Uvular Malformation in the Presence of Deformational Plagiocephaly

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Uvular Malformation in the Presence of Deformational Plagiocephaly CLINICAL STUDY Uvular Malformation in the Presence of Deformational Plagiocephaly Kaete Archer, MD,Ã Eileen Marrinan, MS, CCC,Ã Susan Stearns, PhD,y and Sherard Tatum, MDÃ Background: Deformational plagiocephaly is cranial asymmetry population. This is the first report of uvular malformation in the caused by external forces on the skull. Deformational plagiocephaly presence of deformational plagiocephaly. is seen in 5% to 48% of healthy newborns. Incomplete uvular fusion, in contrast, is one of many uvular malformations. The Key Words: Uvular malformation, deformational plagiocephaly incidence of all degrees of incomplete uvular fusion is approxi- (J Craniofac Surg 2015;26: 836–839) mately 1% in healthy children. Bifid uvula is a malformation that is often considered a microform cleft palate or a marker for sub- mucous cleft palate. Methods: This is a retrospective study of patients with deformational Deformational Plagiocephaly plagiocephaly seen at the Upstate Cleft and Craniofacial Center Plagiocephaly is a general term used to describe cranial asym- between January 1, 2006, and September 30, 2011. Patients were metry. It is derived from the Greek words plagios, meaning 1 identified by the International Classification of Diseases, Ninth ‘‘oblique’’, and kephaleˆ, meaning ‘‘head’’. Deformational plagio- Revision code for plagiocephaly. Seventy-nine patients were cephaly is caused by intrauterine and/or postnatal external forces that deform the skull. In contrast, synostotic plagiocephaly is caused excluded with craniosynostosis and syndromic diagnoses. One by premature fusion of the cranial sutures.2 Deformational plagi- hundred forty-six patients with deformational plagiocephaly were ocephaly is more common than synostotic plagiocephaly, with a included in the study. Data were collected for sex, age at presentation, prevalence of 5% to 48% of healthy newborns.1 The diagnosis of parity, multiple births, delivery, oligohydramnios, cephalohema- posterior deformational plagiocephaly can often be made by toma, uterine abnormalities, fetal position, and intrauterine growth physical examination alone. It is characterized by varying degrees restriction. Clinical findings were collected including location of of abnormal cranial features including asymmetrical parallelogram cranial flattening and uvular malformations. skull shape, unilateral parieto-occipital flattening, ipsilateral frontal Results: Twenty-four of 146 patients with deformational plagio- bossing, and anterior displacement of the ipsilateral ear (Fig. 1).3 cephaly had incomplete fusion of the uvula ranging from complete bifid uvula to a notched uvular tip (16.4%). This association was Risk Factors statistically significant (odds ratio, 18; 95% confidence interval, Multiple risk factors for deformational plagiocephaly have 11.1–28.9). Most patients (62.3%) were male. We recorded primi- previously been identified. Peitsch et al4 identified assisted vaginal parity (44.5%), multiple births (17.1%), vacuum-assisted delivery delivery, prolonged labor, birth position, primiparity, and male sex 5 (6.2%), cesarean section (36.3%), oligohydramnios (4.1%), uterine as risk factors. Kane et al reported that deformational plagioce- abnormalities (2.1%), abnormal fetal position (3.4%), and intra- phaly is associated with fetal constraint: small uterus, large fetus, oligohydramnios, and multiple-birth pregnancies. uterine growth restriction (1.4%). Ten of the 24 patients with After reports that infants who slept in the prone position were at plagio-cephaly and uvular malformation were seen for an initial an increased risk of sudden infant death syndrome, the American consultation only in our chart system. Of the remaining 14 patients Academy of Pediatrics (AAP) Task Force on Infant Positioning and with follow-up, none had recorded signs or symptoms of velopharyn- Sudden Infant Death Syndrome formally recommended that healthy geal insufficiency. infants be placed supine or on their sides.6 The Back to Sleep Conclusions: The incidence of incomplete uvular fusion in infants Campaign has been one of the most successful public health with deformational plagiocephaly is 16.4%, which is significantly initiatives. After this campaign, the incidence of prone sleeping higher than the approximate 1% incidence reported in the general decreased from 70% in 1992 to 17% in 1998, and the incidence of sudden infant death syndrome decreased from a rate of 1.2 cases per 1000 live births in 1992 to 0.72 cases per 1000 live births in 1998.7,8 From the ÃDepartment of Otolaryngology and Communication Sciences; A tertiary care craniofacial center reported a 5- to 6-fold increase in and yDepartment of Cell and Development Biology, State University of referrals for deformational plagiocephaly between 1992 and 1994 New York (SUNY) Upstate Medical University, Syracuse, NY. compared to the previous 13 years, suggesting an association with Received July 16, 2014. the new AAP recommendations for infant positioning.5 Accepted for publication January 15, 2015. Address correspondence and reprint requests to Kaete Archer, MD, Department of Otolaryngology and Communication Sciences, SUNY Uvular Malformations Upstate Medical University, 750 E Adams St, Syracuse, NY 13210; Bifid uvula is a uvular malformation that is often considered a E-mail: [email protected] microform cleft palate or a marker for submucous cleft palate This manuscript was presented at the COSM/AAFPRS meeting in San (SMCP). A complete bifid uvula is less common than incomplete Diego, CA, on April 18, 2012. (partial) fusion of the uvula.9 Other uvular malformations include a The authors report no conflicts of interest. # broad uvula and an abortive cleft uvula. The incidence of incom- Copyright 2015 by Mutaz B. Habal, MD 9,10 ISSN: 1049-2275 plete uvular fusion or bifid uvula is approximately 1%. Meskin DOI: 10.1097/SCS.0000000000001572 et al9 studied incomplete uvular fusion in 2 general populations and 836 The Journal of Craniofacial Surgery Volume 26, Number 3, May 2015 Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. The Journal of Craniofacial Surgery Volume 26, Number 3, May 2015 Uvular Malformation and Plagiocephaly malformations, if present. We have frequently observed uvular malformations in patients with deformational plagiocephaly. These malformations include all degrees of incomplete uvular fusion from grooved or notched uvular tips to complete bifidity. Our study’s aim was to determine if there is a higher incidence of incomplete uvular fusion in patients with deformational plagiocephaly compared to the general population. To date, there have been no reports of uvular malformations in the presence of deformational plagiocephaly. PATIENTS AND METHODS Institutional review board approval was obtained. We performed a retrospective chart review of 225 consecutive patients seen at the FIGURE 1. Infant with posterior deformational plagiocephaly. Upstate Cleft and Craniofacial Center between January 1, 2006, and September 30, 2011, with plagiocephaly. Patients were identified classified the malformations based on the percent length of the using the International Classification of Diseases, Ninth Revision incomplete fusion. He noted a 1.34% and 1.47% incidence of all code for plagiocephaly. Exclusion criteria included patients with degrees of bifidity in these 2 populations. Of note, most of these craniosynostosis and those who did not meet diagnostic criteria for patients (88% and 82%, respectively) did not have a complete bifid deformational plagiocephaly. Posterior deformational plagioce- uvula but rather had less than 25% length of bifidity. Shprintzen phaly was diagnosed by the presence of unilateral parieto-occipital et al11 reported an incidence of uvular abnormalities in a busy flattening often with ipsilateral frontal bossing, asymmetric paral- suburban pediatric practice of 2% to 4%. Uvular abnormality was lelogram shape, and anterior displacement of the ipsilateral ear. described as either notched, bifid, or broad. However, the incidence Wide variation in severity classification in the charts precluded our of each uvular abnormality was not given. ability to consistently gather these data. The diagnostic criteria for SMCP was first defined by Calnan12 After exclusion, we identified 146 patients with deformational as a clinical triad of bifid uvula, zona pellucida, and a notch in the plagiocephaly. The mean age at presentation and diagnosis was posterior border of the hard palate (Fig. 2). The incidence of SMCP 8.2 months, with a range of 1 week to 83 months. Data were is between 0.02% and 0.08%.10,13 Kaplan14 introduced the term collected for sex, parity, maternal uterine abnormalities, fetal ‘‘occult SMCP’’ to describe patients who had velar abnormalities position, intrauterine growth restriction (IUGR), cephalohematoma, but did not exhibit any of the anatomical signs of the classic triad. oligohydramnios, assisted delivery, cesarean section, and singleton The diagnosis of an occult SMCP can only be made by nasophar- versus multiple-birth pregnancies. All patients had an oral exami- yngolaryngoscopy to document a lack of velar eminence on the nation. Physical features were collected including location of nasal surface of the soft palate, causing it to appear flat or concave. cranial flattening (right vs left; anterior vs posterior), uvular mal- This appearance suggests absence of the uvula muscle and diastasis formation, presence of torticollis, and other abnormalities. A uvula
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