's syndrome (80%). Marfan children present a reduction of palatal area and volume when compared to healthy subjects. OSA in Marfan children is not linear correlated to the palatal morphology and it shows a multifactorial aetiology.
Maxillomandibular advancement for obstructive sleep apnea syndrome treatment: Long-term results
2017, Journal of Cranio-Maxillofacial Surgery
We evaluated the long term effectiveness of maxillomandibular advancement (MMA) for OSAS treatment patients. This retrospective study reviewed the patients treated by MMA for OSAS between 1995 and 2009. They were evaluated by complete polysomnography, cephalometry and quality of life questionnaire. The minimum follow up was 3 years. Success rate was defined by an IAH <10 with at least a 50% reduction.
88 patients had MMA during this period. 34 accepted the evaluation (28 men, 6 women). Mean age was 52.4 ± 14. Mean follow-up was 12.5 years.
Long term success rate was 28% for the entire group, postoperative IAH was reduced between 50 and 80% for all the patients except one. Success rate was 100% for young patients (age < 45), with BMI <25 and IAH <45 and SNB <75° and narrow retrobasinlingual space (<8 mm) and with preoperative orthodontics. Esthetic and sleep results were better with a moderate maxillary advancement and anterior impaction. There was no skeletal relapse. The major postoperative complication was inferior alveolar nerve hypoesthesia.
MMA is an effective and stable treatment of OSAS in selected patients. Preoperative orthodontics is recommended.
Enlargement of the Pharynx Resulting from Surgically Assisted Rapid Maxillary Expansion
2016, Journal of Oral and Maxillofacial Surgery
Given that transverse maxillary deficiency is an etiologic factor of obstructive sleep apnea and is intimately connected to pharyngeal size, the objective of this study was to determine whether surgically assisted rapid maxillary expansion (SARME) would promote pharyngeal enlargement in adults.
This prospective study was conducted in patients with uni- or bilateral posterior crossbite who underwent SARME. Participants were recruited from the Integrated Center for the Study of Face Defects, School of Medicine of Ribeirão Preto, University of São Paulo (São Paulo, Brazil). All patients underwent computed tomography of the pharynx before and after surgery (171.5 days on average), and the sagittal and transverse planes and the total area across 3 levels of the pharynx, including the upper (posterior nasal spine), middle (first cervical vertebra), and lower (second cervical vertebra) levels, were measured on the images. A paired-samples t test was used to evaluate changes in the pharynx before and after surgery.
The studied sample consisted of 18 adult patients (10 women and 8 men) with an average age of 37.11 years (standard deviation, 11.73 yr); all patients resided in the region of Ribeirão Preto, São Paulo, Brazil. No statistical changes were observed in the upper level. An enlargement of 17.82% (P = .0107) was observed in the sagittal plane of the middle level. The cross-sectional and area values of this same portion were enlarged (16.96 and 37.38%, respectively), with a trend toward statistical significance (P = .067 and .051, respectively). The airway enlargements in the lower level were 26.41, 24.87, and 53.87% in the sagittal and transverse planes and total area, respectively; these differences were statistically significant (P = .0003, .0033, and .0016, respectively) for all 3 measurements.
SARME promotes pharyngeal enlargement, especially in the lower levels of the pharynx.
Modified maxillomandibular advancement for obstructive sleep apnoea: Towards a better outcome for Asians
2015, International Journal of Oral and Maxillofacial Surgery
Excluding tracheostomy, maxillomandibular advancement (MMA) is the most effective surgical treatment for obstructive sleep apnoea (OSA). However, the anticipated facial changes may prevent acceptance of this procedure by patients with bimaxillary protrusion, a common feature of Asian faces. We therefore developed a modified MMA technique for such cases, consisting of anterior segmental osteotomies together with standard Le Fort I and bilateral sagittal split osteotomies. A prospective study of 20 consecutive Taiwanese adults with moderate-to-severe OSA who underwent modified MMA and postsurgical orthodontics was undertaken to evaluate the efficacy with regard to OSA and the postoperative facial appearance and dental occlusion. After modified MMA, the mean apnoea–hypopnoea index decreased from 41.6 ± 19.2 n/h to 5.3 ± 4.0 n/h (P < 0.001). All patients had a successful outcome. No patient was dissatisfied with their postoperative facial appearance. The mean Peer Assessment Rating score decreased from 21.9 ± 14.3 to 1.7 ± 1.6 (P = 0.001). The data suggest that the modified MMA is effective in treating patients with moderate-to-severe OSA without negatively affecting facial appearance or dental occlusion. To achieve a better outcome, surgical–orthodontic integration is warranted. The surgery-first approach can achieve early improvement.

View all citing articles on Scopus

: Supported by a National Health and Medical Research Council of Australia Scholarship and the Australian Lung Foundation/Sensormedics Fellowship in Sleep Disorders (Dr. Cistulli).

View full text

Chest

Sleep: Clinical InvestigationsInfluence of Maxillary Constriction on Nasal Resistance and Sleep Apnea Severity in Patients With Marfan's Syndrome

Background

Method

Results

Conclusion

Section snippets

Subjects

RESULTS

DISCUSSION

ACKNOWLEDGMENTS

Prog Cardiovasc Dis

Lancet

Chest

Am J Orthod Dentofacial Orthop

Am J Orthod

Chest

Am J Orthod

Chest

The Marfan syndrome: diagnosis and management

N Engl J Med

The Marfan syndrome

Am Fam Physician

Sleep-disordered breathing in Marfan's syndrome

Am Rev Respir Dis

Snoring and sleep apnoea in men: association with central obesity and hypertension

Int J Obes

Sleep: Clinical Investigations
Influence of Maxillary Constriction on Nasal Resistance and Sleep Apnea Severity in Patients With Marfan's Syndrome