Paranasal Sinus Development and Choanal Atresia

ORIGINAL ARTICLE Paranasal Sinus Development and Choanal Atresia

Philomena Mufalli Behar, MD; N. Wendell Todd, MD

Background: Although the determinates of paranasal Main Outcome Measures: Determination of maxil- sinus development and sinusitis are not well defined, a lary sinus volumes and mucoperiosteal thickening on pre- candidate factor is blockage of the choana. operative computed tomograms.

Hypothesis: Maxillary sinuses ipsilateral to unilateral Results: Maxillary sinuses ipsilateral to unilateral choa- choanal atresia are comparatively small and have more nal atresia have slightly larger volumes than, and muco- evidence of sinusitis than do the contralateral sinuses. periosteal thickening that is similar to, the contralateral sinuses. Design: Retrospective. Conclusion: These data suggest that maxillary sinus de- Setting: Children’s hospitals. velopment and sinusitis are independent of posterior nasal ventilation and drainage. Patients: Sixteen nonsyndomic children with isolated unilateral congenital choanal atresia. Arch Otolaryngol Head Neck Surg. 2000;126:155-157

BSTRUCTION of sinona- lary sinuses is adversely affected by severe sal drainage and ventila- sinus infection. This compromised growth tion is a well-accepted is exhibited by the small paranasal sinuses contributor to paranasal of patients with cystic fibrosis.17 Interest- sinusitis.1-11 Such a situ- ingly, Kim et al17 reported that children who ation exists in children with choanal atre- meet the criteria for chronic sinusitis, but O 12 sia. In 1927, Grove reported a case of uni- who do not have cystic fibrosis, have max- lateral choanal atresia, about the 180th at illary sinus development that is compa- the time, and noted that: rable to that of normal subjects. In children with unilateral choanal the free ventilation of the nose is one of the most atresia, ipsilateral anterior nasal dis- potent factors in the prevention of sinus disease charge is a presenting symptom.18 These and in the cure of an early existing sinus infec- children are frequently treated for months tion. As no air current is possible [through the for presumptive sinusitis before the choa- nose with unilateral choanal atresia], it must be- come apparent that the sinuses on the side of a nal atresia is recognized. Because nasal choanal atresia are particularly susceptible to in- breathing is absent on the side of the atre- fection. sia, poor gas exchange, leading to a lower partial pressure of oxygen and improved Proetz6 emphasized, in 1941, the im- environment for bacterial growth, would portance of sinus ventilation and drainage, be expected.2,5 The combination of abnor- noting that “it is generally agreed that stag- mal mucociliary flow, pooling of secre- nation of mucus in the closed side is apt to tions, and poor nasal ventilation would result ultimately in sinus disease.” Later, Fri- seem to predispose these children, in day et al13 and then Wald10 put choanal atre- particular, to sinusitis on the atretic side. sia at the top of the list of mechanical ob- With this background understand- structions predisposing to sinusitis. ing, we hypothesize that children with uni- Because ventilation of the middle ear lateral choanal atresia have abnormal mu- contributes to temporal bone pneumatiza- cociliary flow, relatively poor ventilation, tion, nasal ventilation may be important in and smaller sinuses and sinusitis on the the development of the paranasal si- atretic side. However, we were surprised 6,14-16 From the Department of nuses. Extensive paranasal sinus and and intrigued to encounter a child with Otolaryngology, Emory mastoid development indicate sinus and unilateral choanal atresia and well- University School of Medicine, middle ear health during a person’s growth developed, aerated maxillary sinuses. Was Atlanta, Ga. period.6 Conversely, growth of the maxil- this one case a fluke? Unilateral choanal

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©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 SUBJECTS AND METHODS DETERMINATIONS OF MAXILLARY SINUS VOLUMES STUDY POPULATION The volume of each maxillary sinus was calculated using a The medical records of all children with unilateral choanal technique described by Cavalieri, who was a contemporary atresia were requested from the 3 children’s hospitals in At- and disciple of Galileo. Each maxillary sinus volume was ap- lanta, Ga, and from a private otolaryngology practice in Mem- proximated by calculating the area of each individually traced phis, Tenn. Approval of the Clinical Research Coordinating CT section using a planimeter (Keuffel & Esser Co, Ger- Committee was obtained from the one institution that required many). Correction for magnification (ie, correction for lin- such approval. Excluded from the study were patients with ear compression of each CT image) was accomplished by mea- bilateral atresia or anterior nasal piriform aperture stenosis suring the actual number of centimeters represented by the and patients with any other congenital anomaly or syndrome. centimeter reference on the CT scan. Thus, the volume of Also excluded were children whose initial preoperative com- each maxillary sinus was calculated according to the follow- puted tomographic (CT) scan was unavailable or did not in- ing formula: (linear correction)2 ϫ (centimeters between sec- clude the posterior choanae and maxillary sinuses. tions) ϫ (sum of the object’s areas). The initial preoperative noncontrast axial sinus CT Similarly, the volume of each maxillary sinus contain- scans of 15 children and the coronal CT scan of 1 child were ing soft tissue density (rather than air) was calculated. The available for study. Each sinus was tomographically sec- aerated volume of the sinus was determined by subtract- tioned at regular parallel intervals ranging from 1 to 5 mm. ing the soft tissue–containing volume from the bony out- The identity of each patient and the side of the atresia were line of the maxillary sinus. known and concealed by the first author (P.M.B.) using opaque adhesive tape so that the reviewing author (N.W.T.) STATISTICS could view the scans without bias. Using an x-ray film re- view box for illumination, the bony contours of all maxil- The paired t test was used to compare the mean of the vol- lary sinus sections (caudal to cranial extent) were traced umes of the maxillary sinuses ipsilateral to the choanal atre- onto paper by the reviewing author. Similarly, the area of sia with the mean of the volumes of the maxillary sinuses mucosal thickening (soft tissue density) within each max- contralateral to the atresia. The 1-tailed P value was used illary sinus was traced to compare aerated with nonaer- to reject the null hypothesis (that the mean of the differ- ated maxillary sinus volumes. ences of the pairs is different from 0).

atresia may be considered an experiment of nature, pro- tors include upper respiratory tract infections, both aller- viding an opportunity to explore the relationship of na- gic and nonallergic rhinitis, immunodeficiency states, sal obstruction and sinusitis in children. primary ciliary dyskinesia, cystic fibrosis, Down syndrome, inhalant pollutants, and aspirin sensitivity. The RESULTS list of local factors includes dental infections, cleft palate, and swimming, but mostly items related to nasal ob- Sixteen children, aged 1 month to 17 years, with iso- struction: adenoid hypertrophy or infection, foreign bod- lated unilateral choanal atresia were identified. Twelve ies, tumors, polyps, septal deviation, and choanal atre- children were female and 4 were male, similar to the 2:1 sia.10,13,20 All of these conditions presumably interfere ratio reported by Brown.19 Nine patients had right-sided with normal nasal ventilation and sinonasal mucociliary atresia and 7 had left-sided atresia. flow and lead to obstruction of the osteomeatal unit. A wide range of maxillary sinus volumes was noted Lifelong, persistent, severe paranasal sinusitis is (Figure). The mean maxillary sinus volume ipsilateral thought to be represented radiographically by nonaer- to the choanal atresia was 4.84 mL, compared with 4.48 ated sinuses that are smaller than usual. The nonaerated mL for the contralateral side. The sinuses ipsilateral to (ie, opaque) sinus is attributed to stagnated mucus or pus the atresia were, on average, larger than the contralat- or to thickened mucoperiosteum. Proetz6 suggested that eral sinuses (PϽ.06). Symmetry of maxillary sinus size “the failure of some sinuses to develop may be on a kin- was apparent (r = 0.97; PϽ.001). dred basis [to Wittmaack’s contention that inflammatory The volumes of the soft tissue (presumably muco- middle ear disease limits mastoid pneumatization].” How- periosteal thickening) in the maxillary sinuses ap- ever, Ritter21 stated, “For unknown reasons, during growth peared symmetrical: 6 patients had more soft tissue in the maxillary sinuses may cease development and pro- the sinus ipsilateral to the atresia; 5 patients had more duce a sinus lumen of less than normal size.” soft tissue in the sinus contralateral to the atresia; and 5 Notwithstanding the consensus of conventional wis- patients had complete opacification of both maxillary si- dom, sinusitis is not universal in children with choanal nuses; ie, sinusitis as assessed by the preoperative CT scans atresia. Proetz6 observed that newborns with bilateral was unrelated to the unilateral choanal atresia. choanal atresia do not have clinical infection of the maxillary sinuses. Stewart’s22 1931 report included the ra- COMMENT diographs of 3 patients with unilateral choanal atresia: “The air sinuses on both sides of the skull were found to Classically, chronic sinusitis in children is associated with be well developed.” Mehta,4 in describing an adult with both local and systemic contributing factors. Systemic fac- unilateral choanal atresia, reported near-symmetrical, aer-

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©2000 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 16 laterally is surprising and incongruous with the time- honored teachings that nasal ventilation and drainage are

8 necessary for sinus health. The explanation for this un- 14 expected finding is unknown, and may be a fertile in- vestigative tract for improved understanding of paranasal sinusitis.

12 Accepted for publication July 16, 1999. 7 Presented as a poster at the 14th Annual Meeting of 10 the American Society of Pediatric Otolaryngology and the Third Biannual Meeting of the Interamerican Association 7 of Pediatric Otolaryngology, Palm Desert, Calif, April 28-

8 30, 1999. Reprints: N. Wendell Todd, MD, Department of Oto- Atretic, mL laryngology, Emory University School of Medicine, 1365 Clifton Rd NE, Atlanta, GA 30322. 6 17 5 5 3 13 REFERENCES 4 6 10 <1 1. Dudley JP. Paranasal sinus infections. In: Ballenger JJ, Snow JB Jr, eds. Oto- 3 rhinolaryngology Head and Neck Surgery. 15th ed. Baltimore, Md: Williams & Wilkins; 1996:163-172. 2 2. Johnson JT. Infections. In: Cummings CW, ed. Otolaryngology Head and Neck 1 Surgery. Vol 1. 2nd ed. St Louis, Mo: Mosby–Year Book Inc; 1986:929-940. Girls 3. Gwaltney JM Jr, Jones JG, Kennedy DW. Medical management of sinusitis: edu- <1 Boys cational goals and management guidelines: the International Conference on Si- <1 <1 nus Disease. Ann Otol Laryngol Otol Suppl. 1995;167:22-30. 0 212144 6 8 10 4. Mehta D. Atlas of Endoscopic Sinonasal Surgery. Philadelphia, Pa: Lea & Fe- Nonatretic, mL biger; 1993. 5. Parsons DS, Wald ER. Otitis media and sinusitis: similar diseases. 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Nasal Sinuses: An Anatomic and Clinical Consideration. 2nd ed. stated in the study’s figure legend, “The sinuses are nor- Baltimore, Md: Williams & Wilkins; 1951. mally developed.” Klossek et al24 and Diner et al25 re- 10. Wald ER. Rhinitis and acute and chronic sinusitis. In: Pediatric Otolaryngology. Vol 1. Philadelphia, Pa: WB Saunders Co; 1996:843-858. cently described 2 small series of patients (numbering 6 11. Wigand ME. Endoscopic Surgery of the Paranasal Sinuses and Anterior Skull Base. and 11, respectively) with unilateral choanal atresia: no Suttgart, Germany: Georg Thieme Verlag; 1990. 12. Grove WE. Choanal atresia and sinus infection. Arch Otolaryngol. 1927;6:237- sinusitis! 241. 6 As Proetz suggested: 13. Friday GA Jr, Fireman P, Sukanich A, Steinberg ML. Sinusitis. In: Naspitz CK, Tinkelman DG, eds. Childhood Rhinitis and Sinusitis: Pathophysiology and Treat- A difference apparently exists between the behavior of a nasal ment. 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Am J Rhinol. 1997;11:275-281. lateral choanal atresia, including (1) stagnation of sino- 18. Rothman G, Wood RA, Naclerio RM. Unilateral choanal atresia masquerading nasal mucus per se does not limit development of the as chronic sinusitis. Pediatrics. 1994;94:941-944. maxillary sinuses; (2) absence of nasal ventilation alone 19. Brown OE. Choanal atresia. In: Schaefer SD, ed. Rhinology and Sinus Disease: A Problem-Oriented Approach. St Louis, Mo: Mosby–Year Book Inc; 1998:78-83. does not result in chronic sinusitis or limit paranasal si- 20. Gungor A, Corey JP. Pediatric sinusitis. In: McCaffrey TV, ed. Rhinologic Diag- nus pneumatization; (3) the blocked choana decreases nosis and Treatment. New York, NY: Thieme-Stratton Inc; 1997:382-405. the risk of viral and other pathogens getting into the nose; 21. Ritter FN. The Paranasal Sinuses: Anatomy and Surgical Technique. St Louis, Mo: Mosby–Year Book Inc; 1978. and (4) our findings, and those of other investigators, are 22. Stewart JP. Congenital atresia of the posterior nares. Arch Otolaryngol. 1931; flukes (statistically, this seems unlikely). 13:570-583. 23. Mittermaier R. Otorhinolaryngologic Radiology: A Radiologic Atlas of Ear, Nose and Throat Diseases. New York, NY: Grune & Stratton Inc; 1970. CONCLUSIONS 24. Klossek JM, Ferrie JC, Fourcroy PJ, Desmons C, Basso-Brusa F, Fontanel JP. Unilateral choanal atresia and paranasal sinus growth [in French]. Ann Otolaryn- gol Chir Cervicofac. 1996;113:392-396. That children with congenital unilateral choanal atresia 25. Diner PA, Andrieu-Guitrancourt J, Dehesdin D. Unilateral congenital choanal atre- manifest healthy, well-developed paranasal sinuses bi- sia and maxillary sinus development. J Maxillofac Surg. 1986;14:285-288.

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