congenital midline nasal masses are rare developmental anomalies estimated to occur in one out of every 20,000 - 40,000 births .
a nasal dermoid cyst ( ndc ) typically presents as a midline mass located anywhere from the glabella to columella .
the majority of ndc 's are diagnosed in infancy and early childhood , but in some cases a lesion may not be noticed until complications occur .
tessier craniofacial clefts may involve entire soft tissue and skeletal elements throughout the course of the cleft , resulting in a distorted craniofacial growth pattern and altered potential for normal growth .
the tessier number 0 cleft patient may present with minimal notching in the lip , vermilion and nose , and a minimally bifid nose .
the bifid nose deformity is an indicator of tessier 's 0 cleft with various degrees of skeletal problems .
we report a rare case of tessier 's 0 cleft with presence of midline ndc within the groove of the bifid nose in a 4-year - old female child .
a 4-year - old female child was referred to department of oral and maxillofacial surgery with a chief complaint of swelling on the nose since 1 year which had increased in size from past 2 months .
there was no previous history of facial trauma , infection , or surgery and familial inheritance .
further , clinical evaluation revealed a swelling on the nasal dorsum and a well - defined groove between two alar domes causing increase in columellar width .
the anterior edge of nasal septum was thick and separated in the shape of a 
y ,  contributing to a bifid appearance of nose and there was presence of a midline punctum over the swelling .
ct scan axial view showed a soft tissue swelling overlying glabella and in between nasal septum with no evidence of intracranial extension . taking all of these findings into consideration ,
all the laboratory tests were in normal ranges and treatment was planned in two stages , excision of the dermoid cyst initially and rhinoplasty in the second stage . under general anesthesia , using a midline vertical incision on dorsum of the nose , cystic lining was exposed and a tract was found extending from punctum to a cystic cavity overlying the nasal bridge .
the entire cystic mass was excised and layered closure was done using 3 - 0 polyglactin and 6 - 0 prolene sutures .
histopathological examination revealed that the cyst is lined by keratinizing squamous epithelium with attached pilosebaceous structures suggestive of dermoid cyst [ figures 16 ] .
the surgical wound healed uneventfully and no recurrence was found during the follow - up period .
frontal view photograph showing swelling on dorsum of the nose and mild depression in midline .
note the presence of a punctum at dorsum of the nose ( ellipse ) worms eye view showing swelling on dorsum of the nose and mild depression in the midline .
note the increased width of columella lateral view showing convexity of swelling over dorsum of the nose axial computed tomography ( ct ) scan view showing the dermoid cyst lining within the confines of nasal septum ( ellipse ) intraoperative photograph showing the cyst after exposure surgical specimen of excised dermoid cyst
they are often associated with a sinus tract , and their extent varies . in particular
, they may remain confined to the surface , involve deep , local tissues , or even extend into intracranial compartment .
patients with midline nasal masses require magnetic resonance imaging ( mri ) or ct imaging to assess the lesion and determine possible intracranial extension .
craniofacial clefts were classified by tessier in 1973 based on his personal experience yielded by clinical , radiologic , and surgical observations .
the exact incidence is not known , but estimates range from 1.4 to 4.9 per 100,000 live births .
these clefts are found along the lines of fusion of different processes responsible for development of face during the first 8 weeks of life .
tessier craniofacial clefts may involve entire soft tissue and skeletal elements throughout the course of cleft , resulting in a distorted craniofacial growth pattern and altered potential for normal growth . the number 0 cleft results from failure of the two medial nasal processes to fuse in the midline .
an incomplete median cleft may occur as an isolated entity or as part of syndromes such as oral - facial - digital ( ofd ) syndrome .
the bifid nose is a malformation that falls within a spectrum of tessier number 0 cleft , occurring directly in the midline of the lip and nose .
the columella and nasal tip are typically bifid and broadened , with a midline depression or groove .
the nose appears shortened in the vertical dimension . in our case , it was a mild tessier 's 0 cleft involving nose without orbital hypertelorism .
many different approaches have been advocated for the removal of nasal dermoids , ranging from a simple extracranial excision to complex procedures in which intracranial excision and nasal reconstruction are required .
incision and drainage , aspiration , curettage , and subtotal excision fail to eradicate the cyst , resulting in recurrence .
the cutaneous punctum is removed in continuity with the cyst by making an elliptical incision around the sinus opening .
other approaches ; such as transverse incision , lateral rhinotomy , external rhinoplasty , inverted - u incision , and degloving procedures ; have also been advocated .
failure to diagnose and excise nasal dermoid properly can result in progressive enlargement , skeletal distortion , infection , meningitis , and intracranial abscess .
we advise a complete evaluation and neurosurgical consultation if any clinical or radiographic indication of intracranial involvement is found . from a surgical point of view for managing tessier 's 0 cleft ,
when planning to treat number 0 cleft , the surgeon must assess severity of the cleft . in more severe cases , when the nasal dorsum is affected and duplication of the septum occurs , resection of the excess tissue over the nasal dorsum , and closing in the midline may be performed .
a z - plasty and broken - line scars are performed only if it is necessary to provide significant soft tissue nasal length , avoiding nonanatomic correction .
some authors have surgically managed the patients with resection of dorsal nasal skin , reshaping of cartilaginous structures , and using bicoronal approach in severe cases .
they have proposed performing lateral osteotomies even in children . however , this may have some deleterious effects on nasal growth and it is better to postpone the lateral osteotomy until postadolescent period .
the combined extraoral and intraoral approach enables a clear exposure of all nasal and lateral maxillary structure and especially in relation with the cleft region . for this reason , repair of bifid nose
may be accomplished without previously described skin excision and wide dissections , yielding a better healing . besides lengthening the columella with this incision involving the only nasal sill area , modified forked flap enables exposure of the nose as well . in conclusion ,
tessier 's 0 cleft with presence of midline ndc within the groove of bifid nose is a very rare occurrence .
successful management of the midline ndcs along with tessier 's 0 cleft requires a thorough preoperative evaluation followed by a meticulous excision of dermoid cyst with appropriate planned surgical technique , later for correction of nasal deformity .
 this is a rare anomaly of midline nasal dermoid cyst ( ndc ) along with tessier 's 0 cleft . 
 midline ndcs present most commonly result from aberrant embryological development , and most commonly give rise to bifid nasal deformity resulting in midline cleft of the nose . 
 craniofacial clefts are among the most disfiguring of all facial anomalies . 
 they exist in a multitude of patterns and with varying degrees of severity . 
 the bifid nose deformity is generally an indicator of tessier number 0 cleft . 
 we present a rare combination of midline ndc with a mild tessier 's 0 cleft in a 4-year - old female child who was surgically treated with wide emphasis given on its diagnosis and other treatment modalities . 
INTRODUCTION
CASE REPORT
DISCUSSION