Pediatric Cleft Lip and Palate Treatment & Management

  • Author: Marie M Tolarova, MD, PhD, DSc; Chief Editor: Maureen Strafford, MD   more...
 
Updated: Mar 23, 2009
 

Medical Therapy

Neonatal care

When a neonate with a cleft is born, a pediatrician has 3 major concerns:

  • Risk of aspiration because of communication between oral and nasal cavities
  • Airway obstruction (in addition to sequelae of aspiration, especially in Robin sequence in which the cleft palate [CP] is combined with micrognathia and the tongue has a normal size)
  • Difficulties with feeding of a child with a cleft and nasal regurgitation

These 3 factors are influenced by the presence of other major or minor anomalies that may, in association with a cleft, represent 1 of 300 known cleft syndromes.[4] Therefore, a neonate with an orofacial cleft should be seen by a medical geneticist as soon as possible.

As with any other medical condition, each case is different. A child with a severe cleft may do very well, whereas a child with a much less severe condition may experience many problems. An individual approach is necessary; however, several major rules apply to every neonate born with a cleft.

A pediatrician/neonatologist is usually the first person to take care of a neonate born with a cleft and the first to talk to the parents. As soon as possible, refer each baby born with orofacial cleft to the cleft palate or craniofacial center, where each specialist evaluates the baby, delineates the best management options and treatment plan, and continuously revises individual procedures and treatment during follow-up visits.

Feeding an infant with a cleft

The vast majority of children with cleft lip and palate (CLP) anomalies are born with a normal birth weight. However, because of feeding and other difficulties mentioned above, the most common problem the pediatrician has to deal with is insufficient weight gain. One of the pediatrician's main responsibilities is to closely monitor the infant's weight. Pediatricians may supervise mothers themselves or may refer them to a nutritionist, feeding specialist, experienced nurse practitioner, or other specialist.

Most children born with cleft lip and palate are unable to be breastfed. Those with cleft palate cannot produce the negative pressure necessary for suction. Mothers of children with a unilateral cleft lip may succeed with breastfeeding when the child is positioned so that the cleft in the lip is obstructed by the mother's breast.

No single right or correct method of feeding has been identified. Parents working together with the health care provider should choose the method that is best for their infant. Most infants can complete a feeding in 18-30 minutes. If more than 45 minutes is required, the infant may be working too hard and may be burning calories that should be used for weight gain. An infant who nurses or bottle feeds every 3-4 hours tends to gain weight better than an infant who feeds frequently (< 2 h apart) for short periods.

Helpful hints for a parent are as follows:

  • Breastfeeding an infant with a cleft
    • In a case of an isolated cleft lip, the infant typically does not experience feeding problems beyond learning how to "latch on" to the nipple at the beginning of the feeding. Infants with cleft palate must squeeze the milk out of the nipple by compressing the nipple between the tongue and whatever portion of the palate that remains.
    • Massaging the breast and applying hot packs on the breast 20 minutes before nursing usually helps.
    • The mother should apply pressure to the areola with her fingers to help the engorged nipple protrude. She should hold the infant in a semi-upright, straddle, or football position. She should support the breast by holding it between her thumb and middle finger, making sure that the infant's lower lip is turned out and the tongue is under the nipple.
    • If the infant cannot hold onto the nipple any more, the mother can collect the remaining milk using an electrical or manual breast pump or by squeezing the breast with both hands and can finish the feeding with collected milk in a bottle.
    • The mother should increase her fluid intake (drink lots of water).
  • Feeding breast milk with a bottle
    • Particularly for infants with bilateral cleft lip and palate, breastfeeding is not possible.
    • The mother can use a breast pump (an electric pump ensures the highest level of success). Then, she can feed the baby with a bottle (see below).
  • Feeding milk formula with a bottle
    • The most appropriate milk formula should be selected by a pediatrician or feeding specialist.
    • Various nipples and bottles are made specifically for infants with clefts. The goal is to find a nipple and bottle that make feeding easy for the infant and still allow ample opportunity to suck.
    • A soft nipple is generally better than a hard nipple (some can be softened by boiling).
    • Use a crosscut nipple to prevent choking. Any nipple can be crosscut manually using a single-edged razor blade. The crosscut is on the tongue side.
    • The bottle should be squeezed and released, not continually squeezed.
    • The nipple is angled to a side of the mouth, away from the cleft.
  • Other recommendations
    • More upright or seated positions prevent the milk from leaking to the nose and causing the infant to choke.
    • Advise the mother to stop feeding and allow the infant to cough or sneeze for a few seconds when nasal regurgitation occurs. A palatal obturator may be used.

Gaining weight and preventing aspiration and ear infections are the most important parts of caring for neonates with a cleft during their first days and weeks of life.

Multidisciplinary team

Most individuals with cleft lip, cleft palate, or both (and many individuals with other craniofacial anomalies) require the coordinated care of providers in many fields of medicine and dentistry, as well as those in speech pathology, otolaryngology, audiology, genetics, nursing, mental health, and social medicine.

Treatment of cleft lip and palate anomalies requires years of specialized care and is costly. The average lifetime medical cost for treatment of one individual affected with a cleft lip and palate is $100,000.[1] Although successful treatment of the cosmetic and functional aspects of orofacial cleft anomalies is now possible, it is still challenging, lengthy, costly, and dependent on the skills and experience of a medical team. This especially applies to surgical, dental, and speech therapies.

Because otitis media with effusion is very common among children with cleft palates, involvement of an otolaryngologist in the multidisciplinary treatment plan is very important. The otolaryngologist performs placement of ventilation tubes in conjunction with the cleft palate repair.[32] If a concurrent cleft lip is present, the ventilation tubes are placed during that repair. Many of these children see otolaryngologists well beyond the time they see many of the other specialists because some children continue to have eustachian tube dysfunction after their palates are closed.

A team for the multidisciplinary treatment of a child with an orofacial cleft includes the following specialists:

  • Pediatrician
  • Nurse practitioner
  • Plastic surgeon
  • Pediatric dentist
  • Otolaryngologist
  • Geneticist
  • Genetic counselor
  • Speech pathologist
  • Orthodontist
  • Maxillofacial surgeon
  • Social worker
  • Psychologist

No single treatment concept has been identified, especially for a cleft lip and palate. The timing of the individual procedures varies in different centers and with different specialists.

Below is the most common treatment protocol presently used in most cleft treatment centers:

  • Newborn - Diagnostic examination, general counseling of parents, feeding instructions, palatal obturator (if necessary); genetic evaluation and specification of diagnosis; empiric risk of recurrence of cleft calculated; recommendation of a protocol for the prevention of a cleft recurrence in the family
  • Age 3 months - Repair of cleft lip (and placement of ventilation tubes)
  • Age 6 months - Presurgical orthodontics, if necessary; first speech evaluation
  • Age 9 months - Speech therapy begins
  • Age 9-12 months - Repair of cleft palate (placement of ventilation tubes if not done at the time of cleft lip repair)
  • Age 1-7 years - Orthodontic treatment
  • Age 7-8 years - Alveolar bone graft
  • Older than 8 years - Orthodontic treatment continues

Other surgical procedures can be performed in patients with severe clefts as necessary (see Surgical Therapy).

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Surgical Therapy

Undoubtedly, closure of the cleft lip is the first major procedure that tremendously changes children's future development and ability to thrive. Variations occur in timing of the first lip surgery; however, the most usual time occurs at approximately age 3 months. Pediatricians used to strictly follow a rule of "three 10s" as a necessary requirement for identifying the child's status as suitable for surgery (ie, 10 lb, 10 mg/L of hemoglobin, and age 10 wk). Although pediatricians are presently much more flexible, and some surgeons may well justify a neonatal lip closure, considering the rule of three 10s is still very useful.

Anatomical differences predispose children with cleft lip and palate and with isolated cleft palate to ear infections. Therefore, ventilation tubes are placed to ventilate the middle ear and prevent hearing loss secondary to otitis media with effusion. In multidisciplinary teams with significant participation of an otolaryngologist, the tubes are placed at the initial surgery and at the second surgery routinely. The hearing is tested after the first placement when ears are clear with tubes. If no cleft surgery is planned early, placing the tubes by age 6 months and monitoring hearing with repeated testing is recommended. Complications include eardrum perforation and otorrhea, particularly in patients with open secondary palates in which closure is planned for later.

For preventive reasons, ear tubes are usually placed when the child is still under general anesthesia for cleft repair.

Detailed surgical treatment is described elsewhere (see surgical articles Craniofacial, Bilateral Cleft Lip Repair, Craniofacial, Bilateral Cleft Nasal Repair, Craniofacial, Unilateral Cleft Nasal Repair, Craniofacial, Unilateral Cleft Lip Repair). Pediatricians may find it useful to inform parents of the kinds of procedures with a child with cleft may undergo.

The most common surgical procedures for a child with a cleft lip and palate anomaly are as follows:

  • Repair of the cleft lip
  • Repair of the cleft palate
  • Revision of the cleft lip
  • Closure and bone grafting of the alveolar cleft
  • Closure of palatal fistulae
  • Palatal lengthening
  • Pharyngeal flap
  • Pharyngoplasty
  • Columellar lengthening
  • Cleft lip rhinoplasty and septoplasty
  • Lip scar revision
  • LeFort I maxillary osteotomy

In addition, orthodontic treatment is very specialized and varies case by case. The 2 stages of orthodontic treatment of a child with cleft lip and palate are as follows:

  • Surgery-related orthodontics
    • Early management (since birth until the time of surgical closure of the palate)
    • Orthodontics related to alveolar bone graft
    • Permanent dentition management
  • Cleft-related orthodontics (not related to surgical treatments)

Patient Education

For excellent patient education resources, visit eMedicine's Children's Health Center.

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Future and Controversies

Available research on the association between orofacial clefts and folic acid consumption highly suggests that a certain proportion of these serious anomalies can be prevented by periconceptional supplementation of folic acid and multivitamins. The preventive approach is assumed to be especially successful in those situations in which environmental factors represent a substantial part of the etiological background.

Primary prevention (ie, prevention of a birth defect before it develops in the embryo or fetus) is attempted for prevention of recurrences in at-risk families in which a previous baby with the anomaly has been born; it is also applicable in the general population for prevention of occurrences.

More than 20 years after the first studies in experimental animals indicated that vitamin deficiency in a mother could cause congenital malformations in the offspring,[33, 34, 35] formiminoglutamic acid excretion testing for defective folate metabolism was found to be positive more often in women pregnant with a child with a neural tube defect (NTD) or other congenital abnormality than in control subjects.[36] Furthermore, periconceptional supplementation with multivitamins[37] or folic acid (Laurence, 1981)[38] was found to have a role in the prevention of NTDs.

Nonetheless, prevention of congenital anomalies seemed impossible to realize as the ultimate goal of teratology,[39] until a randomized, controlled, double-blind, multicenter trial sponsored by the British Medical Research Council (MRC) showed a 72% decrease in the recurrence of NTDs when women ingested 4 mg/d of folic acid from the day of randomization before conception and during 12 weeks thereafter.[40, 41]

However, prophylactic multivitamin therapy, including folic acid, was first used to prevent cleft lip (CL) and palate (CLP) anomaly in future offspring of women whose first child had cleft lip with or without cleft palate (CL/P).[42, 43, 44]

Based on the results of those studies, Burian (of the Czechoslovak Academy of Sciences in Prague) initiated a study in which women who had given birth to a child with an orofacial cleft began taking the multivitamin supplement preparation Spofavit (vitamins A, B-1, B-2, B-6, C, D-3, and E; nicotinamide; and calcium pathothenicum) either immediately after a subsequent pregnancy was confirmed or periconceptionally when pregnancy had been planned.[45] Although Burian's observations were mainly empirical, a prospective trial of periconceptional multivitamin and high folic acid supplementation was conducted in women at risk of giving birth to a child with a cleft lip with or without cleft palate.

In a nonrandomized interventional study completed in the Czech Republic, a dramatic reduction of cleft recurrences was found after periconceptional supplementation with multivitamins and a high dose of folic acid.[46, 19] In this study, 221 pregnancies in women at risk for a child with a cleft lip and palate were prospectively evaluated. The 10-step protocol included multivitamin supplementation with Spofavit and folic acid (10 mg/d), beginning at least 2 months before planned conception and continuing for at least 3 months thereafter. A comparison group comprised 1901 women at risk of giving birth to a child with a cleft lip with or without cleft palate; this group received no supplementation and gave birth within the same period as the study group.

In the supplemented group, 3 of 214 informative pregnancies resulted in neonates with cleft lip with or without cleft palate, a 65.4% decrease from the expected value (see Media file 15).

Subset analysis by proband sex, severity of cleft lip with or without cleft palate, and both variables showed the highest supplementation efficacy in probands with unilateral cleft (82.6% decrease from the expected value).

Recurrence of clefts in supplemented and nonsuppleRecurrence of clefts in supplemented and nonsupplemented groups.

No efficacy was observed for female probands with bilateral cleft lip with or without cleft palate. Generally, the efficacy was higher for subgroups with unilateral clefts than for those with bilateral clefts and for male than for female probands (see Media file 17).

Prevention of cleft lip and palate by periconceptiPrevention of cleft lip and palate by periconceptional vitamin (with particularly high folic acid) supplementation.

Similarly, a large population-based case control study of fetuses and live-born infants in the 1987-1989 cohort of births in California reported that periconceptional use of multivitamins, which usually contain 0.4 mg or more of folic acid, reduced the occurrence of cleft lip with or without cleft palate by approximately 27-50% (see Media file 18).[47] In this study, 734 mothers with an infant with an orofacial cleft and 734 control mothers with an infant without a birth defect were evaluated.

Recurrence of clefts in supplemented and nonsuppleRecurrence of clefts in supplemented and nonsupplemented groups, severity of cleft.

In contrast, the study completed by Hayes did not support a protective association between the periconceptional folic acid supplementation and the risk of oral cleft.[48]

However, the most interesting results that strongly support using a high dose of folic acid in the prevention of nonsyndromic clefts are those of Czeizel and his colleagues in the Hungarian Case-Control Surveillance of Congenital Anomalies.[49, 50] The Hungarian randomized double-blind, controlled trial of periconceptional supplementation with a multivitamin including a low "physiologic" (as the authors call it) dose of folic acid (0.8 mg/d) did not show any preventive effect on the first occurrence of isolated cleft lip with or without cleft palate and cleft palate alone.[49, 50] However, the general evaluation of congenital anomalies in this study indicated a reduction of nonsyndromic clefts after the use of high doses of folic acid (3-9 mg/d) in the early postconception period.[50]

Czeizel's latest article discusses these 2 controversial findings and suggests a "dose-dependent effect" of folic acid in the prevention of orofacial clefts.[49]

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Contributor Information and Disclosures
Author

Marie M Tolarova, MD, PhD, DSc  Professor and Executive Director, UOP Craniofacial Team, Cleft Prevention Program, Department of Orthodontics, University of the Pacific School of Dentistry

Marie M Tolarova, MD, PhD, DSc is a member of the following medical societies: American Cleft Palate/Craniofacial Association, American Society of Human Genetics, and International Association for Dental Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Orval Brown, MD  Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas

Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Alan D Murray, MD  Pediatric Otolaryngologist, ENT for Children; Full-Time Staff, Medical City Dallas Children's Hospital; Consulting Staff, Department of Otolaryngology, Medical Center of Lewisville, Children's Medical Center at Dallas, Cook Children's Medical Center; Full-Time Staff, Texas Pediatric Surgery Center, The Pediatric Surgery Center

Alan D Murray, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American College of Surgeons, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Texas Medical Association

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Maureen Strafford, MD  Arnold P Gold Foundation Associate Professor, Departments of Anesthesiology and Pediatrics, Tufts University and Tufts-New England Medical Center

Maureen Strafford, MD is a member of the following medical societies: American Medical Women's Association, American Pain Society, American Society of Anesthesiologists, International Anesthesia Research Society, Society for Education in Anesthesia, Society for Pediatric Anesthesia, and Society of Cardiovascular Anesthesiologists

Disclosure: Nothing to disclose.

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Classification of orofacial clefts.
Examples of cleft lip.
Examples of cleft lip and palate.
Examples of cleft palate.
Submucous cleft palate.
Bilateral cleft lip on ultrasound.
Median cleft lip on ultrasound.
Prevalence of orofacial clefts (Tolarova and Cervenka, 1998).
Recurrence risk in cleft lip with or without cleft palate.
Highest and lowest risk of recurrence of cleft lip with or without cleft palate.
Recurrence risk in cleft palate.
Etiology of cleft lip and palate anomalies.
Multifactorial threshold model for the distribution of liability for cleft lip and palate.
Four-threshold multifactorial threshold model of the liability for cleft lip and palate.
Four categories of 4-threshold model of liability for cleft lip with or without cleft palate (Tolarova, 1990).
Recurrence of clefts in supplemented and nonsupplemented groups.
Prevention of cleft lip and palate by periconceptional vitamin (with particularly high folic acid) supplementation.
Recurrence of clefts in supplemented and nonsupplemented groups, severity of cleft.
Decreased occurrence of orofacial clefts.
 
 
 
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