Cleft Lip and Palate

Author Credentials 

William O. Collins, MD
Associate Professor
Department of Otolaryngology
University of Florida College of Medicine
Gainesville, FL

Module Summary

Cleft lip and palate are the second most common congenital malformation, and can cause significant functional compromise. While diagnosis is relatively straightforward, long term management is best performed with a multidisciplinary team. Successful care of these patients may involve orthodontic treatment, surgical repair, and speech and swallowing therapy. Long term complications such as chronic Eustachian tube dysfunction and velopharyngeal insufficiency are still possible even after surgical repair, and must be managed appropriately.

Module Learning Objectives 
  1. Review the embryologic development of the lip and palate, and how perturbations in that developmental process lead to cleft lip and/or palate.
  2. Classify the anatomical pattern of cleft lip and/or palate.
  3. Explain functional complications associated with the presence of a cleft lip and/or palate.
  4. Develop surgical strategies for repair of a cleft lip and/or palate.

 

Embryology

Learning Objectives 

To describe the embryologic development of the lip and palate.

 

References 
  1. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb;40(1):27-60,vi.
  2. Sykes JM, Tollefson TT. Management of the cleft lip deformity. Facial Plast Surg Clin North Am. 2005 Feb;13(1):157-67.
  3. Hartzell LD, Kilpatrick LA. Diagnosis and Management of Patients with Clefts: A Comprehensive and Interdisciplinary Approach. Otolaryngol Clin N Am. 2014;47:821-852.

Anatomy

Learning Objectives 

To describe patterns of abnormal lip and palate development.

 

References 
  1. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb;40(1):27-60,vi.
  2. Hartzell LD, Kilpatrick LA. Diagnosis and Management of Patients with Clefts: A Comprehensive and Interdisciplinary Approach. Otolaryngol Clin N Am. 2014;47:821-852.

Pathogenesis

Learning Objectives 

To identify factors that can adversely affect lip and palate development in utero.

 

References 
  1. Jones MC. Etiology of facial clefts: prospective evaluation of 428 patients. Cleft Palate J. 1988 Jan;25(1):16-20. 

Basic Science

Learning Objectives 

To identify factors that affect the embryologic fusion of the lip and palate.

 

 

References 
  1. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb;40(1):27-60,vi.
  2. Hartzell LD, Kilpatrick LA. Diagnosis and Management of Patients with Clefts: A Comprehensive and Interdisciplinary Approach. Otolaryngol Clin N Am. 2014;47:821-852.

Incidence

Learning Objectives 

To describe the incidence of:

  • Cleft lip with or without cleft palate
  • Cleft palate alone

 

 

References 
  1. Jones MC. Etiology of facial clefts: prospective evaluation of 428 patients. Cleft Palate J. 1988 Jan;25(1):16-20. 

Genetics

Learning Objectives 
  1. To recognize which ethnic groups may be at a higher risk of development of cleft lip and/or palate.
  2. To identify the role of prenatal diagnosis and genetic counseling in select cases of cleft lip and/or palate.

 

 

References 
  1. Stanier P, Moore GE. Genetics of cleft lip and palate: syndromic genes contribute to the incidence of non-syndromic clefts. Hum Mol Genet. 2004 Apr 1;13 Spec No 1:R73-81.

Patient Evaluation

Learning Objectives 

To describe the comprehensive evaluation of a patient with cleft lip and/or palate.

 

References 
  1. Capone RB, Sykes JM. The cleft and craniofacial team: the whole is greater than the sum of its parts. Facial Plast Surg. 2007 May;23(2):83-6. 
  2. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb;40(1):27-60,vi.
  3. Hartzell LD, Kilpatrick LA. Diagnosis and Management of Patients with Clefts: A Comprehensive and Interdisciplinary Approach. Otolaryngol Clin N Am. 2014;47:821-852.

Measurement of Functional Status

Learning Objectives 

To identify functional deficits associated with the presence of a cleft lip and/or palate.

 

 

References 
  1. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb;40(1):27-60,vi.
  2. Hartzell LD, Kilpatrick LA. Diagnosis and Management of Patients with Clefts: A Comprehensive and Interdisciplinary Approach. Otolaryngol Clin N Am. 2014;47:821-852.

Imaging

Learning Objectives 

To describe the role of imaging in the evaluation and management of patients with cleft lip and/or palate.

 

 

Treatment

Learning Objectives 

To identify factors which are critical in the treatment strategy of patients with cleft lip and/or palate.

 

References 
  1. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb;40(1):27-60,vi.
  2. Hartzell LD, Kilpatrick LA. Diagnosis and Management of Patients with Clefts: A Comprehensive and Interdisciplinary Approach. Otolaryngol Clin N Am. 2014;47:821-852.

Surgical Therapies

Learning Objectives 

To describe surgical techniques to repair:

  1. Cleft lip:
    1. Lip adhesion
    2. Unilateral cleft lip
    3. Bilateral cleft lip
  2. Cleft palate
    1. Unilateral cleft palatoplasty
    2. Bilateral cleft palatoplasty
    3. Repair of secondary cleft palate

 

 

 

References 
  1. Furlow LT. Cleft palate repair by double opposing Z-plasty. Plast Reconstr Surg. 1986 Dec;78(6):724-38. 
  2. Millard Jr DR. A primary camouflage of the unilateral harelook. Transactions of the 1st International Congress of Plastic Surgery, Stockholm. Baltimore: Wiliiams & Wilkins; 1957. 
  3. Sykes JM, Tollefson TT. Management of the cleft lip deformity. Facial Plast Surg Clin North Am. 2005 Feb;13(1):157-67. 
  4. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb;40(1):27-60,vi.
  5. Hartzell LD, Kilpatrick LA. Diagnosis and Management of Patients with Clefts: A Comprehensive and Interdisciplinary Approach. Otolaryngol Clin N Am. 2014;47:821-852.
  6. Tollefson TT, Senders CW, Sykes JM. Changing Perspectives in Cleft Lip and Palate: From Acrylic to Allele. Arch Facial Plast Surg. 2008;10(6):395-400.

Rehabilitation

Learning Objectives 

To recognize the long term implications of treatment in a cleft lip and/or palate patient.

 

References 
  1. Capone RB, Sykes JM. The cleft and craniofacial team: the whole is greater than the sum of its parts. Facial Plast Surg. 2007 May;23(2):83-6. 

Case Studies

  1. A newborn baby is noted to have an isolated unilateral cleft lip. What other specialists should be involved in the care of this child? What are some of the immediate concerns that need to be addressed? What factors go into the decision about when to perform surgical repair? Are there any adjunctive measures that may help with surgical success? What are the surgical approaches for repair of a cleft lip?
  2. A 6 month old child has a unilateral complete cleft lip and palate. What are some options to help the child feed better?  What is the expected timing and sequence of surgical repair of these congenital defects? What otologic conditions is the child most prone to developing? What otologic interventions could be offered? What surgical options exist to repair the cleft palate?

 

 

Complications

Learning Objectives 

To recognize common complications and their management in patients undergoing surgical repair of a cleft lip and/or palate:

  • Otologic complications of palatal dysfunction
  • Airway management
  • Velopharyngeal insufficiency

 

 

References 
  1. Arosarena OA. Cleft lip and palate. Otolaryngol Clin North Am. 2007 Feb; 40(1):27-60,vi.
  2. Mahboubi H, Truong A, Pham NS. Prevalence, demographics, and complications of cleft palate surgery. Int J Pediatr Otorhinolaryngol. 2015;79(6):803-807.
  3. Kuo CL, Tsao YH, Cheng HM, et al. Grommets for otitis media with effusion in children with cleft palate: a systematic review. Pediatrics. 2014;134(5):983-994.

Review

Review Questions 
  1. What is the most common classification scheme or description used to describe cleft lip and palate?
  2. What are commonly associated genetic syndromes?
  3. Who are the members of a typical cleft team, and when should they respectively be involved in the care of a child with a cleft lip +/- palate?
  4. What are some adjunctive surgical measures which can aid in surgical repair of a cleft lip?
  5. Describe the common surgical approaches to repair of a cleft lip, and a cleft palate.
  6. What are some potential long term sequelae of a cleft palate, and what are potential surgical options to treat those sequelae?
Resources 

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