In the episode "The Face Saver," children and their families travel for miles and then spend weeks waiting outside a clinic in Hyderabad, India, to have their facial deformities surgically removed or repaired. Two of the patients — Suresh and Waseem — have rare conditions. Waseem has what is known as an encephalocele, a congenital midline nasal mass acquired at birth. Suresh has a Tessier no. 2 and no. 12 facial cleft, an extremely rare condition named for renowned French surgeon Dr. Paul Tessier, considered the father of craniofacial surgery.
What is a Tessier cleft?
Tessier clefts occur in less than 5 of every 100,000 births. They are numbered 1-14 to indicate the location and extent of the cleft in the area of the mouth, nose and eyes.
What is an encephalocele?
An encephalocele is a condition in which fetal development of the neural structure is incomplete. It does not close as it should. This leaves a hole that usually occurs in the middle of the skull between the forehead and nose, or in the back of the skull. As a result, parts of the brain's contents can seep out of their normal confines and leak through this hole. If the hole occurs in the front of the skull, it causes a mass to appear over the nose, which in severe cases can widen the distance between the eyes.
Signs and Symptoms
Encephaloceles are very rare. They are usually recognized at birth. However, if they are small, they may go undetected for awhile. According to the Encyclopedia of Neurological Disorders, signs can include the excessive build up of cerebrospinal fluid in the brain (a condition called hydrocephalus), paralyzed arms and legs (spastic quadriplegia), an abnormally small head (microcephaly), difficulty in tasks like walking and reaching because of a lack of coordination (ataxia), delayed or impaired mental and physical development (although intelligence is not always affected), problems with vision, and seizures.
Diagnosis and Treatment
Surgery is difficult if normal vascular structures protrude into the mass. In less severe cases the mass can be removed surgically with no serious complications.