Airway Obstruction and the Unilateral Cleft Lip and Palate Deformity

Abstract BACKGROUND Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis. OBJECTIVE To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue. METHODS A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac. RESULTS We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula. CONCLUSION The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.

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A cephalometric study of patients with and without cleft lip and palate undergoing orthognathic surgery: Implications for airway obstruction

Journal of Oral and Maxillofacial Surgery ◽

10.1016/0278-2391(91)90634-x ◽

1991 ◽

Vol 49 (8) ◽

pp. 92-93

Author(s):

Amy Lyn Zuker

Keyword(s):

Airway Obstruction ◽

Orthognathic Surgery ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

Cephalometric Study

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Atrial Septal Defect in Cyanotic CL/Fr Mice

The Cleft Palate-Craniofacial Journal ◽

10.1597/1545-1569_1998_035_0058_asdicc_2.3.co_2 ◽

1998 ◽

Vol 35 (1) ◽

pp. 58-64

Author(s):

Shingo Kadowaki ◽

Michiko Sakamoto ◽

Hiroshi Kamiishi ◽

Takashi Tanimura

Keyword(s):

Atrial Septal Defect ◽

Airway Obstruction ◽

Cleft Lip ◽

Septal Defect ◽

Cleft Lip And Palate ◽

Atrial Septum ◽

Palatal Shelf ◽

Icr Mice ◽

Septum Primum ◽

Septal Development

Objective It is widely known that some newborn CL/Fr mice with cleft lip and palate (CLP) also have cyanotic symptoms, which have been thought to depend on an atrial septal defect (ASD). In a previous study, we found that cyanotic mice tended to have more severe types of CLP. We hypothesize that the mechanical airway obstruction due to a poorly developed palatal shelf and unmoved tongue in CLP(+) mice might be related to the occurrence of cyanosis. The purpose of this study was to examine the relationships between ASD and cyanosis in CLP(+) newborns. Method The newborn hearts from CLP(-), noncyanotic CLP(+), cyanotic CLP(+), CL/Fr mice and ICR mice were examined histologically, and the incidence and size of ASD was determined on neonatal day (ND) 0. In CLP(-) newborns, similar procedures were performed from ND 1 to ND 4. Furthermore, in CLP(+) newborns, development of the palatal shelf was examined. Results While all the ICR mice had a well-developed atrial septum, and the incidence of ASD was 0%, about 80% of CL/Fr mice had ASD, irrespective of the presence or absence of CLP and cyanosis. On ND 0, the septum primum was significantly shorter in cyanotic CLP(+) mice than in CLP(-) mice. It also tended to be shorter in CLP(+) mice than in CLP(-) mice. Between the cyanotics and noncyanotics, there were no significant differences in the incidences of ASD and the rate of septal development. In CLP(-) mice, the septum primum developed well later and no ASD was observed on ND 4. Cyanotic newborns had significantly less developed palatal shelves than did noncyanotics. Conclusions Cyanosis may not be related to ASD and the rate of septal development, but may be related to the occurrence of CLP in this strain. Furthermore, we confirmed that some relationship exists between the development of the palatal shelf and cyanosis. The present study supports our hypothesis concerning the cause of cyanosis in CL/Fr mice.

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Craniofacial, Craniocervical, and Pharyngeal Morphology in Bilateral Cleft Lip and Palate and Obstructive Sleep Apnea Patients

The Cleft Palate-Craniofacial Journal ◽

10.1597/05-175 ◽

2007 ◽

Vol 44 (1) ◽

pp. 1-7 ◽

Cited By ~ 31

Author(s):

Barbara C. M. Oosterkamp ◽

Hendrik J. Remmelink ◽

Gerard J. Pruim ◽

Aarnoud Hoekema ◽

Pieter U. Dijkstra

Keyword(s):

Obstructive Sleep Apnea ◽

Sleep Apnea ◽

Airway Obstruction ◽

Hyoid Bone ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

Reference Group ◽

Obstructive Sleep ◽

Lateral Cephalograms ◽

Bilateral Cleft Lip

Objective: The aim of this study was to analyze craniofacial, craniocervical, and pharyngeal morphology in surgically treated bilateral cleft lip and palate (BCLP) men, untreated men with obstructive sleep apnea (OSA), and a reference group of men. Subjects and methods: Lateral cephalograms were obtained of 27 male BCLP patients (mean age 29.0 ± 8.3 years), 27 untreated male OSA patients (mean age 38.6 ± 5.3 years), and 27 male controls serving as a reference group (mean age 30.8 ± 9.2 years). Tracings were made, and 26 variables representing craniofacial, craniocervical, and pharyngeal dimensions were obtained using Viewbox 3.1.1.6. software. The groups were compared using a one-way analysis of variance. Results: Craniofacial, craniocervical, and pharyngeal morphology of BCLP and OSA patients was similar except for a significantly more retrusive maxilla in the BCLP group. Compared to the reference group, the BCLP and OSA groups had significantly larger craniocervical angulations, smaller depth of the oropharynx at the tip of the velum, and a more inferiorly positioned hyoid bone. Significantly larger vertical dimensions were found in the BCLP group compared to the reference group. Conclusions: Craniofacial, craniocervical, and pharyngeal morphology of BCLP and OSA patients demonstrate substantial similarities except for a significantly more retrusive maxilla in the BCLP group. It is suggested that airway obstruction and postural adaptation to the obstruction may possibly be related to the aberrant craniofacial, craniocervical, and pharyngeal morphology in OSA and in BCLP patients.

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Treating Velopharyngeal Inadequacy Using Bilateral Buccal Flap Revision Palatoplasty

Perspectives of the ASHA Special Interest Groups ◽

10.1044/2019_pers-sig5-2019-0005 ◽

2019 ◽

Vol 4 (5) ◽

pp. 878-892

Author(s):

Joseph A. Napoli ◽

Linda D. Vallino

Keyword(s):

Systematic Review ◽

Obstructive Sleep Apnea ◽

Sleep Apnea ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

A Priori ◽

Sufficient Evidence ◽

Obstructive Sleep ◽

Before And After ◽

Buccal Flap

Purpose The 2 most commonly used operations to treat velopharyngeal inadequacy (VPI) are superiorly based pharyngeal flap and sphincter pharyngoplasty, both of which may result in hyponasal speech and airway obstruction. The purpose of this article is to (a) describe the bilateral buccal flap revision palatoplasty (BBFRP) as an alternative technique to manage VPI while minimizing these risks and (b) conduct a systematic review of the evidence of BBFRP on speech and other clinical outcomes. A report comparing the speech of a child with hypernasality before and after BBFRP is presented. Method A review of databases was conducted for studies of buccal flaps to treat VPI. Using the principles of a systematic review, the articles were read, and data were abstracted for study characteristics that were developed a priori. With respect to the case report, speech and instrumental data from a child with repaired cleft lip and palate and hypernasal speech were collected and analyzed before and after surgery. Results Eight articles were included in the analysis. The results were positive, and the evidence is in favor of BBFRP in improving velopharyngeal function, while minimizing the risk of hyponasal speech and obstructive sleep apnea. Before surgery, the child's speech was characterized by moderate hypernasality, and after surgery, it was judged to be within normal limits. Conclusion Based on clinical experience and results from the systematic review, there is sufficient evidence that the buccal flap is effective in improving resonance and minimizing obstructive sleep apnea. We recommend BBFRP as another approach in selected patients to manage VPI. Supplemental Material https://doi.org/10.23641/asha.9919352

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