International Journal of Open Access Otolaryngology

Objectives: Congenital hairy polyps are rare and benign, pedunculated masses, usually found in the pharynx of newborns and infants. Timing and types of presenting symptoms highly depend on the size and the localization of the mass.

Methods: A case of a healthy female newborn with intermittent, life threatening, and significant airway obstruction due to a large hairy polyp pedunculated on the posterior soft palate is presented. After fiberoptic evaluation, the airway was secured by intubation. Magnetic Resonance Imaging (MRI) confirmed the fatty and pedunculated nature of the mass and excluded any endocranial communication. Results: Surgical resection at the site of pedicle attachment under endoscopic guidance allowed safe extubation and definitive discharge without symptoms 7 days after birth. Follow up checkups showed no signs of recurrence.

Conclusions: Congenital hairy polyps are rare benign malformations with varied localization and size that determine the clinical presentation. Often airway obstruction is the presenting symptom and these cases require proper critical airway management. Imaging is mandatory to narrow down the differential diagnosis. The treatment is surgical excision and definitive diagnosis is histological.

Keywords: Neonatal airway obstruction; Developmental anomaly; Hairy polyp; Endoscopic resection; Dermoids

Neonatal airway obstruction by congenital pharyngeal mass can be catastrophic and itis imperative that an experienced team is involved in the management at the very earliest. An upper airway obstruction with respiratory distress in a newborn should warrant an endoscopic evaluation before any attempt of oro- or naso-tracheal intubation [1]. Indeed, the differential diagnosis is varied, some of which can lead to a challenging intubation. Naso-tracheal versus oro-tracheal intubation should be weighed depending on the type and the vascularity of the lesion and its anatomical site. Endoscopic evaluation of the upper airway before intubation is important, as an adequate diagnosis can be difficult once an endotracheal tube is in place. After extubation, it is difficult to discriminate between a congenital condition and tube-induced modification of the upper airway - especially in the larynx [2]. We report here the management of neonatal airway obstruction due to a large congenital hairy polyp (CHP) with its pedicle attached to the posterior soft palate.

A female newborn, delivered at 38 weeks 6/7 of an uncomplicated gestation via cesarean section, to a healthy 34-year-old G2P2 woman, was transferred to our quaternary center after an immediate onset of intermittent stridor and respiratory distress. Initial evaluation with an intubation laryngoscope by the anesthesiology team at the referring hospital showed an oropharyngeal, mobile mass completely obstructing the supraglottis. Jaw-lift maneuver and upright position allowed for efficient breathing during the short transfer time to our clinic. No intubation was attempted. Senior anesthesiologist trained in neonatal intubation accompanied the newborn during the transfer. Upon arrival in the neonatal intensive care unit, the newborn was breathing spontaneously at room air, with normal O2 saturation in the position used for transfer. This stable situation allowed for a thorough endoscopic evaluation which was performed using a 3.1 mm flexible video bronchoscope (Olympus BF-XP190, Hamburg, Germany) under local anesthesia (Novesin, Omni Vision AG, 8212 Neuhausen Switzerland). All required material and trained staff were present at the bedside in case an emergent intubation or tracheotomy was required. Bilateral nostrils were normal and free up to the nasopharynx. Further evaluation showed a mobile, pedunculated, whitish polypoid mass, attached to the left posterior palate pharyngeal arch, causing a ball-valve effect in the supraglottis with each respiratory cycle. Only a small 2 mm space remained intermittently open on the right side of the nasopharynx due to the mass mobility. The larynx was normal with physiologic vocal fold movements and the subglottic space, although not fully assessable in these settings, seemed normal. Subsequently a 3.5 Portex nasotracheal tube was slid over the fiberscope and positioned under vision to secure the airway via the contralateral nasal fossa. Subsequently, an MRI was done that showed a 21mm x12mm x7mm pedunculated, on-contrast enhancing mass having a discernible attachment to the left posterior palate pharyngeal arch with no intracranial extension (Figure 1).

Endoscopic resection was planned the following day. The nose was adequately decongested with Rinosedin 0.05% (Xylometazoline HCL). Firstly, The nasopharynx was re-evaluated using 0°, 30°& 120° 4mm rigid endoscopes(Storz, 78532 Tuttlingen, Germany) that were passed transorally along with an intubation laryngoscope. The exact site of pedicle attachment of the polyp was confirmed. The nasotracheal tube was then removed under direct laryngoscopy with a 4mm 0° rigid telescope (Storz, 78532 Tuttlingen, Germany). Trans nasal endoscopy confirmed a pedunculated mass, lateral to the left posterior aspect of the uvula, with a thin stalk (Figure 2).

The mass was whitish, of skin appearance with thin hair over its surface (Figure 3).

It was mobile with the respiratory cycles (Video 1). After confirming the details of the mass, the patient were re-intubated using a south facing RAE cuffed oro-tracheal tube. The mouth was kept open with a Negus retractor and the implantation stalk was sectioned by the first surgeon using a microlaryngoscopy scissors (Laryngo FIT HAVAS scissors, Storz, 78532 Tuttlingen, Germany) passed through the left nasal fossa under endoscopic guidance with a 2mm 30° endoscope (Storz, 78532 Tuttlingen, Germany) that was passed through the right nasal fossa (Video 2). The caudal part of the mass was grasped transorally and kept under tension by the assistant surgeon that aided the pedicle sectioning. The implantation area was cauterized with a fine bipolar cautery forceps passed transorally under endoscopic vision. The patient was extubated to non-invasive ventilation that was weaned off on the same day. Postoperative course remained uneventful with no respiratory symptoms, and enteral nutrition was initiated, allowing discharge from the hospital on postnatal day 7.Clinical control one month post-surgery showed an asymptomatic child with adequate growth and endoscopic control excluded any sign of recurrence. Histopathological analysis confirmed a peripheral layer of keratinized squamous epithelium with hair follicles and

Congenital hairy polyps (CHP) are benign, rare masses of ectodermal and mesodermal embryologic origin classified as dermoids according to Arnold’s classification [3]. Some authors have described them as teratomas, despite the absence of endodermal tissues required to fulfill the definition [3]. Size and location of occurrence dictate the symptomatology, age of onset and severity. Respiratory obstruction and dysphagia are the most frequent symptoms cited in the literature [4, 5]. These polyps tend to occur more often in females (10:1 female to male gender distribution) and predominantly on the left side of the naso-oropharynx [6]. CHP are usually not associated with other malformations although some non-syndromic associations, including second branchial arch anomalies and/or cleft palate have been described [7,8].

Depending on the localization, some hairy polyps can be highly symptomatic or/and easily visualized on physical examination resulting in early diagnosis. Esophageal localization will be symptomatic if the polyp is large and endoscopy is required for definitive diagnosis and treatment. Without any further imaging or histopathological examination, the differential

diagnosis includes chronically lodged foreign bodies and rhinoliths, teratomas and meningo-encephaloceles [3]. For the latter, and relating to further surgical management of the polyp, MRI imaging is highly recommended to exclude intracranial extension [9]. Definitive diagnosis is made by histopathological examination of the specimen. Several review articles notably, by Kelly et al., Kalcioglu et al. and Dutta et al., summarize and list the different case reports including year of description, localization, age, gender and clinical presentation [10]. Dutta et al. described the characteristics of hairy polyps up to 2013 [11]. The most recent literature review included 121cases of ENT hairy polyps from 2013 onwards and this has been summarized in table 1 and show interesting facts (Table 1). These congenital lesions are clearly predominant in females and mainly localized in the naso pharynx presenting with severe respiratory distress. The oral cavity hairy polyps are highly symptomatic and easily visualized; hence they are discovered the earliest. Nasopharyngeal or middle ear polyps may long remain silent with presenting symptoms suggesting other, more frequent diagnoses. Examination is not straight forward explaining why they tend to be discovered later in youth. Other factors, such as the geographical localization of the patient, may have an effect on access to health care delaying the diagnosis of these lesions; this may explain why patients with oropharyngeal and nasopharyngeal polyps presenting with respiratory distress have late presentation. It should also be noted that the acuteness, timing and degree of presenting symptoms were not considered in the aggregation of data as they are often not provided.

The origin of hairy polyps is an issue of debate, and many theories have been proposed to explain their embryological origin. The first landmark paper, published by Brown-Kelly in 1918, proposes the development of these lesions as a result of the escape of pluri potent tissue from normal control mechanisms [12]. Holt et al. in 1979 also coin the theory of pluri potent tissue escaping local control via a triggering event leading to the formation of hetero topic tissue presenting as a disorganized mass [13].

The treatment for nasopharyngeal CHP is surgical. In a neonate with CHP and presenting with an airway compromise, early securing of the airway is crucial. The ideal technique for intubation depends on the site of the pathology. Cannot ventilate – cannot intubate situation must be avoided in such patients with an already compromised airway. It is critical that the entire management of the patient must be done at a specialized center and by an experienced team. Early flexible endoscopy under local anesthesia or light general anesthesia maintaining spontaneous breathing prior to intubation allows visualization of the airway morphology, its compromise and the dynamics. The latest technology video bronchoscopes are ideal for such endoscopies. They are thin, have excellent optics with suction channels and well-suited in neonates and children with challenging morphology of the upper airway. Such an endoscopy helps in characterizing a nasopharyngeal mass and establishing a differential diagnosis. It then allows securing the airway without latrogenically traumatizing the anomaly prior to its definitive treatment. MRI should be performed to better characterize the lesion and rule out endocranial communication. Complete resection at the implantation site can then be safely performed. Completely trans-nasal approach in a neonate can be limited. Hence, in our case, we preferred the bi-nasal, with combined nasal and oral approaches to allow complete excision of the mass. We used the microlaryngeal scissor, which is thin, long and well adapted to narrow nasal passages. The angulated thin rigid sinus endoscope passed in the opposite nostril allowed adequate visualization of the nasopharyngeal mass. A second surgeon grasps the mass trans-orally helping to stretch and narrow down the pedicle and then section it. This way, the large mass was removed mono- bloc through the mouth, which would not have been possible trans-nasally. Postoperative recurrence is very rare and complications such as velopharyngeal insufficiency are sporadic [3-14, 15]. Postoperative surveillance is recommended to ensure proper breathing and feeding.

Congenital hairy polyps are rare benign malformations with varied localization and size that determine the clinical presentation. Often airway obstruction is the presenting symptom and these cases require proper critical airway management. Imaging is mandatory to narrow down the differential diagnosis. The treatment is surgical excision and definitive diagnosis is histological.

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The authors have no conflict of interest to disclose

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1. Monnier, Philippe. Pediatric airway surgery: management of laryngotracheal stenosis in infants and children. Springer Science & Business Media. 2011.
2. Bull, P.D. Evaluation of the pediatric airway by rigid endoscopy. In: Cotton, R.T., Myer III, C.H.M. (eds.) Practical Pediatric Otolaryngology, pp. 477–481. Lippincott-Raven, Philadelphia/New York. 1999
3. George LC, Jonathan AP, Scott CM. Nasopharyngeal teratomas and dermoids: a review of the literature and case series. International journal of pediatric otorhinolaryngology. 2000; 52(3): 219-227.
4. Agrawal, Namit, Deepak K, Gavin A M. Combined transoral and nasendoscopic resection of an eustachian tube hairy polyp causing neonatal respiratory distress. American journal of otolaryngology. 2009; 30(5): 343-346.
5. Richter A, Mysore K, Schady D, Chandy B. Congenital hairy polyp of the oropharynx presenting as an esophageal mass in a neonate, a case report and literature review. International journal of pediatric otorhinolaryngology. 2015, 80:26-29. doi: 10.1016/j.ijporl.2015.11.015
6. Mehmet Y, Metin I, Ozcan O, Emin K, Mehmet A. Congenital hairy polyp of the soft palate. International journal of pediatric otorhinolaryngology. 2012; 76(1): 5-8. doi: 10.1016/j.ijporl.2011.10.008
7. Burns BV, Axon PR, Pahade A. ‘Hairy polyp’of the pharynx in association with an ipsilateral branchial sinus: evidence that the ‘hairy polyp’is a second branchial arch malformation. The Journal of Laryngology & Otology. 2001; 115(2): 145-148.
8. Haddad J, Senders CW, Leach CS, Stool SE. Congenital hairy polyp of the nasopharynx associated with cleft palate: report of two cases. International journal of pediatric otorhinolaryngology. 1990; 20(2): 127-135. doi: 10.1016/0165-5876(90)90078-6
9. Christianson B, Ulualp S O, Koral K, Rakheja D, Deskin R. Congenital hairy polyp of the palatopharyngeus muscle. Case reports in otolaryngology. 2013; 2013:1-3. doi:10.1155/2013/374681
10. Kalcioglu M T, Can S, Aydin N E. Unusual case of soft palate hairy polyp causing airway obstruction and review of the literature. Journal of pediatric surgery. 2010; 45(12):e5-e8. doi: 10.1016/j.jpedsurg.2010.08.008
11. Dutta M, Soham R, Soumya G. Naso-oropharyngeal choristoma (hairy polyps): an overview and current update on presentation, management, origin and related controversies. European Archives of Oto-Rhino-Laryngology. 2015; 272(5):1047-1051.
12. Brown-Kelly A. Hairy or dermoid polyp of the pharynx and naso-pharynx. J Laryngol Otol. 1918; 33(3):65-70. doi: 10.1017/S1755146300018114
13. Holt G R. Dermoids and teratomas of the head and neck. Eur Nose Throat J. 1979; 58 (12): 520-531.
14. Olivares-Pakzad B A, Henry D T, Louis P D, Jan L K, Uldis. Oropharyngeal hairy polyp with meningothelial elements. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology. 1995; 79(4): 462-468. doi: 10.1016/S1079-2104(05)80129-5
15. Rickul V, Jacob P, Yazeed A, Yolene L. Hemorrhagic hairy polyp causing velopharyngeal dysfunction in a newborn. The Cleft Palate-Craniofacial Journal. 2015; 52 (5):625-628. doi: 10.1597/13-265