Volume 1, Issue 4 , Pages 306-309, December 2006
Nasopharyngeal hairy polyp in association with West syndrome: Case report and literature review
Article Outline
Summary
Hairy polyps are rare benign congenital tumors of the oro-nasopharynx. Depending on the size and location of the tumor, the neonate usually presents with upper aerodigestive tract obstruction and/or failure to thrive. Despite adequate discussion in the literature, no definitive embryogenesis of the hairy polyp is known. We report a case of what we believe is the first concomitant occurrence of nasopharyngeal hairy polyp, West syndrome and laryngomalacia. The clinical presentation, investigation, management, histopathological features, and literature review of this lesion will be described and discussed.
Keywords: Hairy polyp, Dermoid, Pharyngeal tumors, Teratoma
1. Introduction
A hairy polyp or dermoid is a rare developmental malformation that is usually found in the oro-nasohparynx before the patient's first birthday. The lesion arise during early embryogenesis; derived from only two germinal layers, ectoderm and mesoderm and invariably benign. Depending on the size and location of the tumor, it typically presents at, or shortly after, birth with airway obstruction and failure to thrive. Occasionally, if asymptomatic, they may remain undetected until later in life.
Hairy polyps present as a single benign lesion with very limited growth potential and the consequent surgical excision is always curative although sporadic cases of recurrence have been reported. In this paper we report what we believe is the first concomitant occurrence of a nasopharyngeal hairy polyp, infantile spasm (IS) and laryngomalacia. Infantile spasm is a specific type of seizure seen in an epilepsy syndrome of infancy and early childhood known as West syndrome. The onset is predominantly in the first year of life, typically between 3 and 6 months. West syndrome is characterized by infantile spasms, hypsarrhythmia (abnormal, chaotic brain wave patterns), and mental retardation. Other neurological disorders, such as cerebral palsy, may be seen in 30–50% of those with IS.
2. Case report
A 3-month old female was referred to our department complaining of recurrent left ear discharge, noisy breathing induced by feeding and sleeping and failure to thrive since birth. The mother had a full term pregnancy and the infant was delivered through a lower cesarean section due to fetal distress during labour. Since birth and till presentation to our department, the patient was diagnosed with: infantile spasm, ischemic hypoxic encephalopathy, cerebral palsy, gastro-oesphageal reflux, and recurrent left ear discharge. The infant was on phenobarbitone 10
mg once daily and vigabartin 300mg twice daily.
On examination, the infant had nosiy breathing, microcephaly, temperature of 37.8C, pulse of 140min−1, respiratory rate of 48min−1, and oxygen saturation of 85% on air. Examination of the left ear showed an external auditory canal full of pus. Examination of the nose showed excessive nasal secretions. A fibro-optic nasoendoscopy was performed at a pediatric intensive care unit under sedation with orpharyngeal airway in place. The examination showed a pearl shaped gray mass almost completely obstructing the nasopharynx with movement of the mass during inspiration and expiration (Fig. 1). The computed tomography described a mass arising from the left side of the nasopharynx and protruding into the nasopharyngeal air column with no intracranial extension (Fig. 2).
Fig. 1.
An endoscopic view of the left side of the nose showing a nasopharyngeal mass.
Fig. 2.
Axial section of CT scans showing the nasopharyngeal mass.
The infant then underwent examination under anesthesia and excision of the lesion. We noted a pedunculated mass arising from the left nasopharyngeal wall almost completely obstructing the nasopharynx and the left Eustachian tube orifice. The mass was excised (1.5cm in diameter) from the lateral wall at pedicel site and delivered through the oropharynx.
Histopathological examination showed the lesion to be a hairy polyp of the pharynx. It consisted of squamous epithelium, hair follicles, and fibrofatty tissue with blood vessels (Fig. 3). There was no evidence of malignancy.
Fig. 3.
Histopathological features of hairy polyp. Note the presence of squamous epithelium (arrow) with underlying loose connective tissue containing a hair follicle (H&E satin x200).
Post-operatively the infant made a good recovery with marked improvement of her ear symptoms and partial improvement in her breathing. The infant therefore, underwent bronchoscopy which showed mild laryngomalacia (indrawing of floppy arytenoids and aryepiglottic folds). The patient made good improvements in her ENT symptoms and still under a regular follow up from our department.
3. Discussion
Hairy polyp is a rare developmental malformation that usually affects the nasopharynx. Despite its rarity, it is the most common congenital nasopharyngeal mass that can be encountered [1], [2]. The lateral wall of the nasopharynx and superior surface of the soft palate house an estimated 60% of these tumors [3], [4], [5]. The oropharynx is the site of origin for the majority of the rest [3], [4], [5]. The middle ear and mastoid cavity have also been sites of origin [3]. For unknown reasons hairy polyps are six times more common in females than in males [6]. Malignant transformation has never been described.
The total number of documented cases in the literature is approximately 135 [3]. The number of reported cases has risen sharply in the last 10 years, most likely secondary to better investigation methods which include nasoendoscopy, computed tomography, and magnetic resonance imaging rather than an actual increase in incidence [1].
Clinical presentations depend on the size and location of the polyp. Variable sizes have been described ranging from 0.5 to 6cm in diameter [5]. The patients usually presents at or shortly after birth with more than 50% of the cases reported below the age of 12 months [3]. Hairy polyp can however, present at any age with a documented case at the age of 71 years [7], [8]. The most common clinical presentations are upper airway obstruction, feeding difficulties, and failure to thrive. Other presentations include nasal discharge and obstruction, epistaxis, Eustachian tube dysfunction with ear discharge, asphyxia, vomiting, haemoptysis, and a detectable mass [1], [4], [5]. Hairy polyp usually presents as an isolated defect. Associated anomalies have, however, been described and include soft and hard palate clefts (10% of cases), agenesis of the uvula and external auricle, ankyloglossia, facial hemihypertrophy, left carotid artery atresia, and osteoporosis [1], [4], [5], [9]. Hairy polyps are not associated with a specific congenital syndrome, nor are there any genetic predispositions or relationships. To the best of our knowledge, this is the first case to be reported of hairy polyp with an association of infantile spasm and laryngomalacia.
The first goal of management is to secure an airway. This could be achieved by repositioning of the patient, endotracheal intubation and in extreme cases a tracheotomy. It should be stressed that a tracheotomy is rarely needed in these cases. In our case repostioning the patient was enough, however, an oropharyngeal tube was needed during fibro-optic nasoendoscopy. Once investigation was done, the surgical excision remains to be the ideal modality of treatment as these tumors rarely recur after excision. There have been, however, reports in the literature of recurrence [5], [7].
Histopathologically, the hairy polyp has been described as a club or pear shaped lesion that may be sessile but is more often pedunculated. The proximal pedicle is usually pink and sharply demarcated from the gray or white distal bulk of the polyp.
Microscopically, hairy polyp is composed of skin and adenexal structures overlying benign adipose tissue. Cartilage, muscle, nerves, lymph nodes, minor salivary glands, and bone are occasionally found.
The first description of hairy polyps was by Arnold in the year 1870. He described four categories of masses arising from the nasopharynx: teratomas, teratoids, epignathi, and ‘dermoids’ (or hairy polyp). Some defined hairy polyp as a hamartoma, while others believe it is a choristoma [10]. Hamartomas are composed of tissue that is indigenous to the site and hairy skin in the nasopharynx excludes this diagnosis; choristomas are similar but are composed of tissue that is foreign to the site. The hairy polyp is not a dermoid as no ectodermal inclusion cysts are sequestered within the mesoderm in the hairy polyp. Teratomatous lesion (teratomas and teratiods) consists of three germ layers (ectoderm, mesoderm, and endoderm), have equal sex incidence, and a potential for malignant transformation. All these features do not occur in hairy polyps.
Despite much discussion in the literature, no definitive embryogenesis of the hairy polyp is known. The ‘Accessory auricle theory’, proposed that hairy polyps were displacements of first or second arch tissue with ‘auricular hillock’ potential, and that these lesions represented ‘pharyngeal accessory auricles’ [11]. However, not all hairy polyps have cartilage, and not all are within the distribution of first or second arches. Other theories proposed failure of the nasopharyngeal membrane to regress during the seventh week of gestation; and disturbed development during the fusion of the epiblast of the stomodeum with the anterior foregut [12]. The above two theories would not account for all sites of hairy polyps, such as the middle ear, Eustachian tube, and oropharynx. Furthermore, there is no association between choanal atresia and hairy polyp as one would expect if the theory was correct.
4. Conclusion
Hairy polyps are the most common congenital tumor of the nasopharynx. All infants suspected of upper airway obstruction should therefore be investigated for a pharyngeal tumor and/or choanal atresia. Fibro-optic nasoendoscopy, computed tomography, and/or magnetic resonance imaging should be performed in all patients prior to surgical excision. Significant morbidity can be avoided if early diagnosis and treatment are undertaken.
Hairy polyp should be considered as a distinct entity. The use of the word dermoid is misleading and should be dropped for the more accurate description of a hairy polyp.
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PII: S1871-4048(06)00089-X
doi:10.1016/j.pedex.2006.09.004
© 2006 Elsevier Ireland Ltd. All rights reserved.
Volume 1, Issue 4 , Pages 306-309, December 2006
