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Obstructive sleep apnea syndrome in children with 22q11.2 deletion syndrome after operative intervention for velopharyngeal insufficiency.

Crockett DJ, Goudy SL, Chinnadurai S, Wootten CT - Front Pediatr (2014)

Bottom Line: We hypothesize that 22q11.2 deletion patients are at greater risk of obstructive sleep apnea (OSA) after surgical correction of VPI, due, in part, to their functional hypotonia, large velopharyngeal gap size, and the need to surgically obstruct the velopharynx.Monitoring for OSA should be considered after surgical correction of VPI due to a high occurrence in this population.Furthermore, families should be counseled of the risk of OSA after surgery and the potential need for treatment with CPAP.

View Article: PubMed Central - PubMed

Affiliation: Department of Otolaryngology, Vanderbilt University Medical Center , Nashville, TN , USA.

ABSTRACT

Introduction: Surgical treatment of velopharyngeal insufficiency (VPI) in 22q11.2 deletion syndrome is often warranted. In this patient population, VPI is characterized by poor palatal elevation and muscular hypotonia with an intact palate. We hypothesize that 22q11.2 deletion patients are at greater risk of obstructive sleep apnea (OSA) after surgical correction of VPI, due, in part, to their functional hypotonia, large velopharyngeal gap size, and the need to surgically obstruct the velopharynx.

Methods: We performed a retrospective analysis of patients with 22q11.2 deletion syndrome treated at a tertiary pediatric hospital between the years of 2002 and 2012. The incidence of VPI, need for surgery, post-operative polysomnogram, post-operative VPI assessment, and OSA treatments were evaluated.

Results: Forty-three patients (18 males, 25 females, ages 1-14 years) fitting the inclusion criteria were identified. Twenty-eight patients were evaluated by speech pathology due to hypernasality. Twenty-one patients had insufficient velopharyngeal function and required surgery. Fifteen underwent pharyngeal flap surgery, three underwent sphincter pharyngoplasty, two underwent Furlow palatoplasty, and one underwent combined sphincter pharyngoplasty with Furlow palatoplasty. Of these, eight had post-operative snoring. Six of these underwent polysomnography (five underwent pharyngeal flap surgeries and one underwent sphincter pharyngoplasty). Four patients were found to have OSA based on the results of the polysomnography (average apnea/hypopnea index of 4.9 events/h, median = 5.1, SD = 2.1). Two required continuous positive airway pressure (CPAP) due to moderate OSA.

Conclusion: Surgery is often necessary to correct VPI in patients with 22q11.2 deletion syndrome. Monitoring for OSA should be considered after surgical correction of VPI due to a high occurrence in this population. Furthermore, families should be counseled of the risk of OSA after surgery and the potential need for treatment with CPAP.

No MeSH data available.


Related in: MedlinePlus

Image of a persistent velopharyngeal gap causing velopharyngeal insufficiency in a patient with a submucous cleft palate undergoing video nasopharyngeal endoscopy (PPW = posterior pharyngeal wall, VPG = velopharyngeal gap, SP = soft palate).
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Figure 1: Image of a persistent velopharyngeal gap causing velopharyngeal insufficiency in a patient with a submucous cleft palate undergoing video nasopharyngeal endoscopy (PPW = posterior pharyngeal wall, VPG = velopharyngeal gap, SP = soft palate).

Mentions: The incidence of VPI in patients with 22q11.2 deletion syndrome ranges from 27% of patients to 80% (7, 8). Poor functioning of the velopharyngeal mechanism may be due to various anatomic and functional reasons. Overt cleft palate and submucous cleft palate are infrequently identified in patients with this syndrome and are known to be associated with VPI (Figure 1) (9). Generalized muscular hypotonia, also, often affects the muscles of the palate, contributing to a poor velopharyngeal mechanism.


Obstructive sleep apnea syndrome in children with 22q11.2 deletion syndrome after operative intervention for velopharyngeal insufficiency.

Crockett DJ, Goudy SL, Chinnadurai S, Wootten CT - Front Pediatr (2014)

Image of a persistent velopharyngeal gap causing velopharyngeal insufficiency in a patient with a submucous cleft palate undergoing video nasopharyngeal endoscopy (PPW = posterior pharyngeal wall, VPG = velopharyngeal gap, SP = soft palate).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4127662&req=5

Figure 1: Image of a persistent velopharyngeal gap causing velopharyngeal insufficiency in a patient with a submucous cleft palate undergoing video nasopharyngeal endoscopy (PPW = posterior pharyngeal wall, VPG = velopharyngeal gap, SP = soft palate).
Mentions: The incidence of VPI in patients with 22q11.2 deletion syndrome ranges from 27% of patients to 80% (7, 8). Poor functioning of the velopharyngeal mechanism may be due to various anatomic and functional reasons. Overt cleft palate and submucous cleft palate are infrequently identified in patients with this syndrome and are known to be associated with VPI (Figure 1) (9). Generalized muscular hypotonia, also, often affects the muscles of the palate, contributing to a poor velopharyngeal mechanism.

Bottom Line: We hypothesize that 22q11.2 deletion patients are at greater risk of obstructive sleep apnea (OSA) after surgical correction of VPI, due, in part, to their functional hypotonia, large velopharyngeal gap size, and the need to surgically obstruct the velopharynx.Monitoring for OSA should be considered after surgical correction of VPI due to a high occurrence in this population.Furthermore, families should be counseled of the risk of OSA after surgery and the potential need for treatment with CPAP.

View Article: PubMed Central - PubMed

Affiliation: Department of Otolaryngology, Vanderbilt University Medical Center , Nashville, TN , USA.

ABSTRACT

Introduction: Surgical treatment of velopharyngeal insufficiency (VPI) in 22q11.2 deletion syndrome is often warranted. In this patient population, VPI is characterized by poor palatal elevation and muscular hypotonia with an intact palate. We hypothesize that 22q11.2 deletion patients are at greater risk of obstructive sleep apnea (OSA) after surgical correction of VPI, due, in part, to their functional hypotonia, large velopharyngeal gap size, and the need to surgically obstruct the velopharynx.

Methods: We performed a retrospective analysis of patients with 22q11.2 deletion syndrome treated at a tertiary pediatric hospital between the years of 2002 and 2012. The incidence of VPI, need for surgery, post-operative polysomnogram, post-operative VPI assessment, and OSA treatments were evaluated.

Results: Forty-three patients (18 males, 25 females, ages 1-14 years) fitting the inclusion criteria were identified. Twenty-eight patients were evaluated by speech pathology due to hypernasality. Twenty-one patients had insufficient velopharyngeal function and required surgery. Fifteen underwent pharyngeal flap surgery, three underwent sphincter pharyngoplasty, two underwent Furlow palatoplasty, and one underwent combined sphincter pharyngoplasty with Furlow palatoplasty. Of these, eight had post-operative snoring. Six of these underwent polysomnography (five underwent pharyngeal flap surgeries and one underwent sphincter pharyngoplasty). Four patients were found to have OSA based on the results of the polysomnography (average apnea/hypopnea index of 4.9 events/h, median = 5.1, SD = 2.1). Two required continuous positive airway pressure (CPAP) due to moderate OSA.

Conclusion: Surgery is often necessary to correct VPI in patients with 22q11.2 deletion syndrome. Monitoring for OSA should be considered after surgical correction of VPI due to a high occurrence in this population. Furthermore, families should be counseled of the risk of OSA after surgery and the potential need for treatment with CPAP.

No MeSH data available.


Related in: MedlinePlus