Classification and Anatomy
A cleft lip is classified as unilateral or bilateral and as complete or incomplete. Complete clefts extend through the lip and into the nasal floor, so involve the alveolar ridge or gums. Incomplete clefts do not involve the nasal floor or gums and can vary in extent from a mild scar or notch to a separation through most of the lip. The main anatomic problem in a cleft lip is not only the skin deformity but the fact that the muscle of the lip is separated and aligned incorrectly, attaching to the base of the nose instead of forming a complete sphincter.
Incidence
Cleft lip, with or without cleft palate (CL/P), is genetically, embryologically, and anatomically distinct from the isolated cleft palate (CP). There are some areas of the world that have an increased incidence in cleft lip, with or without cleft palate (CL/P). It is most common in Asians with about 2.1 per 1000 births, Caucasians about 1 per 1000 births, and Africans about 0.4 per 1000 births. CL/P is also about twice more frequent in males than in females. The Left unilateral cleft lip and palate deformity is 2 times more common than the Right, which in turn is 3 times more common than the Bilateral cleft lip and palate deformity.
Isolated cleft palate (CP) in contrast occurs in 0.5 per 1000 births regardless of race, and is twice more frequent in females than males.
Etiology of Cleft Lip and Palate
Many different genetic and environmental factors have been implicated in the etiology of cleft lip and palate. The majority of clefts occur for unknown reasons and affect all populations and socioeconomic groups. There is a slightly higher incidence in areas where nutrition is poor, but the condition is not the fault of any one thing and certainly not the mother or father.
Management
The goals of cleft lip and palate surgery are normal speech, hearing, maxillofacial growth, occlusion, and aesthetic results. It is well established that the care of the cleft lip and palate patient requires a multidisciplinary approach, a dynamic team consisting of craniofacial surgeons, pediatric anesthesiologists, speech therapists, orthodontists, otolaryngologists, pediatricians, nurses, and the parents.
The initial evaluation is usually within the first few weeks of life. Babies with a cleft palate are often not able to create the suction necessary for breast feeding. Our nursing team educates and assists parents with feeding techniques and supplies. Occasionally an appliance must be custom made to temporarily occlude the palate so that the baby can eat and become healthy enough for surgery. This is also the opportunity to discuss with the parents the general plan of care, introduce them to the multidisciplinary team, determine the need for further evaluation by other specialties, and begin planning for surgical repair.
In general the order and timeline is as follows: cleft lip repair as early as 3 months, palatoplasty at about 1year, pharyngeal flaps as early as 4 years old if necessary to improve speech, alveolar bone graft (ABG) at 7-8 years old, definitive septorhinoplasty at 14 years for women and 16 for men, jaw surgery when required. Orthodontics and Speech Therapy are involved throughout the process in the necessary capacity.
Cleft Lip Repair
The first step in repair of the cleft lip is to ensure the baby is mature enough and healthy enough to undergo the surgical procedure. There is a general rule called “The rule of 10s: 10 weeks, 10 lbs, Hct 10” When these conditions are met, the risks of anesthesia in a baby are significantly decreased. Even more importantly is the need to have dedicated Pediatric Anesthesiologists administering the anesthesia for maximum safety. The surgical repair should be performed by surgeons who are specialized in cleft lip and palate surgery in order to ensure safety and optimal results. The repair of a cleft lip includes not only the skin, but also meticulous alignment of the muscle and even repositioning the nasal cartilages to provide maximum symmetry and function.
After cleft lip surgery, it is important that for the next 10 days the baby does not strain the lip muscles by sucking on a nipple or bottle. The LEAP team will aid in determining the best methods of post operative feeding during the healing process. The sutures that are placed on the skin should be removed within 7 days of the surgery for best results. If they stay in longer there is a risk of scarring. After the ten days of syringe feeding, the baby can return to using the nipple or bottle. If there is still a cleft palate, breast-feeding will still not be possible so feeding should continue as taught.
Cleft Palate Repair
The cleft palate is usually repaired at about 1 year of age. The main reason why this is not done sooner, like at the time of lip repair at 3 months, is that surgical dissection around the bones of the palate can cause restriction in growth of the maxilla. Waiting to at least a year of age decreases the risk for growth restriction and so results in better long-term results. Once again, the baby must be healthy and a Pediatric Anesthesiologists should be involved in order to ensure maximum safety during the surgical procedure. Specialized cleft surgeons should perform this procedure in order to minimize blood loss and ensure adequate repair of the muscle and mucosal layers of the palate. If this is not performed meticulously, there is a much greater risk of complications such as tissue loss, fistula formation, and significant scarring resulting in poor speech and problems feeding.
After cleft palate surgery, the LEAP team will aid in determining the best methods of post operative feeding during the healing process. The sutures in this surgery are all in the mouth and are dissolvable, so there is no need to remove them. After 10 days, the baby can begin bottle or nipple feeding. By this time, the opening in the palate has been closed so that the baby can create the suction necessary to feed normally.
Hearing and Speech
The anatomy of a cleft palate deformity includes misalignment of the muscles of the soft palate. The effect of this is two-fold.
The Tensor Veli Palatini is a muscle that attaches from the Eustachian tubes, around the Hamulus, to the posterior hard palate. Its function is to open the Eustachian tubes to allow drainage of the middle ear. Nearly all patients with a cleft palate have malalignment of this muscle and so fluid collection in the ears. This causes frequent ear infections and problems with hearing development.
About 10 -15% of patients who have had a cleft palate, even though it was repaired, still have problems with speech. Usually this is noticed when the child is about 4-5 years old and their speech has developed. This can be due to poor muscle function of the palate or pharynx, and / or scarring in the palate. The result is that speech is hyper nasal, meaning too much air comes out the nose during speech. If this is the case, another surgery may be necessary to simulate the function of the palate in directing airflow though the mouth during speech. This is called a pharyngeal flap, and can be done as early as 4 years old if necessary.
Alveolar Cleft
The cleft lip and palate are repaired as babies, but the alveolar cleft when wide should be repaired at a later time to avoid growth disturbance. Usually this is done when the permanent teeth are coming in, around 7-8 years old. The cleft surgeons work closely with orthodontists in preparing for the procedure so that the teeth and gums are optimally positioned for surgery.
The procedure requires taking bone graft (usually some bone marrow from the hip bone) to fill the gap in the alveolus or gums. After the procedure, the patient needs to be on a soft diet for ten days and be careful in brushing the teeth not to damage the healing incisions in the mucosa of the gums. It takes about 3 months for bone formation to show on an X-ray and confirm that the bone graft has taken. Once this is confirmed, then the orthodontist can resume work on aligning the teeth.
Septorhinoplasty
Although the nose is straightened during the initial repair of the cleft lip, as the baby grows and their face matures, there is still some growth deficiency of the affected cartilage on the side of the cleft. This often results in a deviated septum and nasal asymmetry that require more surgery. We wait until the face has finished growing in order to perform the final repair of the nose, in order to avoid repeat surgeries and increased scarring. This is usually about 14 years for women and 16 for men.
The procedure is again performed under general anesthesia. If significant deformity is present, sometimes a rib cartilage graft is necessary. This means a small incision is made on the lower chest to obtain a piece of spare rib to be used as extra support for the nasal repair. The septorhinoplasty is performed to improve the ability to breathe comfortably through the nose as well as the appearance of the nose.
Summary
The patient with a cleft lip and palate requires a multidisciplinary team approach for adequate evaluation and surgical management. As described in this booklet, multiple surgeries are often required at various stages of growth for optimal results.
On average one of every 600 babies is born with a cleft. It affects all populations. These babies are usually totally normal in every other way and have normal capacities physically and mentally. Unfortunately, without the proper surgery and care, they have little chance of leading normal lives. The LEAP team is dedicated to working with local health care providers and other volunteer groups to provide the best possible care for these patients and their families. In a country of 1,000,000, there would be expected to be 1,700 patients with clefts. In a country of 10 million, that would increase to 17,000. It is necessary to develop programs where a centralized data center can be accessed to see exactly what care has been given and what future care is needed, and then multiple teams be involved in providing this care. The LEAP team is dedicated to helping institute such programs and providing ongoing support to them.
