The varying degrees of facial clefting

Facial clefts are deformities of the face that are very rare and involve malformation of a part of the face. The etiology of facial clefts is still unclear though it is thought to be caused by failure of the fusion process during the development of the face. Genetics too is thought to play a role in the occurrence of facial clefts as the incidence of parents with facial clefts having children with facial clefts is quite high. There have also been instances where facial clefts have occurred in children with no family history of facial clefting.

Facial clefting was studied in depth by Dr Paul Tessier who came up with the first classification of facial clefting. Dr. Paul Tessier is also considered to be the father of craniofacial surgery. Tessier’s classification of facial clefts ranges from 0-14. For example, Tessier cleft 0 bisects the maxilla and the nose. Children with these deformities would need multiple surgical procedures for rehabilitation of the defects.

Patient born with cleft deformity and proboscis lateralis

The patient is a 14-year-old boy from near New Delhi, India who was born with a cleft defect and proboscis lateralis. This involved gross deformity of the soft tissues of the nose. He was operated by a board certified plastic surgeon in Delhi shortly after birth for the cleft defect along with primary correction for proboscis lateralis. Cleft alveolar reconstruction/alveolar cleft defect reconstruction was also performed at the same hospital before he completed one year; however, the results of the plastic surgery were unsatisfactory and the patient has always been depressed about his facial deformities.

What is proboscis lateralis?

Proboscis lateralis is a congenital facial abnormality that is characterized by the presence of an incompletely formed rudimentary nasal appendage that is found in association with cleft lip defects. It is located off center to the vertical midline of the face. It is usually attached at the inner canthus of the eye and often associated with maldevelopment of the nasal cavity or paranasal sinuses of the affected side. Proboscis Lateralis is also associated with other craniofacial abnormalities such as orbital anomalies, cleft lip/palate, frontal encephalocele, and holoprosencephaly.

Parents seek surgical correction of the nasal deformity

Seeing how this was affecting their son’s life, the parents took him to Kolkota for consultation with a plastic surgeon. After a thorough examination, the plastic surgeon explained to the parents that this was a complex deformity that had to be addressed by a maxillofacial surgeon who was also a facial cosmetic surgery specialist. It was explained to the parents and the patient that the surgery procedures that he would need would include reconstructive surgery and cosmetic procedures for nose reshaping. He then referred the patient to our hospital for surgical correction of his problem. Our hospital is renowned for cosmetic nose surgery in India.

Patient presents to our hospital for treatment

Dr SM Balaji examined the patient and ordered comprehensive imaging studies. A 3D CT scan was obtained to fully evaluate the extent of the defect. Clinical examination revealed that the patient had a depressed nasal bridge with a deformed nose. His right nostril was smaller in size. Treatment planning was formulated and explained to the patient.

Rhinoplasty or nose job would be performed to correct the patient’s nasal defect. The nasal defect was planned to be repaired using a double decker rib graft, which would be held in place using wires. Size of the right nostril would also be increased by means of a triangular flap along with a Wier excision.

Surgical correction of the patient’s nasal deformity

Under general anesthesia, rib grafts were first harvested through a right inframammary incision. This was followed by a Valsalva maneuver to ensure that there was no perforation of the thoracic cavity. The incision was then closed in layers.

A midline nasal incision was then made and dissection was done up to the dorsum of the nose. The nasal bridge was then augmented using a rib graft followed by placement of a strut graft. A Wier excision was done on the right side. A triangular flap was raised from the right nasolabial region to increase the right nostril size following which the incision was closed with sutures.

The patient and his parents were extremely happy with the results of the surgery and expressed their complete satisfaction before final discharge from the hospital.

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