Cleft Lip and Palate

Presented by:

Joseph Bernstein, M.D.
Director Of Pediatric Otolaryngology

Joseph Rousso, M.D.
Assistant Professor,
Division of Facial Plastic & Reconstructive Surgery

Co-Directors of New York Eye and Ear Infirmary of Mount Sinai's Cleft Lip and Palate, and Pediatric Craniofacial Anomalies Service

Introduction to Cleft Lip and Palate

Facial clefting is a congenital phenomenon whose explanations have remained elusive since its earliest documented descriptions. With advances in the fields of genetics and an evolving understanding of the embryologic development process, a multifactorial and case specific explanation for clefting has developed.  

Cleft lip and palate comes in many varieties and can be caused by several factors. Additionally, it can be associated with various conditions. It may be related to other syndromes and anomalies or may occur as a completely isolated occurrence. There are over 17,000 known genes that contribute to craniofacial development and over 300 known syndromes with associated clefting.

Cleft lip and palate literally occurs in a variety of shapes and sizes, which synergistically determine the functional and aesthetic consequences. Below are some images depicting clefts of the palate immediately before and immediately after repair.

Additionally an image one year after palate repair shows a strong palate with the ability to function well for speech and swallow.

Different widths and lengths of the cleft lip exist. Some of the cleft lips that we deal with consist of a partial cleft that does not go all the way through to the nose while others are significantly bigger and include significant nasal deformity.

Below is an image showing a partial cleft lip deformity before and immediately after surgical repair. Notice that although, the nose is not directly involved with the cleft, there is an obvious asymmetry to the right nostril (the same side as the cleft lip) as compared to the left nostril. This is slightly overcorrected immediately after surgery in anticipation of exact symmetry once all healing forces occur. From an aesthetic standpoint, it is as important to correct the child’s nose as it is to correct the more obvious lip deformity.

Orofacial clefting can be divided into one of two categories:

1.

Cleft Lip +/- Palate:  occurring at a frequency of about 2 cases per 1,000 births

2..

Isolated Cleft Palate: occurring at a frequency of about 1 case per 1,000 births

These are 2 distinct entities with different patterns of development.

Cleft Lip +/- Palate occurs most typically in the setting of genetic association, although non-mendelian form of inheritance. This means that there seems to be genetic factors, as demonstrated by the higher prevalence in certain races like Asians, Native Americans, and Caucasians as compared to others like African Americans. However, the more stringent rules of genetic predictability do not apply to this malformation.

Isolated cleft palate does not seem to have a predilection for any race and is found with equivelant frequency in infants of all backgrounds.  There is, however, a higher prevalence of this form in females than in males.

Treatment Options:

First and foremost, parents of children with clefts of the lip and/or palate should understand that this is not a condition caused by any fault of their own. Although its development is multifactorial, the cleft deformities are a result of improper fusion during embryonic development and this is not a process that can be controlled by expecting parents.

A high quality interdisciplinary approach is required for treatment of the child with cleft lip and palate. This means that a reputable center with a team of different specialists, each of whom can take care of specific needs, is the best option. A comprehensive diagnosis and treatment plan should begin early after birth and the child consistently followed thereafter.

New York Eye and Ear Infirmary team consists of a broad variety of specialists that treat everything from the surgical correction of the cleft to the therapy necessary for improved speech and feeding. The schematic below represents a basic timeline of the typical ages for treatment. New York Eye and Ear offers a cleft deformity clinic and a team of professionals with a goal-oriented, patient specific agenda for every baby with a cleft deformity. Not every treatment plan will be identical, because every child gets the individual attention necessary for the highest standards of functional and aesthetic reconstruction.

Each child receives an individualized functional and cosmetic assessment as they are evaluated. From a functional standpoint the following questions need to be answered to determine the appropriate courses of actions:

  1. Is this affecting the child’s feeding and growth?
  2. Is this affecting the child’s speech and intelligible language development?
  3. Is this affecting the child’s hearing?

As far as aesthetic considerations in the child with the cleft lip, the following questions need to be answered to determine the appropriate courses of action:

    1. Is this a unilateral (one sided) or bilateral (two sided) cleft lip
    2. Is this a complete cleft of the entire lip going through the floor of the nose?
    3. Is this a partial cleft lip affecting only some parts of the visible lip
    4. Regardless of the size of the cleft how much of the nasal shape and form is affected, and from how far away is the external nasal asymmetry noticed?

Age

Intervention

Birth

Diagnosing clefting and associated anomlies and creating tentative plan of care, including immediate feeding interventions. Identifing whether the child may be a candidate for Naso-Alveolar Molding device (NAM)

10 weeks

Cleft lip repair and cleft nose repair. Insertion of ear tubes. This may be delayed to 4-5 months of age in certain situations including the use of NAM.

9mos- 1year

Cleft palate repair

3-5 years

Speech therapy and adjunctive surgical procedures

10-14 years

Bone grafting for Alveolar clefting

14-17 years

Cleft Rhinoplasty for definitive nasal repair when necessary

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