Pediatric Reports 2016; volume 8:6456

Rarely seen nasal congenital obstruction. The diagnosis and treatment of all cases were performed in Adnan Menderes Correspondence: Ye im Ba al, ENT-Head and problems causing neonatal University Medical Faculty Hospital (Turkey) Neck Department,ş ş Adnan Menderes upper respiratory obstruction: between years of 2012 and 2015. University, Kepez Mevkii, 09010 Aydın, Turkey. a case series Tel.: +90.256.2145400.2721 E-mail: [email protected] Ye im Ba al,1 Abdullah Bari Akcan,2 ş Case Report #1 Key words: Congenital; choanal atresia; chromo- Yaseminş Durum Polat,3 Cerenş Günel,1 some 18 deletion syndrome; nasolacrimal duct; Aylin Eryilmaz,1 Sema Ba ak1 nose. A 2-day-old baby girl was admitted with com- Departments of 1Ear, Nose,ş Throat-Head plaints of swelling of left eye, redness, and Contributions: the authors contributed equally. and Neck Surgery, 2Pediatrics, asphyxiation during feeding. In addition to 3 , Faculty of , Adnan swelling around her left eye punctum, in nasal Conflict of interest: the authors declare that there Menderes University, Aydın, Turkey examination, a cystic structure was identified, is no conflict of interest regarding the publication which fully obstructed the nasal passage in the of this paper. inferior meatus. The white blood cell count of the patient was 11.000. Following consultation Received for publication: 4 February 2016. Abstract of the patient with Revision received: 16 February 2016. Department, the patient was diagnosed with Accepted for publication: 18 February 2016. unilateral infected dacryocystocele. The Since newborns are obligatory nasal This work is licensed under a Creative Commons breathers, upper respiratory tract problems patient recovered clinically, following adminis- Attribution NonCommercial 4.0 License (CC BY- may sometimes be life threatening. The most tration of parenteral medical treatment (ampi- NC 4.0). common causing dyspnea and stri- cillin-sulbactam 40 mg/kg/day) and punctual dor in newborns is laryngomalacia. Nasal cavi- massage for ten days. The second year follow- ©Copyright Y. Ba al et al., 2016 ty that risk the neonatal airway are up examination revealed normal findings. Licensee PAGEPress,ş Italy Pediatric Reports 2016; 8:6456 more rarely met. These anomalies may be seen only doi:10.4081/pr.2016.6456 either as solitary anomalies or as a part of a syndrome. While choanal atresia is one of the best-known nasal cavity anomalies, choanal Case Report #2 stenosis, congenital nasal mid-line masses, use ized in the neonatal intensive care unit. Her congenital nasal pyriform aperture stenosis, A 10-day-old baby girl was admitted with physical examination revealed a cystic struc- and nasal tip anomalies are more rarely seen complaints of difficulty in nasal breathing and ture with hyperemic appearance, which fully structural pathologies. Choanal atresia may be swelling at the inner side the left eye. The obstructed the passage and was bulging from inner canthus of the right eye was normal in present either as an isolated congenital anom- inferior meatus to the left nasal passage. By inspection; however, there was a questionable aly or as a part of CHARGE syndrome. Some magnetic resonance imaging (MRI), a cystic hardness by palpation. Dense purulent dis- rare chromosome anomalies may also cause structure with smooth borders was detected in significant problems during nasal respiration charge was detected within both nasal pas- the neighborhood the left nasolacrimal duct. in newborns. With this study, we presented a sages together with severe edema of the nasal Antibiotic treatment was initiated by adminis- case series of newborns with pathologies that mucosa. The white blood cell count of the tration of ampicillin and cefotaxime. Surgical affected nasal respiration. Although the diag- patient was 14.800. By ultrasonography, ane- treatment was planned due to the upward and nosis and treatment of choanal atresia and choic cystic lesion of approximately 12×11 mm outward displacement of the eye. The was congenital dacryocystocele are well known, the commercialsize, with smooth borders at the location of left marsupialized and Ritleng tube was placed information on the diagnosis and treatment of epicanthus, and anechoic cystic lesion of under general anesthesia. Five and a half the other two uncommon cases are limited. approximately 8×11 mm size, at the location of With this study, we aimed to contribute to the right epicanthus were identified. The patient months later, the tube was removed and lavage literature by presenting our approach in six was hospitalized in the neonatal service and revealed that the punctum was patent. The cases having congenital pathologiesNon that cause treatment with IV ampicillin and cefotaxime second year follow-up examination revealed nasal respiratory obstruction. was initiated. Since swelling and erythema at normal findings. the inner side of the left eye decreased, drainage was not required. The patient was discharged following completion of the antibi- Introduction otic treatment in ten days. The sixth month fol- Case Report #4 low-up examination revealed normal findings. The most common pathology causing dysp- A 20-day-old infant was admitted with com- nea and stridor in newborns is laryngomalacia. plaints of swelling and deformity at the tip of Choanal atresia is one of the best-known nasal the nose, together with difficulty in breathing cavity anomalies, while choanal stenosis, con- Case Report #3 during feedings. Both nostrils were narrow genital nasal mid-line masses, congenital and asymmetrical (Figure 1B). A swelling with nasal pyriform aperture stenosis, and nasal tip A 20-day-old baby girl was admitted due to undefined borders was palpated at the nasal anomalies are more rarely seen.1 Choanal findings of upward and outward displacement tip. A homogeneously hyperechoic 9×8 mm atresia may be present alone or as a part of of the left eye and redness with 2×1 cm size at sized lesion with smooth borders, located at CHARGE syndrome.2 the inner canthus, and swelling sensitive to the skin and the subcutaneous tissue on the We present here six cases of congenital palpation (Figure 1A). The baby had a white right wing of nose, was identified by ultra- pathologies that cause nasal respiratory blood cell count of 15.000 and she was hospital- sonography. A 10×8 mm sized mass lesion with

[Pediatric Reports 2016; 8:6456] [page 9] Case Report smooth borders, which was hyperintense in was observed that nasal septum was thicker ple periorbital or and underly- T1A and T2A series, was suppressed in the fat- than normal and both nasal cavities were oblit- ing malignancy.12-14 Malignancy is rare in suppressed series, and not showing diffusion erated by soft tissue. Thereupon, short-term newborn.14 limitation, was identified on the right wing of topical decongestant and nasal steroid treat- Neonatal nostril and/or nasal tip anomalies the nose by magnetic resonance imaging. With ments were recommended, in addition to nasal are very rare. These anomalies usually coexist the indication of respiratory distress affecting lavage. The infant breathed from time to time with other nasal cavity anomalies such as the nutrition of infant, the mass was removed comfortably; however, sometimes intubation choanal atresia and congenital nasal pyriform by performing open rhinoplasty and using flap was required when the infant experienced aperture stenosis.15,16 In our fourth case, the technique. The pathological diagnosis was recurrence of the respiratory problems. Since preoperative radiological diagnosis of the reported as fibrolipomyomatous tissue. Infant the baby did not benefit from medical treat- mass, which led to extreme prominence of the sustained normal growth. At the second post- ment, it was decided to place tubes into the nasal tip together with narrowing and defor- operative year follow-up examination, no diffi- nasal passages. To place the tube, 1-2 mm car- culty was encountered during nasal respira- tilage was removed with a limited incision tion and follow-up was recommended. made on the existing septum deviation, and the deviated septum was pushed to the right side; by this way, the passage was completely opened, the choana became easily exposed, Case Report #5 and the aspiration catheter (#10 aspiration catheter) was placed (Figure 1C). The com- The baby boy, born in an external center by plaints of the infant, who was comfortable for optional cesarean section at 38 weeks of gesta- the first two days following this procedure, tion with 3000 gr weight, was transferred to started again, despite the aspiration probe was our hospital due to the diagnosis of an still in its place and open. 46XX,del(18)(11,2) omphalocele. The omphalocele defect was was determined by the peripheral blood kary- closed by the Department otype analysis (50 metaphase) which was under emergency conditions. During the post- made due to the dysmorphic facial appear- only operative period, the baby encountered severe ance. Patient was discharged by planning close respiratory distress. Bilateral choanal atresia follow-up in terms of the growth and neuromo- was detected by nasal endoscopic examina- tor development of the patient together with tion. The patient was radiologically diagnosed genetic counseling for the family. use as osseomembranous choanal atresia and was urgently operated. A Discussion and Conclusions

Case Report #6 The congenital dacryocystocele is a rare pathology, seen in only 0.1% of infants with The baby girl was born at 38th week of first congenital nasolacrimal obstruction.3 Its typi- gestation of 20-year-old mother, with birth cal appearance is a bluish, cystic, and hard weight of 3145 grams. Dysmorphic facial fea- mass, localized under the medial canthus.4 tures (rough-round faces, hypertelorism, large The clinical features of infection in this con- ears, micrognathia, flattened and broad nasal genitalcommercial cyst is described as . 5,6 bridge), breathing by nasal flaring, tachypnea, Meningoencephalocele, hemangioma and der- and intercostal-subcostal retractions were moid should be considered in differential B detected by physical examination. diagnosis.7 If the general status of infant is Due to increasing respiratory distress, the suitable, conservative treatment should be patient was intubated and connectedNon to tried first. Cyst marsupialization can be per- mechanical ventilator, following hospitaliza- formed either as a single procedure or togeth- tion to the intensive care unit. During follow- er with stent.8 Our first two cases had benefit- up after intubation, her tachypnea regressed ed from conservative approach. and the baby was extubated on the second day. In our third case, the surgery was directly Following extubation, the patient’s breathing performed, due to complete obstruction of was comfortable, but she was encountering nasal passage, severe displacement of the left cyanosis during feeding and significant sub- eye, and periorbital cellulitis; Ritleng tube was costal retractions and inspiratory stridor were inserted and was removed five and a half starting thereafter, respiratory acidosis was months later. Ritleng tube is recommended to developing, necessitating the patient to be be kept in place for 3-6 months.9,10 reintubated. The paranasal sinus computed Ultrasound is used in diagnosis. This non- C tomography (CT) revealed that both posterior invasive diagnostic method allows the identifi- choanal apertures were normal (measured cation and differentiation of different masses. Figure 1. A) Redness swelling around left 5.08 mm at the right side, 6.56 mm at the left Additionally, it shows the content of dacryocys- eye punctum; B) narrow and asymmetric side). The postero-inferior width of vomer was tocele, such as fluids and debris.11 Cross-sec- nostrils, swelling with undefined borders increased (measured 4.8 mm). The width of tional image resolution (CT or MRI) might be on the nose; C) appearance of the patient pyriform aperture was measured as 11 mm. It requested to exclude complications for exam- with catheter postoperatively.

[page 10] [Pediatric Reports 2016; 8:6456] Case Report mation of the nostril, was considered as der- diagnosis was confirmed and surgery was per- Congenital dacryocystocele: diagnosis moid cyst. Since it caused problems in nasal formed. No matter which additional patholo- using ante and post-natal ultrasonography. breathing especially while feeding, it was gies are present, the investigation for choanal Arq Bras Oftalmol 2014;77:261-3. decided to remove the mass. Open Rhinoplasty atresia should not be delayed in newborns. 12. LeBedis CA, Sakai O. Vascular and other and Flap Technique were chosen for the proce- emergencies in the head. Nontraumatic dure. The pathological diagnosis of the mass orbital conditions: diagnosis with CT and was reported as atypical fibrolipomyomatous MR imaging in the emergent setting. tissue. We encountered radiological atypical References fibrolipomyomatous tissue located in the nasal RadioGraphics 2008;28:1741-53. dorsum, neither in newborns nor in other age 1. John DJ, Hernandez JA, Swischuck LE. 13. Ludwig BJ, Foster BR, Saito N, et al. groups, within the scope of the medical litera- Airway imaging in children In: Bailey BJ, Diagnostic imaging in nontraumatic pedi- ture. Johnson JT, Newland SD, eds. Head and atric head and neck emergencies. 18p deletion is a chromosome anomaly that neck surgery otolaryngology. Philadelphia: RadioGraphics 2010;30:781-99. may occur via hereditary transition or sponta- Lippincott Williams and Wilkins; 2006. p 14. Capps EF, Kinsella JJ, Gupta M, et al. neously. Its phenotypic features are not com- 1072. Emergency imaging assessment of acute, pletely defined.17 It is characterized by find- 2. Kaplan LC. The CHARGE association: non traumatic conditions of the head and ings of craniofacial dysmorphism such as men- choanal atresia and multiple congenital neck. RadioGraphics 2010;30:1335-52. tal retardation, growth retardation, dystonia, anomalies. Otolaryngol Clin North Am 15. Abdollahifakhim S, Mousaviagdas M. round face, prominent anti-helix of the ear, 1989;22:661-72. Association of nasal nostril stenosis with hypertelorism, tip-tilted nose and broad nasal 3. MacEven CJ, Young JD. during bilateral choanal atresia: a case report. bridge, micrognathia, high-arched palate, fish the first year of life. Eye 1991;5:596-600. mouth, tooth abnormalities and anomalies of 4. Wong RK, VanderVeen DK. Presentation Iran J Otorhinolaryngol 2014;26:43-6. extremities, genitalia, brain, eye, and and management of congenital dacryocys- 16. Gungor AA, Reiersen DA. Balloon dilata- heart.18,19 There are more than 100 cases in tocele. 2008;122:1108-12. tion for congenital nasal piriform aperture the literature.19 However, inspiratory stridor 5. Ali MJ. Pediatric acute dacryocystitis. stenosis (CNPAS): a novel conservative problem, which was life threating in our case, Ophthal Plast Reconstr Surg 2015;31:341-7 onlytechnique. Am J Otolaryngol 2014;35:439- was not reported in any of these cases. In this 6. Barham HP, Wudel JM, Enzenauer RW, et 42. respect, our case was the first case with life- al. Congenital nasolacrimal duct 17. Peng D, Long PP, Wen B, et al. A study of a threatening upper respiratory tract obstruction cyst/dacryocystocele: an argumentuse for a rare chromosomal disorder: mosaic during the neonatal period in the literature. genetic basis. Rhinol 2012;3:46-9. 46,XX,del(18)(p11.2)/46,XX,i(18q). J We were not able to determine by which mech- 7. Becker BB. The treatment of congenital Genet 2013;92:611-5. anism this chromosome anomaly causes life- dacryocystocele. Am J Ophthalmol 18. De Grouchy J, Lamy M, Thieffry S, et al. threatening respiratory problem. We suggest 2006;142: 835-8. that dystonia might be one of the probable 8. Dogan E, Yüksel NG, Ecevit MC, et al. Dysmorphie complexe avec oligophrenie: mechanisms. Microdebrider assisted endoskopic marsu- Deletion des bras courts d’un chromosome Choanal atresia was first described by pializasyon of congenital intranasal naso- 17-18. C R Acad Sci 1963;256:1028. Roeder in 1755.20 Since it can be symptomatic lacrimal duct cysts. Int J Pediatr 19. Wester U, Bondeson ML, Edeby C, et al. very early and can also lead to severe respira- Otorhinolaryngol 2012;76:488-91. Clinical and molecular characterization of tory distress when it is bilateral, making the 9. Pelit A, Caylakli F, Yaycioglu RA, et al. individuals with 18p deletion: a genotype- diagnosis just following delivery has utmost Silicone intubation with the Ritleng phenotype correlation. Am J Med Genet importance.21 Various congenital anomalies commercialmethod using intranasal to 2006;140:1164-71. may accompany in approximately 40-50% of treat congenital nasolacrimal duct 20 Coniglio JU, Manzione JV, Hengerer AS. patients with choanal atresia; however, ours obstruction. Int J Pediatr Otorhinolaryngol Anatomic findings and management of was the first case that choanal atresia and 2009;73:1536-8. choanal atresia and the CHARGE associa- omphalocele association was defined. 10 Yalaz M, Ozcan AA, Akcali C, et al. Lacrimal Focusing primarily on the treatmentNon of intubation with the Ritleng system in tion. Ann Otol Rhinol Laryngol omphalocele had delayed the diagnosis of recurrent congenital nasolacrimal duct 1998;97:448-53. choanal atresia, despite the presence of respi- obstruction in children. ORL J 21. Brown OE, Smith T. The evaluation of ratory distress. However, by clinical suspicion, Otorhinolaryngol Relat Spec 2004;66:35-7. choanal atresia by CT. Int J Pediatr endoscopic and radiological investigations, 11. Machado MAC, Junior LA, Silva JAF, et al. Otorhinolarygol 1986;12:85-98.

[Pediatric Reports 2016; 8:6456] [page 11]