Planning for Surgery

At your initial consultation, your doctor will discuss the details of the procedure he or she will use, including where the surgery will be performed, the type of anesthesia to be used, possible risks and complications, recovery, costs, and the results you can expect. Your surgeon will also answer any questions you may have about feeding your baby, by breast or by bottle, both before and after the surgery. In most cases, health insurance policies will cover most or all of the cost of cleft lip or cleft palate surgery. Check your policy to make sure your child is covered and to see if there are any limitations on what types of treatment are covered.

All Surgery Carries Some Uncertainty and Risk

When surgery is done by a qualified plastic surgeon with experience in repairing cleft lip or palate, the results can be quite positive. Nevertheless, as with any operation, there are risks associated with surgery and specific complications associated with this procedure. In cleft lip surgery, the most common problem is asymmetry, when one side of the mouth and nose does not match the other side. The goal of cleft lip surgery is to close the separation in the first operation. Occasionally, a second operation may be needed. In cleft palate surgery, the goal is to close the opening in the roof of the mouth so the child can eat and learn to speak properly. Occasionally, poor healing in the palate or poor speech may require a second operation.

Complications

Patients with cleft palate may have the following complications: abnormal midface development, velopharyngeal incompetence, speech defects, and abnormal Eustachian tube function.

Clefts of the primary palate usually have underdeveloped maxillary alveolar arches, and facial growth is abnormal. Cleft patients have an incompetent velopharyngeal valve, which results in nasal regurgitation while swallowing, as well as "typical cleft palate speech": Nasal escape of air (heard as snorting or grunting), a hypernasal quality, and difficulty with consonants. Normal infants begin to develop consonants in speech at age nine months, which influences the timing of operative repair of cleft palate. Even after palate repair, velopharyngeal function may not return entirely to normal, and speech therapy is often helpful.

One aspect of patients with cleft palate which will effect virtually every otolaryngologist is the Eustachian tube dysfunction and middle ear disease. In a landmark article in 1971, Bluestone compared the Eustachian tube function of patients with unrepaired cleft palate and normal-palate patients with a history of serous otitis. Although both groups demonstrated evidence of Eustachian tube dysfunction, the cleft palate patients had a "functional" obstruction of the tube at the nasopharyngeal end--as opposed to the "mechanical" obstruction found in serous otitis patients.

Historically, two muscles have been implicated in the Eustachian tube opening--the levator palatini, and the tensor veli palatini. The levator arises from the petrous bone, courses under and parallel to the tube, and inserts into the dorsal surface of the soft palate. The tensor veli palatini originates from the membranous wall of the tube, and converges into a tendon which wraps around the hamulus and inserts into the aponeurosis. Experimental animal studies from Pittsburgh in the 1980s showed that tensor dysfunction was probably responsible for this middle ear disease. However, this point is controversial and there are several authors who feel that abnormalities of the levator cause the ET dysfunction in cleft palate patients.

How common ?

A cleft lip and palate is the most common type of birth defect in the UK. One in every 700 babies is born with a cleft. It's more common in children who are of northern European and Asian origin, and less common in children of African origin.

The cause of clefts is unknown. However, most researchers believe that they are caused by a combination of environmental and genetic factors.

Approximately 25% of affected babies will be born with a cleft lip, 50% will be born with a cleft lip and a cleft palate, and 25% will be born with a cleft palate.

Cleft lips and a combination of a cleft lip and palate are more common in boys. Cleft palates on their own are more common in girls.

Incidence

Cleft lip and palate are present in one of 1000 live Caucasian births. The incidence of cleft palate alone is one in 500 live births. Considering the cleft deformities of all races grouped together, 50% are cleft lip and palate, 30-35% are palate only, and 15-20% are cleft lip only.

The genetics of cleft lip and palate are not classical Mendelian, but siblings of cleft patients are at greater risk. The risk is directly related to the frequency and severity of the clefts. Approximately 10% of patients with a cleft deformity will have other anomalies at birth.

Classifications/anatomy

Clefts of the lip are incomplete or complete. Incomplete means only a portion of the lip is involved, and may mean anything from a muscular diastasis only, to a large cleft with only a small band of tissue connecting the two sides. A complete cleft lip involves the entire thickness of the lip. Cleft lips are unilateral or bilateral.

The primary palate is anterior to the incisive foramen, and the secondary palate is posterior to the foramen. Clefts of the palate are unilateral if on one side the palatal process of the maxilla is fused with the nasal septum. A bilateral cleft is not attached to the nasal septum, and the septum is visible through the cleft. A complete palatal cleft involves the primary and secondary palate; an incomplete cleft involves the secondary palate only.

Embriology

The lip and primary palate begin to develop at four to five weeks gestational age. The two medial nasal swellings and the maxillary swellings fuse to form the upper lip. The nasal swellings merge at deeper levels also and form the intermaxillary segment, which becomes the triangular primary palate, so embryologically, clefts of the lip are almost invariably associated with clefts of the primary palate.

The secondary palate develops at approximately nine weeks developmental age. It is formed by medial growth of the palatal shelves of the maxilla, which normally fuse together and with the nasal septum as the tongue is pushed down during development. So, the pathogenesis of secondary cleft palate is failure of this fusion to occur.

Histories

Hippocrates (400 BC) and Galen (150 AD) mention cleft lip, but not cleft palate in their writings. For centuries, perforations of the palate were considered to be secondary to syphilis, and cleft palate was not recognized as a congenital disorder until 1556, by Fanco. The first successful closure of a soft palate defect was reported in 1764 by LeMonnier, a French dentist. The first closure of the hard palate was performed in 1834 by Dieffenbach. In the 1930's, Kilner and Wardill independently developed the "pushback" procedure.

A cleft lip and palate is a type of birth defect that affects the top lip and the roof of the mouth (the palate). A cleft is an opening, or split, in either the lip or the palate, or sometimes in both.

Cleft lips and palates are sometimes part of a wider series of birth defects, but usually happen on their own.

Cleft Lip

Cleft lip, with or without cleft palate, is a congenital malformation. Generally, facial clefting results when medial, lateral, and maxillary nasal processes on either left, right or both sides of the forming craniofacial complex do not fuse completely.

Early embryonic changes (during the fourth and tenth weeks of gestation) may result in clefting.

Suspected causes include:

1) environmental insults (i.e. maternal diseases, chemotherapy, radiation, alcohol, excess retinoic acid and anticonvulsant medications); or

2) genetic factors.

Kendall Tullis's Story

I am Kendall Tullis, and I was born with a cleft lip and palate. I am 14 years old. I would have to say that being born with a cleft lip has left its mark on me. I have learned that in order for people to accept me, I have to just be myself. When I was in elementary school, it was hard. I was so very shy then, and I was afraid of most people. I would come home crying many days, because some stupid person would have decided that because I was different they had the right to make fun of me. Those people made me feel like I was less of a person because I was born with something I could not help. During that time, I had only a few friends who I felt comfortable with and who liked me for me. Even one of my good friends Jason made fun of me one day. He did it because one of his friends was teasing me, but it broke my heart. Fortunately, we have become closer since then. I finally realized that not everyone judges you on how you look when I meet Halie, who today is still my best friend. Halie taught me that I should like myself for who I am. She discovered that I was a great person, but that I was just not letting people see it. I had my 22nd surgery on June 17, 2003. I am so afraid of surgeries, but I realize that they will help me make it through my life more easily. Now that I am in high school, I know that when I am myself, people overlook what they see on the outside and see the truly great person I am on the inside. I am not one of the girls who gets the most dates at my school, but I am the most liked because I don’t look at my cleft palate as something horrible but as something that makes me who I am. I cannot stand people who belittle other people. Watching a person being picked on who just stands there and takes people’s insults bothers me, too. When I was little, I used to think if I just ignored people who picked on me, they would stop. But I soon realized that sometimes I could not just go on letting people belittle me, so I faced the bullies. When they realized how insensitive they were being, they felt bad. Having a cleft palate sometimes makes you have to act older than you really are because you have experienced more than most people will in their lifetime. Having a cleft palate has made me realize that I am unique and special in many ways. I would not change anything about me if I could. From www.cleftline.org/story_of_the_month/apr04

Etiology

The upper lip is derived from medial nasal and maxillary processes. Failure of merging between the medial nasal and maxillary processes at 5 weeks' gestation, on one or both sides, results in cleft lip.

Cleft lip usually occurs at the junction between the central and lateral parts of the upper lip on either side. The cleft may affect only the upper lip, or it may extend more deeply into the maxilla and the primary palate. (Cleft of the primary palate includes cleft lip and cleft of the alveolus.) If the fusion of palatal shelves is impaired also, the cleft lip is accompanied by cleft palate, forming the cleft lip and palate abnormality.

Introduction

Cleft lip (cheiloschisis) and cleft palate (palatoschisis) (colloquially known as harelip), which can also occur together as cleft lip and palate, are variations of a type of clefting congenital deformity caused by abnormal facial development during gestation.

Note that harelip is now considered as a derogatory term. A cleft is a fissure or opening -- a gap. It is the non-fusion of the body's natural structures that form before birth.

Clefts can also affect other parts of the face, such as the eyes, ears, cheeks and forehead. In 1976, Dr. Paul Tessier described fifteen lines of cleft. These craniofacial clefts are rare and are frequently described as Tessier clefts using the numerical locator devised by Dr. Tessier.

A cleft lip or palate can be successfully treated with surgery soon after birth. Cleft lips or palates occur in somewhere between one in 600-800 births.