Congenital Nasal Dermoid and Sinus Cysts: A Case Report

Dr. Sheetal Dandale

MBBS, MD, DDV, FCPS Consultant Dermatologist, Trichologist and Laser Surgeon Skinconsult Clinic, Mumbai

Introduction

Congenital nasal dermoid and sinus cysts (NDSCs) are rare congenital malformations. They are the most common type of congenital nasal midline lesions.1

Other types include nasal meningoencephaloceles and gliomas. The incidence of dermoid cysts and fistulas in the midline of the nose is 1/20,000 to 1/40,000.1 NDSCs constitute approximately 11% of dermoid cysts in the head and neck, 1% of dermoid cysts in the whole body, and 61% of median lesions in children.

Herewith we describe a rare case of NDSC in an Indian adult female.

Case Report

An otherwise healthy 41-yearold female presented with an asymptomatic swelling over her nasal dorsum of 10 years duration. She reported a life-long history of a dorsal nasal pit, from which she also noted the growth of hair from within the pit.

The patient denied a history of nasal infection or abscess. She had never sought any type of medical attention for the pits.

On examination, the area around the middle nasal vault was skin coloured and swollen. A midline punctum and a sinus was present without any discharge. On probing, the sinus tract was ending on the nasal bone.

Based on this clinical presentation, the diagnosis of nasal dermoid sinus cyst was made.

In this case, we successfully performed the external rhinoplasty approach. This approach offers a good surgical exposure and an excellent cosmetic effect.2 1 (a)

Discussion

In 1817, Cruvelier first described a nasal dermoid cyst when he identified a hair-containing sinus tract of the nasal dorsum in a child.

A nasal dermoid typically presents as a midline mass, and may be located anywhere from the base of the collumella, along the nasal dorsum, to the nasoglabellar region.3 It is typically a noncompressible mass that does not transilluminate and has a negative Furstenberg sign (i.e. there is no enlargement with compression of the jugular veins).

A sinus opening with intermittent discharge of sebaceous material is frequently encountered. Local infection is common, but infection related to intracranial extension (e.g. meningitis and brain abscess) is rare. Hair protruding through a cutaneous punctum over the nasal dorsum is pathognomonic for a nasal dermoid.4 The mean age of presentation varies from 14 to 34 months,3,5 but reports of nasal dermoids presenting in adults exist in the literature.6,7,8

There are some reports about male predominance.9

Intracranial extension varied from 5 to 45% in several recently published series.3,4, 9, 10

Nasofrontal dermoid sinus cysts have a unique embryological origin. A midline basal frontal dermoid associated with a dimple or sinus opening on the nasal dorsum with or without protruding hair and sebaceous discharge is the classical presentation.

The first report about NDSC was published by Bramann in 1890.11

There are lots of theories like sequestration, trilaminary and prenasal, about NDSCs.

The most accepted theory is Pratt’s prenasal theory.12

Pratt described the common embryologic pathway of gliomas, encephaloceles and nasofrontal dermoid sinus tract.11,5

The nasal bones are separated from the adjacent cartilaginous nasal capsule by a Prenasal space (Fig. 1).

Faulty regression of the embryologic neuroectodermal tract pulls skin elements into the prenasal space, resulting in a dermoid cyst or sinus. The sinus tract may form anywhere along the course of the diverticulum, from the columella to the anterior cranial fossa. The lesion may be extranasal, intranasal, or a combination of the two. The majority of nasal dermoids are confined to the superficial nasal area. Intracranial extension most often communicates through the foramen cecum or the cribriform plate to the base of the frontal fossa with extradural adherence to the falxcerebri. There are rare reported cases of involvement of the brain parenchyma.3

Imaging is a crucial component in the evaluation of nasal dermoids since intracranial extension has been reported in up to 45% of cases.

Computed tomography (CT) and magnetic resonance imaging (MRI) scans are the gold standards in radiographic evaluation of nasal dermoids.

Complete surgical excision of nasal dermoid cyst and any associated sinus tract is essential for cure. Recurrence rates of 50–100% have been reported in cases in which dermal components were incompletely removed.4,13 Any surgical approach for removal of nasal dermoid cysts should adhere to the following four tenets: permit access to all midline lesions and allow for medial and lateral osteotomies; allow repair of skull base defects or cerebrospinal fluid leak; facilitate reconstruction of any nasal deformity; and result in a cosmetically acceptable scar.13

The midline vertical incision continues to be the most popular and straightforward technique for removal of the cyst.

Conclusion

Although nasal dermoids are rarely encountered, their potential for local bony atrophy and distortion of the nose, as well as serious infections such as meningitis and brain abscess, warrant early recognition, accurate diagnosis, and prompt treatment. A thorough understanding of the embryogenesis of midline nasal congenital lesions is important when the diagnosis is not obvious on physical examination alone. Manipulation of a midline nasal mass prior to excluding or confirming any central communication may result in cerebrospinal fluid leak or infection. Imaging of the midface and brain is essential for accurate diagnosis, assessment for any intracranial extension, and appropriate surgical planning. Complete excision of the cyst and its tract is the only cure. Any residual ectodermal elements result in a high rate of recurrence and complicated infections.

References

1. Hughes GB, Sharpino G, Hunt W, et al. Management of the congenital midline nasal mass—a review. Head Neck Surg 1980;2:222–33

2. Locke R, Kubba H. A case of a nasal dermoid presenting as a median upper lip sinus. Int J Oral MaxiofacialSurg 2011;40:985–7. [PubMed] [Google Scholar]

3. Rahbar R, Shah P, Mulliken JB, et al. The presentation and management of nasal dermoid: a 30-year experience.

4. Wardinsky TD, Pagon RA, Kropp RJ, et al. Nasal dermoid sinus cysts: association with intracranial extension and multiple malformations. Cleft palate Craniofac J 1991; 28:87–95

5. Penslar JM, Bauer BS, Naidich TP. Craniofacial dermoids. PlastRenconstrSurg 1988; 82:953–959

6. Hacker DC, Freeman JL. Intracranial extension of a nasal dermoid sinus cyst in a 56-year-old man. Head Neck 1994; 16:366–371.

7. Vaghela HM, Bradley PJ. Nasal dermoid sinus cyst in adults. J LaryngolOtol 2004; 118:955–962.

8. Post G, McMains KC, Kountakis SE. Adult nasal dermoid sinus cyst. Am J Otolaryngol 2005; 26:403–405

9. Denoyelle F, Ducroz V, Roger G, Gara-bedian EN. Nasal dermoid sinus cysts in children. Laryngoscope 1997;107:795-800

10. Van Aalst JA, Luerssen TG, Whitehead WE, Havlik RJ. ‘Keystone’ approach for intracranial nasofrontaldermoid sinuses. PlastReconstrSurg 2005; 116:13–19. Excellent discussion of the intracranial/ extracranial approach to dermoids pathology

11. Hanikeri M, Waterhouse N, Kirkpatrick N, et al. The management of midline transcranial nasal dermoid sinus cysts. Br J PlastSurg 2005;58:10431050.

12. Emel CT, Ömer TS, Güleser SAO, Hakan K. The management of rare nasal mass – nasal dermoid sinus cysts: open rhinoplasty. Rare Tumors. 2009 Dec 28;1(2):e40.

13. Posnick JC, Bortoluzzi P, Armstrong DC, Drake JM. Intracranial nasal dermoid sinus cysts: computed tomographic scan findings and surgical results. PlastReconstrSurg 1994; 93:745–754.

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