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Reconstruction of Cleft Lip and
        Palate Defect

         Amin Abusallamah
Outline
1.   INTRODUCTION
2.   EMBRYOLOGY
3.   DEVELOPMENT OF THE PALATE
4.   INCIDENCE AND ETIOLOGY
5.   CLASSIFICATION
6.   DEFECTS IN CLEFT LIP AND PALATE PATIENTS
7.   RECONSTRUCTION OF CLEFT LIP AND PALATE
INTRODUCTION

• A cleft lip and palate is a type of birth defect that
  affects the upper lip and the roof of the palate.
  During the 6th to 10th week of pregnancy, the bones
  and tissues of a baby’s upper jaw, nose, and mouth
  normally fuse to form the palate and the upper lip. If
  the tissue in the developing mouth and the palate
  don’t fuse together, a baby could be born with a
  condition called cleft lip or cleft palate.
INTRODUCTION

• Cleft lip and palate are the
  one of the commonest group
  of congenital malformations
  affecting the head and neck.
EMBRYOLOGY
• The lateral swellings will form the
  alae of the nose.
• The medial swellings will give rise
  to four areas:
(1)   The middle portion of the nose.
(2)   The middle portion of the upper lip.
(3)   The middle portion of the maxilla.
(4)   The maxillary swellings will
      approach the medial and lateral
      nasal   swellings     but    remain
      separated from them by well
      marked grooves.
EMBRYOLOGY

• These swellings simultaneously merge with each
  other and with the maxillary swellings laterally.

• The upper lip is formed by the two medial nasal
  swellings and the two maxillary swellings.
EMBRYOLOGY

• The structures formed
  by the two merged
  swellings are known
  together as the inter-
  maxillary segment.
Development of the Palate

• It develops from two parts:

1. The primary palate.
                                2
2. The secondary palate.        1
                                    2
Primary palate
• The primary palate appears earlier than the
  secondary palate at the beginning of the 6th W.I.U.
• The primary palate develops from the inter-maxillary
  segment.
• At first the primary palate and the central parts of the
  upper lip are formed as one unite. At the 8th week IU
  the primary palate is separated from the upper lip by
  the formation of the vestibular lamina.
Secondary palate
• The secondary palate is that part of the palate
  posterior to the incisive fossa.
• It formed of the remaining part of the hard palate
  and the soft palate .
Palatal Shelf Elevation
• From 8-9 weeks I.U., the lateral palatine shelves slide
  or roll over the tongue and acquire a horizontal
  position (shelf elevation).


• This process occurs when
  the shelves have developed
  sufficient strength to slide
  over the tongue.
Fusion of the Primary & Secondary Palates

• The palatine closure begins
  from the 9th to the 12th
  W.I.U. After the shelves are
  in a horizontal position and
  they attain their final
  growth, shelf fusion occurs.
Developmental Anomalies Cleft Palate

• It may be due to lack of growth.
• It may also be due to interference with palatal
  shelves elevation.
• Failure of fusion between the median and lateral
  palatine processes and the nasal septum.
• It may be due to initial fusion with interruption of
  growth at any point along its course.
CLASSIFICATION

Clefts can be :
• Unilateral or bilateral;
• Incomplete or complete;
  and may involve the
  lip,     nose,      primary
  palate, and/or secondary
  palates
INCIDENCE

• Cleft lip = 1: 700                     Males (2:1)

• Cleft palate (isolated) = 0.45: l000   Females (2:1)


• Cleft Lip & Palate: 0.74 : l000        Males (2:1)

• Left Side more Involved.
ETIOLOGY


• The exact cause of clefting is unknown.

• Instead clefts are thought to be of a multifactorial
  etiology with a number of potential contributing
  factors. These factors may include chemical
  exposures, radiation, maternal hypoxia, teratogenic
  drugs, nutritional deficiencies, physical obstruction,
  or genetic influences.
ETIOLOGY

• Some     of    these     genes      include     the
  MSX, LHX, goosecoid, and DLX genes.

• Some Syndromes Associated with Cleft Palate like:


 • Marfan syndrome          • Edward syndrome
 • Apert syndrome           • Treacher collins
 • Downs syndrome           • Pierre Robin
 • Digeorge syndrome        • Stickler syndrome
DEFECTS IN CLEFT LIP AND PALATE
                   PATIENTS
Deformities:
1. Dental Problems:
Number, Shape, Eruption, Mineralization.

2. Skeletal Problems:
Max. deficiency, Mand. Prognth, Class III Occlusion.

3. Nasal problems:
Alae flared, Columella pulled to non cleft side.

4. Feeding Problems:
Nasal Regurg, Weak sucking, Weak swallowing reflux.
DEFECTS IN CLEFT LIP AND PALATE
                  PATIENTS
4. Ear Problems:
Recurrent Middle Ear infection, Possible Deafness


5. Speech Problems:
Retardation of consonants, Hypernasality,
Articulation Defects, Hearing problem


6. Associated Anomalies:
Congenetal heart defects, Mental retardation
RECONSTRUCTION OF CLEFT LIP AND
              PALATE
• The timing of cleft lip and palate repair is
  controversial. Despite a number of meaningful
  advancements in the care of patients with cleft lip
  and palate, a lack of consensus exists regarding the
  timing and specific techniques used during each
  stage of cleft reconstruction.
Reconstruction of cleft lip and palate defect
Presurgical Taping
• Facial taping with elastic devices is
  used for application of selective
  external pressure and may allow
  for improvement of lip and nasal
  position prior to the lip repair
  procedure.

• Some surgeons prefer presurgical orthopedic (PSO)
  appliances rather than lip taping to achieve the
  same goals.
Presurgical Orthopedics
• appliances are composed
  of a custom-made acrylic
  base plate that provides
  improved anchorage in
  the     molding       of
  lip, nasal, and alveolar
  structures   during  the
  presurgical phase     of
  treatment.

                             Nasoalveolar Molding (NAM)
1- Unilateral Cleft Lip Repair
• Clefts of the lip and nose that are unilateral present
  with a high degree of variability.

• The repair technique is usually performed after 10
  weeks of age.
1- Unilateral Cleft Lip Repair
• The basic premise of the repair is to create a three-
  layered closure of skin, muscle, and mucosa that
  approximates normal tissue and excises hypoplastic
  tissue at the cleft margins.

• Critical in the process is the reconstruction of the
  orbicularis oris musculature into a continuous
  sphincter.
Millard rotation-advancement
                technique
• has the advantage of allowing for each of the incision
  lines to fall within the natural contours of the lip and
  nose. This is an advantage because it is difficult to
  achieve symmetry in the unilateral cleft lip and nose
  with the normal side.

• Primary nasal reconstruction may be considered at
  the time of lip repair to reposition the displaced
  lower lateral cartilages and alar tissues
McComb`s techniques
• McComb`s technique that has
  become popular, consisting of
  dissecting the lower lateral
  cartilages free from the alar base
  and the surrounding attachments
  through an alar crease incision.
  This allows the nose to be
  bolstered and/or stented from        complete unilateral cleft of the lip is
                                       shown highlighting the hypoplastic tissue
  within the nostril to improve        in the cleft site that is not used in the
                                       reconstruction.
  symmetry.
2- Bilateral Lip Repair
• Bilateral cleft lip repair can be
  one of the most challenging
  technical procedures performed
  in children with clefts.

• The lack of quality tissue present and the widely
  displaced segments are major challenges to
  achieving exceptional results, but superior technique
  and adequate mobilization of the tissue flaps usually
  yields excellent esthetic results.
2- Bilateral Lip Repair
• Some surgeons have used aggressive techniques to
  surgically lengthen the columella and preserve
  hypoplastic tissue.

• Early and aggressive tissue flaps in the nostril and
  columella areas do not look natural after significant
  growth has occurred.
The bilateral cleft of the lip and maxilla shown here
 is complete and highlights the hypoplastic tissue
                along the cleft edges.
3- Cleft Palate Repair
• two main goals of cleft palate repair during infancy:
1. The water-tight closure of the entire oronasal
   communication involving the hard and soft palate.
2. The repair of the musculature within the soft palate
   that is critical for normal creation of speech.
3- Cleft Palate Repair
• The exact timing of repair of a palate cleft is
  controversial. Generally the velum must be closed
  prior to the development of speech sounds that
  require an intact palate.

• Average this level of speech production is observed
  by about 18 months of age in the normally
  developing child.
3- Cleft Palate Repair
• If the repair is completed after this
  time, compensatory speech articulations may result.

• Repair completed prior to this time allows for the
  intact velum to close effectively, appropriately
  separating the nasopharynx from the orophayrynx
  during certain speech sounds.
3- Cleft Palate Repair
• When repair of the palate is performed between 9
  and 18 months of age, the incidence of associated
  growth   restriction   affecting   the   maxillary
  development is approximately 25%.

• Cleft palate reconstruction requires the mobilization
  of multilayered flaps to reconstruct the defect due to
  the failure of fusion of the palatal shelves.
3- Cleft Palate Repair
• surgeon must also reconstruct the musculature of
  the velopharyngeal mechanism.

• The musculature of the levator palatini is abnormally
  inserted on the posterior aspect of the hard palate
  and therefore must be disinserted and reconstructed
  in the midline.
Bardach two-flap palatoplasty
• Uses two large full thickness layered dissection and
  brought to the midline for closure

A, Unilateral cleft of the primary and secondary
palates, from the anterior vestibule to the uvula.
B, This tech.requires two large full-thickness
mucoperiosteal flaps to be elevated from each
palate shelf. The anterior of the cleft is not
reconstructed until the mixed dentition stage.
C, layered closure is performed palatoplasty by
reapproximating the nasal mucosa.
D, Once the nasal mucosa and musculature of
the soft palate are approximated, the oral
mucosa is closed in the midline
Furlow double-opposing Z plasty
• Common technique attempts to lengthen the palate
  by taking advantage of a Z-plasty technique on both
  nasal mucosa and oral mucosa.
A, complete cleft of the secondary palate (both
hard and soft). B, Z-plasty technique requires
that separate Z-plasty flaps be developed on the
oral and then nasal side.
C, The flaps are then transposed to theoretically
lengthen the soft palate. A nasal side closure is
completed in the standard fashion anterior to
the junction of the hard and soft palate.
 D, The oral side flaps are then transposed and
closed in a similar fashion completing the palate
closure.
4- Bone Grafting
• At one time, bone grafting tended to be performed
  only in bilateral alveolar clefts to fix a mobile
  premaxilla to the maxilla. however now been
  extended to include the achievement of a continuous
  alveolus in all cleft patients with an alveolar defect..
References
1. Wray, David, et al. Textbook for General and Oral Surgery.

2. Langman's Medical embryologyM, 2003..

3. Classification and birth prevalence of orofacial clefts. Tolarova MM, Cervenka.
   2, San Francisco : Am J Med Genet, 1998, Vol. 75, pp. 126–37. 1552-4833.

4. Contemporary Oral and Maxillofacial Surgery, 4 ED.

5. Identification of susceptibility loci for nonsyndromic cleft lip with or without
   cleft palate in a two stage genome scan of affected sib pairs. Prescott NJ, Lees
   MM,Winter RM,Malcolm S. 3, London : Hum Genet, 2000, European Journal of
   Human Genetics, Vol. 106, pp. 345-350.

6. PETERSON'S PRINCIPLES OF ORAL AND MAXILLOFACIAL SURGERY Second
   Edition.
Thank
 you

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Reconstruction of cleft lip and palate defect

  • 1. Reconstruction of Cleft Lip and Palate Defect Amin Abusallamah
  • 2. Outline 1. INTRODUCTION 2. EMBRYOLOGY 3. DEVELOPMENT OF THE PALATE 4. INCIDENCE AND ETIOLOGY 5. CLASSIFICATION 6. DEFECTS IN CLEFT LIP AND PALATE PATIENTS 7. RECONSTRUCTION OF CLEFT LIP AND PALATE
  • 3. INTRODUCTION • A cleft lip and palate is a type of birth defect that affects the upper lip and the roof of the palate. During the 6th to 10th week of pregnancy, the bones and tissues of a baby’s upper jaw, nose, and mouth normally fuse to form the palate and the upper lip. If the tissue in the developing mouth and the palate don’t fuse together, a baby could be born with a condition called cleft lip or cleft palate.
  • 4. INTRODUCTION • Cleft lip and palate are the one of the commonest group of congenital malformations affecting the head and neck.
  • 5. EMBRYOLOGY • The lateral swellings will form the alae of the nose. • The medial swellings will give rise to four areas: (1) The middle portion of the nose. (2) The middle portion of the upper lip. (3) The middle portion of the maxilla. (4) The maxillary swellings will approach the medial and lateral nasal swellings but remain separated from them by well marked grooves.
  • 6. EMBRYOLOGY • These swellings simultaneously merge with each other and with the maxillary swellings laterally. • The upper lip is formed by the two medial nasal swellings and the two maxillary swellings.
  • 7. EMBRYOLOGY • The structures formed by the two merged swellings are known together as the inter- maxillary segment.
  • 8. Development of the Palate • It develops from two parts: 1. The primary palate. 2 2. The secondary palate. 1 2
  • 9. Primary palate • The primary palate appears earlier than the secondary palate at the beginning of the 6th W.I.U. • The primary palate develops from the inter-maxillary segment. • At first the primary palate and the central parts of the upper lip are formed as one unite. At the 8th week IU the primary palate is separated from the upper lip by the formation of the vestibular lamina.
  • 10. Secondary palate • The secondary palate is that part of the palate posterior to the incisive fossa. • It formed of the remaining part of the hard palate and the soft palate .
  • 11. Palatal Shelf Elevation • From 8-9 weeks I.U., the lateral palatine shelves slide or roll over the tongue and acquire a horizontal position (shelf elevation). • This process occurs when the shelves have developed sufficient strength to slide over the tongue.
  • 12. Fusion of the Primary & Secondary Palates • The palatine closure begins from the 9th to the 12th W.I.U. After the shelves are in a horizontal position and they attain their final growth, shelf fusion occurs.
  • 13. Developmental Anomalies Cleft Palate • It may be due to lack of growth. • It may also be due to interference with palatal shelves elevation. • Failure of fusion between the median and lateral palatine processes and the nasal septum. • It may be due to initial fusion with interruption of growth at any point along its course.
  • 14. CLASSIFICATION Clefts can be : • Unilateral or bilateral; • Incomplete or complete; and may involve the lip, nose, primary palate, and/or secondary palates
  • 15. INCIDENCE • Cleft lip = 1: 700 Males (2:1) • Cleft palate (isolated) = 0.45: l000 Females (2:1) • Cleft Lip & Palate: 0.74 : l000 Males (2:1) • Left Side more Involved.
  • 16. ETIOLOGY • The exact cause of clefting is unknown. • Instead clefts are thought to be of a multifactorial etiology with a number of potential contributing factors. These factors may include chemical exposures, radiation, maternal hypoxia, teratogenic drugs, nutritional deficiencies, physical obstruction, or genetic influences.
  • 17. ETIOLOGY • Some of these genes include the MSX, LHX, goosecoid, and DLX genes. • Some Syndromes Associated with Cleft Palate like: • Marfan syndrome • Edward syndrome • Apert syndrome • Treacher collins • Downs syndrome • Pierre Robin • Digeorge syndrome • Stickler syndrome
  • 18. DEFECTS IN CLEFT LIP AND PALATE PATIENTS Deformities: 1. Dental Problems: Number, Shape, Eruption, Mineralization. 2. Skeletal Problems: Max. deficiency, Mand. Prognth, Class III Occlusion. 3. Nasal problems: Alae flared, Columella pulled to non cleft side. 4. Feeding Problems: Nasal Regurg, Weak sucking, Weak swallowing reflux.
  • 19. DEFECTS IN CLEFT LIP AND PALATE PATIENTS 4. Ear Problems: Recurrent Middle Ear infection, Possible Deafness 5. Speech Problems: Retardation of consonants, Hypernasality, Articulation Defects, Hearing problem 6. Associated Anomalies: Congenetal heart defects, Mental retardation
  • 20. RECONSTRUCTION OF CLEFT LIP AND PALATE • The timing of cleft lip and palate repair is controversial. Despite a number of meaningful advancements in the care of patients with cleft lip and palate, a lack of consensus exists regarding the timing and specific techniques used during each stage of cleft reconstruction.
  • 22. Presurgical Taping • Facial taping with elastic devices is used for application of selective external pressure and may allow for improvement of lip and nasal position prior to the lip repair procedure. • Some surgeons prefer presurgical orthopedic (PSO) appliances rather than lip taping to achieve the same goals.
  • 23. Presurgical Orthopedics • appliances are composed of a custom-made acrylic base plate that provides improved anchorage in the molding of lip, nasal, and alveolar structures during the presurgical phase of treatment. Nasoalveolar Molding (NAM)
  • 24. 1- Unilateral Cleft Lip Repair • Clefts of the lip and nose that are unilateral present with a high degree of variability. • The repair technique is usually performed after 10 weeks of age.
  • 25. 1- Unilateral Cleft Lip Repair • The basic premise of the repair is to create a three- layered closure of skin, muscle, and mucosa that approximates normal tissue and excises hypoplastic tissue at the cleft margins. • Critical in the process is the reconstruction of the orbicularis oris musculature into a continuous sphincter.
  • 26. Millard rotation-advancement technique • has the advantage of allowing for each of the incision lines to fall within the natural contours of the lip and nose. This is an advantage because it is difficult to achieve symmetry in the unilateral cleft lip and nose with the normal side. • Primary nasal reconstruction may be considered at the time of lip repair to reposition the displaced lower lateral cartilages and alar tissues
  • 27. McComb`s techniques • McComb`s technique that has become popular, consisting of dissecting the lower lateral cartilages free from the alar base and the surrounding attachments through an alar crease incision. This allows the nose to be bolstered and/or stented from complete unilateral cleft of the lip is shown highlighting the hypoplastic tissue within the nostril to improve in the cleft site that is not used in the reconstruction. symmetry.
  • 28. 2- Bilateral Lip Repair • Bilateral cleft lip repair can be one of the most challenging technical procedures performed in children with clefts. • The lack of quality tissue present and the widely displaced segments are major challenges to achieving exceptional results, but superior technique and adequate mobilization of the tissue flaps usually yields excellent esthetic results.
  • 29. 2- Bilateral Lip Repair • Some surgeons have used aggressive techniques to surgically lengthen the columella and preserve hypoplastic tissue. • Early and aggressive tissue flaps in the nostril and columella areas do not look natural after significant growth has occurred.
  • 30. The bilateral cleft of the lip and maxilla shown here is complete and highlights the hypoplastic tissue along the cleft edges.
  • 31. 3- Cleft Palate Repair • two main goals of cleft palate repair during infancy: 1. The water-tight closure of the entire oronasal communication involving the hard and soft palate. 2. The repair of the musculature within the soft palate that is critical for normal creation of speech.
  • 32. 3- Cleft Palate Repair • The exact timing of repair of a palate cleft is controversial. Generally the velum must be closed prior to the development of speech sounds that require an intact palate. • Average this level of speech production is observed by about 18 months of age in the normally developing child.
  • 33. 3- Cleft Palate Repair • If the repair is completed after this time, compensatory speech articulations may result. • Repair completed prior to this time allows for the intact velum to close effectively, appropriately separating the nasopharynx from the orophayrynx during certain speech sounds.
  • 34. 3- Cleft Palate Repair • When repair of the palate is performed between 9 and 18 months of age, the incidence of associated growth restriction affecting the maxillary development is approximately 25%. • Cleft palate reconstruction requires the mobilization of multilayered flaps to reconstruct the defect due to the failure of fusion of the palatal shelves.
  • 35. 3- Cleft Palate Repair • surgeon must also reconstruct the musculature of the velopharyngeal mechanism. • The musculature of the levator palatini is abnormally inserted on the posterior aspect of the hard palate and therefore must be disinserted and reconstructed in the midline.
  • 36. Bardach two-flap palatoplasty • Uses two large full thickness layered dissection and brought to the midline for closure A, Unilateral cleft of the primary and secondary palates, from the anterior vestibule to the uvula. B, This tech.requires two large full-thickness mucoperiosteal flaps to be elevated from each palate shelf. The anterior of the cleft is not reconstructed until the mixed dentition stage. C, layered closure is performed palatoplasty by reapproximating the nasal mucosa. D, Once the nasal mucosa and musculature of the soft palate are approximated, the oral mucosa is closed in the midline
  • 37. Furlow double-opposing Z plasty • Common technique attempts to lengthen the palate by taking advantage of a Z-plasty technique on both nasal mucosa and oral mucosa. A, complete cleft of the secondary palate (both hard and soft). B, Z-plasty technique requires that separate Z-plasty flaps be developed on the oral and then nasal side. C, The flaps are then transposed to theoretically lengthen the soft palate. A nasal side closure is completed in the standard fashion anterior to the junction of the hard and soft palate. D, The oral side flaps are then transposed and closed in a similar fashion completing the palate closure.
  • 38. 4- Bone Grafting • At one time, bone grafting tended to be performed only in bilateral alveolar clefts to fix a mobile premaxilla to the maxilla. however now been extended to include the achievement of a continuous alveolus in all cleft patients with an alveolar defect..
  • 39. References 1. Wray, David, et al. Textbook for General and Oral Surgery. 2. Langman's Medical embryologyM, 2003.. 3. Classification and birth prevalence of orofacial clefts. Tolarova MM, Cervenka. 2, San Francisco : Am J Med Genet, 1998, Vol. 75, pp. 126–37. 1552-4833. 4. Contemporary Oral and Maxillofacial Surgery, 4 ED. 5. Identification of susceptibility loci for nonsyndromic cleft lip with or without cleft palate in a two stage genome scan of affected sib pairs. Prescott NJ, Lees MM,Winter RM,Malcolm S. 3, London : Hum Genet, 2000, European Journal of Human Genetics, Vol. 106, pp. 345-350. 6. PETERSON'S PRINCIPLES OF ORAL AND MAXILLOFACIAL SURGERY Second Edition.