|Year : 2015 | Volume
| Issue : 1 | Page : 70-73
Craniofacial anomalies with toothy nose
Rakesh Kain, Satyavrat Arya
Department of Burns Plastic and Maxillofacial Surgery, Safdarjung Hospital, New Delhi, India
|Date of Web Publication||4-Feb-2015|
Dr. Satyavrat Arya
Department of Burns Plastic and Maxillofacial Surgery, Safdarjung Hospital, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
A simple subcutaneous lesion such as an epidermoid cyst can present as an aesthetic challenge when located in the nasal tip. Usually the nasal dermoid presents as swelling over the dorsum of nose extending from the nasal tip to the nasal bridge with or without intracranial extensions. In some cases, the overlying skin gets rupture and underlying abnormal structures developed from pluripotent cells of dermoid become visible with variable discharges such as cerebrospinal fluid or sequestrum. Origin of nasal dermoid sinus cyst (NDSC) during embryological development requires two necessary and sufficient conditions. One is competence of the ectodermal and meso-ectodermal cells to form dermoid tissue with epithelial-mesenchymal interactions and second, presence of a topographical site of ectodermal inclusion, which fits well with the various clinical presentations of NDSCs. The case presented here has naso-ethmoidal dermoid cyst with ectopic teeth at the tip of the nose. The patient had a cranial defect with bulging of tissue in naso-ethmoidal region that was in line of 1-13 facial cleft of Tessier. Computerized tomography revealed a bony gap in the region of right fronto-ethmoidal region and brain cyst with craniosynostosis in left frontoparietal suture. Transcranial approach was used to manage the case and all the teeth at the tip of the nose were removed.
Keywords: Craniofacial anomalies, facial cleft, nasal dermoid sinus, teeth in nose, tessier cleft
|How to cite this article:|
Kain R, Arya S. Craniofacial anomalies with toothy nose. J Cleft Lip Palate Craniofac Anomal 2015;2:70-3
| Introduction|| |
Nasal dermoid sinus cyst (NDSC) in children is a rare malformation (1/20,000-1/40,000). Apart from visible local swelling, they present as median nasal lump or pit on the dorsum, and their diagnosis and treatment are often delayed due to ignorance. Consequences of untreated nasal dermoid sinuses and cysts are local infection, rupture, meningitis, and empyema due to their frequent intracranial extension.  Such developmental anomaly arises during the embryological migration of various processes in the craniofacial region. The abnormal entrapment of tissues and their failure to separate leads to various craniofacial abnormalities.
The presence of teeth at nasal tip is a rare abnormality. The present case had four well-developed teeth that were removed from the tip of the nose and histological examination confirmed presence of elements of teeth. These abnormal teeth might have developed from pluripotent cells that transformed into ectodermal and mesodermal tissues leading to dentin and enamel formation. The proper migration of neural crest cells is essential for the development of face and teeth. The tooth germ is derived from ectoderm of the first arch, and its supporting apparatus are derived directly from neural crest cells, and their depletion prevents proper dental development. Normally, the tooth germ subsequently develops into enamel organ, dental papilla and dental follicle which finally develop into enamel, dentine and pulp.
| Case Report|| |
An 8-year-old female child presented to our outpatient department with swelling in fronto-nasal region. The patient was the second child of non-consanguineous marriage with non-relevant history. Her nose was developed but deformed as broad, deviated towards the right side and teeth like structures were visible through skin disruptions at the tip of the nose. The right orbit was displaced with reduction in orbital volume. There was protuberance in left fronto-glabellar region with over growth of nose and shift of nose toward right side with involvement of medial orbital wall [Figure 1]. Also, there was a skeletal defect on right fronto-ethmoidal region. No obvious abnormality was found in neurological and biochemical examination. Intra-orally patient had all normally developed and well aligned teeth in both jaws.
|Figure 1: Non-cooperative countries and territories scan shows fronto-nasoethmoidal dysplasia with herniation of intracranial structure|
Click here to view
The non contrast computed tomography (NCCT) face with three-dimension reconstruction was done to confirm the diagnosis. It depicted right side fronto-naso-ethmoidal dysplasia with herniation of the intracranial structure and facial deformity and Tessier 1-13 cleft.  Also intracranially, there was a huge brain cyst filled with cerebrospinal fluid (CSF) [Figure 2]. However, no CSF leak was noticed from nasal defect.
|Figure 2: Clinical photo depicting deformed nose as broad, deviated towards right side and teeth like structures visible through skin disruptions at the tip of nose|
Click here to view
A magnetic resonance imaging (MRI) scan showed skeletal gap in right naso-ethmoidal region, the brain cyst and intracranial communication of naso-ethmoidal dysplasia. The internal bony defect was at the junction of right frontal and ethmoid bone anterior to crista galli in the floor of anterior cranial fossa. The anterior nares were unequal.
The defect was approached trascranially via bicoronal incision. The right hypertrophied ethmoid sinuses were excised, and extra bone was resected using reciprocating saw to gain smooth contour at nasal bridge [Figure 3]. Four malformed teeth were excised from tip of the nose along with abnormal bone [Figure 4]. Next, the projected left frontal and parietal bones were reshaped. As both eyes were shrunken without any proptosis of the eyeball, the right frontoparietal advancement was not done. Also, other associated abnormalities like cranio-orbital bone deformity and facial soft tissue deformity were dealt at the same time along with brain cyst drainage.
|Figure 3: Brain cyst was drained. Excessive bone in the fronto-orbital region was removed and nasal bone was reshaped. Bone flaps were replaced and fixed with wires|
Click here to view
The communication between the anterior cranial fossa and meninges (as diagnosed preoperatively) was surgically repaired with drainage of the cyst. Then the right medial canthus was fixed with the help of 28 gauge wire to the contralateral nasal bone. Surgery was uneventful, and recovery was satisfactory [Figure 5].
Patient was kept on regular follow up and acceptable aesthetic outcome was achieved without any recurrences till 3 years of follow up [Figure 6].
The histological examination of all teeth revealed presence of enamel, dentine, pulp and epithelial element of the root. Also, scanty cemento-osteoblasts were found with the lack of the periodontal ligament.
| Discussion|| |
The developmental anomalies arise during the embryological migration of various tissues in the craniofacial region. The abnormal entrapment of tissues and their failure to separate which is further complicated by abnormal fusion of cranium sutures lead to such abnormalities. The transformation of embryological cells into teeth and projected at nasal tip is an un-described association.
When the embryo is 12 and 13 days old, nasal fossa is formed as a cleft communication between the skull base and oral cavity. The nasal pits are separated from the nasal cavity by a fusion of the opposed surface of the medial and lateral nasal (frontal) processes.  Further, dorsally there is no mesenchymal penetration. In association with the expansion of the nasal cavity, the epithelial wall become stretched thus thin thereby developing into a temporary bucco-nasal membrane. Deviation from the normal development occurs with penetration of mesenchymal cells and transformation of cells leads to the development of ectodermal and mesodermal derivatives. Trans-ethmoidal meningoencephalocele is caused by a part of brain and meninges herniating through an ethmoidal defect which is a subtype of basal meningoencephalocele. ,, The NDSC located at the anterior skull base level, and the basal NDSCs, located at the middle skull base level are different entities in terms of different tissue of origin and embryology.
The most common congenital midline nasal masses are NDSCs. Their clinical importance hinges on their potential to communicate with the central nervous system. Preoperative diagnosis of an intracranial extension allows for referral to a craniofacial team with the appropriate skills and experience for a trans-cranial approach. , All patients require imaging with high resolution multi-planar MRI scans and complimentary fine cut NCCT scan to reveal the anatomical extent of the tract and its relationship to the anterior cranial fossa. A single-stage craniofacial approach for resection of midline dermoid or abnormal tissue extending to the anterior cranial base is effective with minimal morbidity. ,, The significant complications resulted from an early postoperative CSF leak, and residual deformity requires re-operation.
| Acknowledgment|| |
I gratefully acknowledge Prof R. P. Narayan for their valuable contribution.
| References|| |
Melnick M. Current concept in craniofacial anomalies. A symposium in honour of Joseph J Bonner. J Craniofac Genet Dev Biol Suppl 1986; 2:319-30.
Monasterio FO, Taylor JA. Major craniofacial clefts: Case series and treatment philosophy. Plast Reconstr Surg 2008;122: 534-43.
Hughes GB, Sharpino G, Hunt W, Tucker HM. Management of the congenital midline nasal mass: a review. Head Neck Surg 1980;2:222-33.
Al-Ani SA, Locke MB, Rees M, de Chalain TM. Our experiences managing a rare cranio-orbital cleft. J Craniofac Surg 2008;19:819-22.
Nasu W, Kobayashi S, Kashiwa K, Honda T. Secondary craniofacial reconstruction of huge frontoethmoidal encephalomeningocele after primary neurosurgical repair. J Craniofac Surg 2008;19: 171-4.
Charrier JB, Delattre J, Denoyelle F, Garabédian EN. Clinical and embryological approaches to nasal dermoid sinus cysts. Ann Otolaryngol Chir Cervicofac 2003;120:315-29.
Pfaff MJ, Bickerton S, DiLuna M, Steinbacher DM. Transcranial nasoethmoidal dermoids: A review and rationale for approach. J Plast Reconstr Aesthet Surg 2013;66:1725-31.
Hanikeri M, Waterhouse N, Kirkpatrick N, Peterson D, Macleod I. The management of midline transcranial nasal dermoid sinus cysts. Br J Plast Surg 2005;58:1043-50.
David LR, Fisher D, Argenta L. New technique for reconstructing the affected cranium and orbital rim in unicoronal craniosynostosis. J Craniofac Surg 2009;20:194-7.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]