cleft lip

INTRODUCTION TO MANAGEMENT OF CLEFTS OF THE FACE: PRINCIPLES OF CLEFT REPAIR

Presenter Information

  • Professor: Otasowie D. Osunde, BDS, PhD, FWACS, FAOCMF

  • Department: Oral and Maxillofacial Surgery

  • Faculty: Dentistry, University of Calabar, Calabar

OUTLINE

  • Introduction

  • Embryonic Basis for Cleft Formation

  • Aetiology of Facial Cleft

  • Classification of Orofacial Cleft

  • Problems Associated with Facial Cleft

  • Management

  • Pre-surgical Orthopedics (Naso-Alveolar Moulding)

  • Surgical Repair of Cleft Lip

  • Surgical Repair of Cleft Palate

  • Complications

INTRODUCTION

  • Cleft of the lip and palate are recognized as the second most common congenital anomaly following clubfoot.

  • Infants affected by facial clefting may present with the condition in isolation or as part of a broader range of associated anomalies known as a syndrome.

INCIDENCE

  • The incidence of cleft lip/palate (CL/P) and isolated cleft palate (CP) varies significantly by geographic area and is influenced by racial and ethnic factors.

  • Global Incidence: 1:700 live births

    • Asia: Highest incidence at 1:500 live births

    • Black Africans: Lowest incidence at 1:2,500 live births

EMBRYONIC BASIS FOR CLEFT FORMATION

  • The embryonic development of the face occurs between the 4th to 8th weeks of gestation.

  • The face is formed from the fusion of five developmental processes:

    • Frontonasal process

    • Paired maxillary processes

    • Paired mandibular processes

    • These five processes encircle the primitive mouth, referred to as the stomodeum.

  • The nasal placodes, which are thickened ectodermal structures, develop on either side of the frontonasal process, leading to the formation of bilateral horseshoe-shaped structures.

  • Limbs of the nasal placode evolve into the medial and lateral nasal processes.

FORMATION DETAILS

  • The median nasal process contributes to the formation of:

    • The philtrum

    • Premaxilla (primary palate)

    • Middle segment of the maxilla

  • These structures fuse with the maxillary process to complete the upper lip formation.

  • The lower lip and the mandible develop from the fusion of the paired mandibular processes.

  • The secondary palate is formed via fusion of two shelf-like outgrowths from the maxillary processes.

AETIOLOGY

  • The etiology of facial clefts is multifactorial, encompassing both hereditary and environmental influences.

ENVIRONMENTAL FACTORS

  • Notable environmental influences include:

    • Viral Infections: Such as German measles (Rubella)

    • Irradiation

    • Medications:

    • Salicylates

    • Antiepileptics

    • Diazepam

    • Steroids

    • Substance Use:

    • Alcohol consumption

    • Excessive smoking

    • Nutritional Deficiencies:

    • Folic acid

    • Vitamin A

GENETIC FACTORS

  • Genetic predispositions can contribute to transmission through male sex-linked recessive genes.

  • Several syndromes are associated with cleft lip and palate, including:

    • Van der Woude Syndrome: Characterized by paramedian lip pits, cleft lip/palate, and sometimes tooth agenesis.

    • Oro-facial-digital Syndrome: Includes nasal hypoplasia, cleft palate, digital malformations, and tooth number anomalies (hypodontia/hyperdontia).

    • Patau’s Syndrome (Trisomy 13): Presents with cranial anomalies, cleft lip/palate, mental defects, and cardiac defects.

    • Edward’s Syndrome (Trisomy 18): Involves cranial abnormalities, cleft palate, and microstomia.

    • Down’s Syndrome: Associated with cranial abnormalities, maxillary hypoplasia, dental anomalies, and various developmental delays.

CLASSIFICATION

  • Several classification schemes exist; a simplified classification includes:

    • Syndromic Cleft

    • Non-Syndromic Cleft

    • Cleft of the Lip:

    • Unilateral

    • Bilateral

    • Complete

    • Incomplete

    • Cleft of the Palate:

    • Unilateral

    • Bilateral

    • Complete

    • Incomplete

    • Isolated cleft palate

    • Submucous cleft

PROBLEMS ASSOCIATED WITH CLEFT

  • Muscular Defects:

    • Cleft lip affects the orbicularis muscle.

    • Cleft palate affects:

    • Levator veli palatini

    • Tensor veli palatini

    • Uvula

    • Palatopharyngeus

    • Palatoglossus muscles

  • Nasal Deformities:

    • Shortened columella with base angled towards the non-cleft side

    • Deviation and distortion of the nasal septum on the cleft side

    • Hypertrophy of the inferior turbinate on the cleft side

    • Collapse of medial crura of lower lateral nasal cartilage inferiomedially on the cleft side

    • Collapse and buckling of the lateral crura of lower lateral cartilage on the cleft side

    • Flaring of the alar base

  • Skeletal Deformities:

    • Features can include midface deficiency and maxillary transverse deficiency, leading to class III skeletal and occlusal deformities.

    • Prognathic mandible is also noted.

  • Dental Defects:

    • Congenitally missing teeth and abnormal eruption patterns, especially on the cleft side, along with hypomineralized teeth.

  • Feeding Problems:

    • Difficulty in sucking and nasal regurgitation issues.

  • Speech Difficulties:

    • Often results in hypernasal speech.

  • Otitis Media:

    • Increased susceptibility to ear infections.

MANAGEMENT OF CLEFT LIP AND PALATE PATIENTS

  • Goals of Treatment:

    • To enhance appearance, speech, and self-image

    • To achieve functional and stable occlusion

  • Multi-disciplinary Approach:

    • Treatment involves a coordinated effort from various specialties including:

    • Oral and maxillofacial surgeons

    • Plastic surgeons

    • Orthodontists

    • ENT surgeons

    • Pediatricians

    • Restorative dentists

    • Geneticists

    • Speech therapists

    • Psychologists

    • Social workers

    • Specialists often form a cleft team to provide comprehensive care.

GENERAL MANAGEMENT PROTOCOL FOR CLEFT PATIENT

  • Immediately after Birth:

    • Initial pediatric consultation and evaluation to rule out concomitant anomalies (e.g., cardiac issues, respiratory problems).

    • Parent counseling and feeding instructions provided at this stage.

  • Within Few Weeks of Life:

    • Further evaluation by the cleft team including hearing tests.

  • At Age 10 to 12 Weeks:

    • Surgical repair of the lip typically occurs around this time, often based on the Rule of Ten:

    • Baby approximately 10 weeks old

    • Weight about 10 pounds

    • Hemoglobin concentration of 10 g/dL

  • 12-18 Months:

    • Repair of the cleft palate, ideally before speech development begins.

  • 3 Months Post-Repair of Cleft Palate:

    • Evaluation for speech and language by a speech therapist.

  • 3 to 6 Years:

    • Further evaluation and potential medical and behavioral interventions.

    • Other treatments may include speech therapy and palatal fistula repair.

    • Addressing otitis media is crucial.

  • 5 to 6 Years:

    • Lip/nasal revision if necessary

    • Pharyngeal surgery to correct any speech issues.

  • At 7 Years:

    • Onset of orthodontic treatment (Phase I).

  • At 9-11 Years:

    • Alveolar bone grafting is performed.

  • At 12 Years or Later:

    • Full orthodontic treatment commences (Phase II).

  • 15-18 Years:

    • At the conclusion of orthodontic treatment, implants or fixed bridges for missing teeth may be placed.

  • 18-21 Years:

    • Surgical advancement of the maxilla (orthognathic surgery) may be indicated when growth is completed.

PRE-SURGICAL ORTHOPAEDICS

  • Definition:

    • A specialized orthodontic treatment conducted prior to surgical repair of clefts aimed at narrowing the cleft, especially those involving the alveolus.

  • Technique:

    • Utilizes a Naso-Alveolar Moulding (NAM) appliance, a non-surgical method that reshapes the gingiva, lips, and nose in infants with cleft lip and palate.

    • Repositions maxillary segments and modifies the maxillary arch arrangement to facilitate repair and improve aesthetic outcomes.

  • Timing:

    • Typically performed within the first 3 months of life.

INDICATIONS

  • Indicated in cases where:

    • Cleft divides the alveolar ridge

    • Cleft involves more than 1/3rd of the hard palate length

    • Bilateral clefts of lip or palate

    • Pierre-Robin sequence (micrognathia with cleft palate)

CONTRAINDICATIONS

  • Contraindicated in:

    • Patients residing far away (for regular reviews)

    • Individuals with severe neurological issues

    • Cleft patients with significant concurrent medical problems (e.g., heart defects)

    • Severe feeding issues that may worsen with presurgical treatment

CLEFT LIP REPAIR (CHEILOPLASTY)

  • Timing of Repair:

    • Varies by surgeon, unit, and country; commonly performed between a few days old to 6 months, with repairs at 3 months being preferred.

CRITERIA FOR SUCCESS

  • Successful repair includes:

    • Good apposition of muscle, skin, and mucosa

    • Proper reconstitution of orbicularis oris muscle

    • Continuity of the vermilion border

    • Nasal symmetry

    • Closure of the nasal floor

TECHNIQUES OF REPAIR FOR UNILATERAL CLEFT LIP

  • Common techniques include:

    • Straight Line Repair: (Rose-Thompson technique)

    • Triangular Flaps Technique: (Tennison-Randall)

    • Rectangular Flap Technique: (Le Missurer)

    • Rotation-Advancement Flaps Technique: (Milliard)

TECHNIQUES OF REPAIR FOR BILATERAL CLEFT LIP

  • Techniques include:

    • Veau’s III: (Straight line repair)

    • Noordhoff

    • Manchester techniques

    • Stage Repair: One side repaired at a time.

COMPLICATIONS OF CLEFT LIP REPAIR

  • Potential complications include:

    • Excessive intraoperative bleeding

    • Wound dehiscence

    • Notching of the lip

    • Infection

    • Hypertrophic scar or keloid formation

    • Increased vertical length of the lip

CLEFT PALATE REPAIR (PALATOPLASTY)

  • Timing of Repair:

    • Typically performed between 12 to 18 months of age.

CRITERIA FOR SUCCESS

  • Successful palatoplasty is characterized by:

    • Closure of the oronasal communication

    • Restoration of suction

    • Adequate lengthening of the soft palate

    • Restoration of normal Eustachian tube function

    • Adequate velopharyngeal contact

TECHNIQUES OF CLEFT PALATE REPAIR

  • Commonly utilized techniques include:

    • Van Langenbeck Technique

    • Wardill-Kilner-Veau Technique

    • Furlow’s Technique: (double reversing Z-plasty)

    • Bardach Two-Flap Palatoplasty

COMPLICATIONS OF CLEFT PALATE SURGERY

  • General complications related to surgical procedures (e.g., hemorrhage, postoperative infections) and long-term problems may include:

    • Palatal Fistulas

    • Velopharyngeal Incompetence (VPI)

    • Maxillary Hypoplasia