Abstract
Median cleft lip with nasal dermoid is a very rare occurrence. Midline nasal dermoid results from the abnormal fusion of embryological processes. They can present as asymptomatic midline nasal swelling, infection, or meningitis due to intracranial extension. Median cleft lip also results from failure of fusion of embryological processes and can be complete or incomplete or associated with other congenital anomalies or as a part of syndrome. We present a rare combination of both in a 7-month-old female child who was surgically treated.
KEYWORDS: Intracranial extension, median cleft lip, nasal dermoid
INTRODUCTION
A median cleft lip is a congenital anomaly with a cleft in the central part of upper lip. The incidence is 0.43%–0.73% in the cleft patients and 1:1,000,000 in the general population.[1] It is caused by incomplete fusion of median nasal prominences. It can range from simple notching of vermillion to complete cleft of the lip.
Congenital midline nasal swellings are rare, and the estimated incidence is 1:20,000–40,000 births.[2] A nasal dermoid cyst (NDC) clinically presents as a midline nasal swelling anywhere from the glabella to columella. Midline cleft lips are rare and nasal dermoid are uncommon, while the combination of median cleft lip and nasal dermoid is very uncommon. We report a case of median cleft lip with an NDC in a 7-month-old infant.
CASE REPORT
A female infant weighing 6.5kg at 7 months of age presented to OPD. She had midline cleft of upper lip extending half the distance to the base of nose. Depression and widening of the philtrum was also noted. [Figure 1].
Figure 1.
Preoperative photograph
The child was the first issue of full-term normal vaginal delivery. No previous family history of any craniofacial anomalies was noted. The antenatal history was insignificant with no exposure to any viral infection, drugs, alcohol, or radiation. There were no associated systemic anomalies.
On examination, the median cleft was found to be extending to the gingivolabial sulcus with intact alveolus. The primary and secondary palate was intact. Soft palate movements present on crying. X-ray of the face was done to rule out cleft of the nasal septum which revealed no abnormality of nasal septum or nasal bone.
After routine workup and preanesthetic checkup, the patient was planned for repair of median cleft lip under general anesthesia. The cleft margins were marked in an inverted “V” fashion. Incisions were made as per the markings. When the columnar base was lifted for columnellar lengthening, a cyst/sinus was noted containing whitish material inside it [Figure 2]. On probing it with infant feeding tube 6 Fr in size, it was found extending approximately 1–1.5 cm inside nasal floor. Cyst/sinus was completely excised after ensuring the end over the infant feeding tube, and the specimen was sent for histopathological examination.
Figure 2.
Intraoperative photograph showing white material containing cyst
Repair of the median cleft lip was done after approximating orbicularis oris muscle in midline with vicryl 4-0 and skin closure with 6-0 prolene. Postoperative period was uneventful. Histopathological report of the cyst came out to be a dermoid cyst.
DISCUSSION
The rare occurrence and variety of presentations of the median facial clefts of the upper lip are responsible for the relative paucity of literature. The developmental error that results in midline facial defects usually occurs during the 3rd week of gestation.[3] Median cleft lip occurs due to failure of fusion of the frontonasal process with maxillary process.
NDC occurs due to embryologic defects during formation of the frontonasal region. Nasal dermoid accounts for around 1% of all dermoids. Dermoid cysts usually occur as a sporadic finding. The midline cleft may occur as a secondary event due to the presence of the nasal dermoid.[4]
A complete preoperative workup is required in patients with median cleft lip as they are rare and more frequently associated with other congenital anomalies or be a part of syndromes. In patients with NDCs, intracranial extent should be ruled out preoperatively as they can be a cause of meningitis.[5]
CONCLUSION
Median cleft lip with nasal dermoid is a very rare occurrence. A surgeon should be vigilant when dealing with such case. Proper preoperative evaluation of such cases yields favorable outcomes.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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