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Cleft lip and palate /certified fixed orthodontic courses by Indian dental academy


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Cleft lip and palate /certified fixed orthodontic courses by Indian dental academy

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  2. 2. INDIAN DENTAL ACADEMY Leader in continuing dental education
  3. 3. INTRODUCTION   Cleft  lip  and  palate  is  one  of  the  most  common  congenital  anomalies afflicting humans. The potential problems that may beset  the  unfortunate  victims  of  the  condition  include  such  severe  handicaps  as  impaired  suckling  and  resultant  failure  to  thrive,  speech impediment, deafness, malocclusion, gross facial deformity,  and severe psychological problems.
  4. 4. The clefting of lip and/or palate occurs at such a strategic  place  in  the  orofacial  region  and  at  such  a  crucial  time  (before  birth)  that  it  becomes  a  complex  congenital  deformity  and  a  major  therapeutic  challenge  incapable  of  resolution  by  any  individual  clinician  and  it  warrants  the  coordinated  skills  of  many modern specialists for its correction.  Plastic  surgeons,  pediatricians,  orthodontists,  prosthodontists,  speech  pathologists,  otolaryngologists,  anesthesiologists,  and  audiologists  form  a  less  than  comprehensive  list  of  those  who  may  be  required  to  provide  a  high  degree  of  interdisciplinary  communication  and  understanding in the treatment of cleft lip and palate deformities.
  5. 5. HISTORY In  ancient  times  many  congenital  deformities  including  cleft  lip and palate, were considered to be an evidence of evil spirit in the  afflicted child. These children were often removed from the tribe or  cultural unit and left to die in the surrounding wilderness. The  repair  of  cleft  lip  is  noted  in  an  ancient  monograph  during  the  Chin  Dynasty  (255-206  BC)  and  is  the  earliest  report of  such surgery any where in the world.  Jehan  Yipperman,  a  Flemish  surgeon  (1295-1351),  LeMonnier, a French dentist (1753) described in detail the repair of  a  cleft  lip  and  palate  patient.  In  the  United  States,  Josiah  Flagg,  surgeon- dentist, advertised in a handbill in Boston in 1786, ……….  that among the many other things he does, he also sews up hare lip.
  6. 6. Stevens  performed  the  first  cleft  palate  repair  in  the  United  States in 1827.  Fauchard,  in  1786,  made  valuable  contributions  and  innovation  in  prosthodontics,  particularly  in  the  area  of  obturators  for cleft lip and palate patients. Kingsley who shares with others the claim to be the Father of  Orthodontia  has  written  over  100  articles  on  cleft  palate  rehabilitation,  the  inadequacies  of  cleft  palate  surgery,  obturators,  orthodontic diagnosis and orthodontic appliances bear his name.  His first book, a treatise on Oral Deformities, as a Branch of  Mechanical Surgery, in 1880, featured the orthodontic appliances of  the  period,  including  jack  screws,  retainers  and  arches  with  ligatures.
  7. 7. Calvin  Case  published  a  Prosthetic  Treatise  on  the  Techniques  and  Principles  of  Dental  Orthopedia  and  Prosthetic  Correction  of  the  Cleft  Palate  in  1921.  The  appliances  described  were all of the fixed type. It  was  only  a few  years  ago  that  orthodontists  demonstrated  that  palatal  surgery  interfered  with  maxillary  growth.  Surgeons  were  asked  not  to  repair  the  palate  until  maxillary  growth  was  complete so as to avoid midfacial deformity.  At  the  same  time  speech  and  language  pathologists  were  requesting for early operation on palate to prevent the development  of compensatory mechanisms of articulations.
  8. 8. Taking  note  of  the  suggestions  made  by  both  orthodontists and speech pathologists the surgeons have modified  their techniques, the timing of surgery, and the number of palatal  procedures that were performed.  Along  with  surgical  refinements,  advances  have  been  made  in  orthodontics  and  other  fields  concerned  with  rehabilitation  of  cleft  lip  and  palate  to  such  an  extent  that,  the  treatment  of  children  with  clefts  have  resulted  in  a  cleft  population  that  is  distinctly  different  from  the  facially  crippled  stereo-types of previous generations.
  9. 9. INCIDENCE OF CLEFT LIP AND PALATE     Ø     In India, Incidence of major malformations in total births  is  estimated to be 14.82 per 1000 births.  Ø     The incidence of Cleft lip with or without cleft palate was  1.25 and that of cleft palate alone was 0.46 per 1000 births.  Ø      In other parts of the world, the incidence of this anomaly is  reported to be the highest in Afghans as 4.9 and lowest in  the Negroid population as 0.4 per 1000 live births.
  10. 10. EMBRYOLOGY OF CLEFT LIP AND PALATE   Ø     Ralph Millard Jr. once said, “Though various theories have  been  put  forth  to  enlighten  the  panorama  of  the  embryological  basis, no theory enjoys universal acceptance as no one has been  an eye witness to the entire 'in utero show' as yet and thus it is  impossible to propose and prove what exactly is happening.”   Ø      Wilhelm  His  popularized  the  theory  that  separate  facial  processes fuse to form the mid portion of the face.  Ø     It was Richard B. Stark of St. Luke Hospital, New York, in  1954, who wrote extensively explaining mesodermal migration.
  11. 11. There are five principal stages in craniofacial development:   Ø     Stage – I Germ layer formation and initial organization  of craniofacial structures. Ø     Stage – II Neural tube formation and initial formation of  the oropharynx. Ø   Stage – II Origins,  migration  and  interactions  of  cell  populations,  especially  neural  crest  cells  and  their  derivatives. Ø    Stage – IV  formation  of  organ  systems,  especially  the  pharyngeal  arches  and  the  primary  and  secondary  palates, and Ø     Stage – V  Final  differentiation  of  tissues  (skeletal,  muscular, and nervous elements).
  12. 12. EMBRYOLOGY OF FACE, NOSE AND PALATE   Ø  After  the  formation  of  the  head  fold,  the  developing  brain  and  the  pericardium  form  two  prominent  bulging  on  the  ventral aspect of the embryo.  Ø   These bulging are separated by the stomatodaeum.  Ø The floor of the stomatodaeum is formed by the           buccopharyngeal membrane, which separates it from the  foregut.  Ø   Soon,  mesoderm  covering  the  developing  forebrain        proliferates,  and  forms  a  downward  projection  frontonasal  process that overlaps the upper part of the stomatodaeum.
  13. 13. Ø      The  pharyngeal  arches  are  laid  down  in  the  lateral  and  ventral walls of the cranial most part of the foregut. These    are  also,  therefore,  in  very  close  relationship  to  the  stomatodaeum.  Ø      It will now be readily appreciated that the face is derived  from  the  following  structures  that  lie  around  the  stomatodaeum: (a)   The frontonasal process and (b)   The first pharyngeal (or mandibular) arch of each side. Fig.1-Head end of the embryo just before formation of the frontonasal process
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  26. 26.        At this stage each mandibular arch forms the lateral wall of th stomatodaeum.        This  arch  gives  off  a  bud  from  its  dorsal  end  called  th maxillary process.        It  grows  ventro-medially  cranial  to  the  main  part  of  the  arc which is now called the mandibular process.
  27. 27.       The  ectoderm  overlaying  the  frontonasal  process  soon  shows  bilateral  localized  thickenings  that  are  situated  a  little  above  the  stomatodaeum  called  the  nasal  placodes.  These  placodes  soon  sink  below the surface to form nasal pits.       The pits are continuous below with the stomatodaeum.       The edges of each pit are raised above the surface; the medial  raised edge is called the medial nasal process and the lateral edge is  called the lateral nasal process.
  28. 28. Development of the face We are now in a position to study the formation of the various parts  of the face.
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  31. 31. Lower lip       The  mandibular  processes  of  the  two  sides  grow  towards  each      other and fuse in the midline.       They now form the lower margin of the stomatodaeum.       The mouth develops from the stomatodaeum       The  fused  mandibular  processes  give  rise  to  the  lower  lip,  and     the lower jaw.
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  35. 35. Upper lip      Each maxillary process now grows medially and fuses, first          with  the  lateral  nasal  process,  and  then  with  the  medial  nasal     process.       The medial and lateral nasal processes also fuse with each        other.        In  this  way  the  nasal  pits  (now  called  anterior  nares)  are  cut      off from the stomatodaeum.      The maxillary processes undergo considerable growth.       The frontonasal process becomes much narrower from side       to side, so that the two anterior nares come closer together.
  36. 36.      The stomatodaeum is now bounded above by the upper  lip which is derived as follows:    (i)    The mesodermal basis of the lateral part of the lip is   formed  the maxillary process.  (ii)    The overlaying skin is derived from ectoderm covering  this  process. (iii)   The  mesodermal  basis  of  the  lip  (called  philtrum)  is  formed   from the frontonasal process.  (iv)  The  ectoderm  of  the  maxillary  process,  however,  overgrows  this  mesoderm  to  meet  that  of  the  opposite  maxillary process in the midline. (v)     As  a  result,  the  skin  of  the  entire  upper  lip  is  innervated by  the maxillary nerves.
  37. 37.        The muscles of the face (including the lips) are derived from  mesoderm  of  the  second  branchial  arch  and  are,  therefore,  supplied by the facial nerve.
  38. 38. Nose       The  anterior  nares  are  formed  when  the  nasal  pits  are  cut  off  from the stomatodaeum by the fusion of the maxillary  process with the medial nasal process.        The anterior nares gradually approach each other.       This is a result of the fact that the frontonasal process  becomes  progressively  narrower  and  its  deeper  part  ultimately forms the nasal septum.       Mesoderm becomes heaped up in the median plane to form  the  prominence  of  the  nose.  Simultaneously,  a  groove  appears  between  the  region  of  the  nose  and  the  bulging  forebrain (which may now be called forehead).      As  the  nose  becomes  prominent  the  anterior  nares  come  to  open  downwards  instead  of  forwards.  The  external  form  of the nose is thus established.
  39. 39. Cheeks    After  formation  of  the  upper  and  lower  lips  the  stomatodaeum (which can now be called the mouth) is very  broad.       In  its  lateral  part,  it  is  bound  above  by  the  maxillary  process  and below by the mandibular process.       These processes undergo progressive fusion with each other    to form the cheeks.        During  the  formation  of  upper  lip  the  maxillary  process  fuses  with the lateral nasal process.
  40. 40. This fusion occurs, not only in the region of the lip, but also  extends from the stomatodaeum to the medial angle of the  developing eye.        For  some  time  this  line  of  fusion  is  marked  by  a  groove  called  the naso-optic furrow or nasolacrimal sulcus.      A strip of ectoderm becomes buried along this furrow and  gives rise to the nasolacrimal duct. 
  41. 41. Development anomalies of the face It has been that the formation of various parts of the face  involves fusion of diverse components. This fusion is occasionally  incomplete and gives rise to various anomalies.
  42. 42. Harelip   The upper lip of the hare normally has a cleft. Hence the  term  hare-lip is used for clefts of the lips.  When one or both maxillary processes do not fuse with the  medial nasal process, this gives rise to defects in the upper lip.  These may vary in degree and may be unilateral or bilateral.   Defective  development  of  the  lowermost  part  of  the  frontonasal  process  may  give  rise  to  a  midline  defect  of  the  upper lip.  When the two mandibular processes do not fuse with each  other the lower lip shows a defect in the midline. The defect  usually extends into the jaw.
  43. 43. Oblique facial cleft       Non-fusion  of  the  maxillary  and  lateral  nasal  processes  gives  rise to  cleft  running  from the medial angle of the eye to  the  mouth.       The nasolacrimal duct is not formed.
  44. 44. INTRODUCTION Cleft  lip  and  palate  is  one  of  the  most  common  congenital  anomalies  afflicting  humans.  The  potential  problems  that  may  beast  the  unfortunate  victims  of  the  condition  include  such  severe  handicaps  as  impaired  suckling  and  resultant  failure  to  thrive,  speech  impediment,  deafness,  malocclusion,  gross  facial  deformity, and severe psychological problems.
  45. 45. The clefting of lip and/or palate occurs at such a strategic place  in the orofacial region and at such a crucial time (before birth)  that  it  becomes  a  complex  congenital  deformity  and  a  major  therapeutic  challenge  incapable  of  resolution  by  any  individual  clinician and it warrants the co-ordinated skills of many modern  specialists  for  its  correction.  Plastic  surgeons,  pediatricians,  orthodontists,  prosthodontists,  speech  pathologists,  otolaryngologists, anesthesiologists, and audiologists form a less  than comprehensive list of those who may be required to provide  a  high  degree  of  interdisciplinary  communication  and  understanding  in  the  treatment  of  cleft  lip  and  palate  deformities.
  46. 46. The  abundance  of  published  material,  however,  indicates  a  remarkable  lack  of  consensus  in  the  treatment  of  these  deformities.  Each  multidisciplinary  team  develops  its  own  protocol  for  management  of  children  with  cleft  lip  and  palate  and  those  ideas  are  held  tenaciously.  What  is  the  effect  of  surgery as a factor in facial growth. As contrasted with the effect  of  the  inherent  growth  disturbance  associated  with  the  deformity? What is the optimal timing for surgical procedures?  By what standards can results be judged? Is there value in using  different  methods  of  investigation?  These  questions  have  been  much discussed over the years and still await resolution.
  47. 47. One  reason  for  lack  of  consensus  is  the  apparent  inability  to  design  and  complete  adequate  prospective  studies  that  produce  unequivocal  results  and  enable  comparison  with  the  work  of  others.  The  reasons  for  this  failure  are  apparent.  Adequate  studies  take  years-  almost  the  professional  lifetime  of  any  one  clinician.  Often  patients  move  and  are  lost  to  follow-up.  Professionals  move,  and  others  take  their  place  and  introduce  changes  in  the  original  protocol.  When  a  research  design  must  be  applied  to  a  complex  biological  problem,  the  number  of  variables  may  take  meaningful  standardization  almost  impossible.
  48. 48. HISTORY In ancient times many congenital deformities including cleft  lip and palate, were considered to be an evidence of evil spirit in  the afflicted child. These children were often removed from the  tribe  or  cultural  unit  and  left  to  die  in  the  surrounding  wilderness.
  49. 49. The repair of cleft lip is noted in an ancient monograph during  the Chin Dynasty (255-206 BC) and is the earliest report of such  surgery  any  where  in  the  world.  Jehan  Yipperman,  a  Flemish  surgeon  (1295-1351),  LeMonnier,  a  French  dentist  (1753)  described in detail the repair of a cleft lip and palate patient. In  the United States, Josiah Flagg, surgeon- dentist, advertised in a  handbill in Boston in 1786, ………. that among the many other  things he does, he also sews up hare lip. The first cleft palate repair in the United States was performed  by  Stevens  in  1827.  Fauchard,  in  1786,  made  valuable  contributions  and  innovation  in  prosthodontics,  particularly  in  the area of obturators for cleft lip and palate patients.
  50. 50. Two  men  stand  out  for  their  early  contribution  to  the  orthodontic  and  prosthodontic  treatment  of  these  deformities.  Kingsley  who  shares  with  others  the  claim  to  be  the  Father  of  Orthodontia  has  written  over  100  articles  on  cleft  palate  rehabilitation,  the  inadequacies  of  cleft  palate  surgery,  obturators,  orthodontic  diagnosis  and  orthodontic  appliances  bear his name. His first book, A treatise on Oral Deformities, as  a  Branch  of  Mechanical  Surgery,  in  1880,  featured  the  orthodontic  appliances  of  the  period,  including  jack  screws,  retainers  and  arches  with  ligatures.  Calvin  Case  published  A  Prosthetic  Treatise  on  the  Techniques  and  Principles  of  Dental  Orthopedia  and  Prosthetic  Correction  of  the  Cleft  Palate  in  1921. The appliances described were all of the fixed type.
  51. 51. It  was  only  a  few  years  ago  that  orthodontists  demonstrated  that palatal surgery interfered with maxillary growth. Surgeons  were  asked  not  to  repair  the  palate  until  maxillary growth  was  complete  so  as  to  avoid  midfacial  deformity.  At  the  same  time  speech  and  language  pathologists  were  requesting  for  early  operation on palate to prevent the development of compensatory  mechanisms of articulations.
  52. 52. TTaking note of the suggestions made by both orthodontists and  speech pathologists the surgeons have modified their techniques,  the timing of surgery, and the number of palatal procedures that  were performed. Along with surgical refinements, advances have  been  made  in  orthodontics  and  other  fields  concerned  with  rehabilitation  of  cleft  lip  and  palate  to  such  an  extent  that,  the  treatment  of  children  with  clefts  have  resulted  in  a  cleft  population  that  is  distinctly  different  from  the  facially  crippled  stereo-types of previous generations.
  53. 53. INCIDENCE OF CLEFT LIP AND PALATE Incidence of major malformations in total births at 26 large hospitals from big cities and towns from different parts of the country is estimated to be 14.82 per 1000 births (2695 total malformations from amongst 1,81,798 births). Of these malformations, the incidence of CL+P was 1.25 and that of cleft palate alone was 0.46 per 1000 births. In other parts of the world, the incidence of this anomaly is reported to be the highest in Afghans as 4.9 and lowest in the Negroid population as 0.4 per 1000 live births.
  54. 54. EMBRYOLOGY OF CLEFT LIP AND PALATE Ralph Millard Jr. once said Though various theories have been put forth to enlighten the panorama of the embryological basis, no theory enjoys universal acceptance as no one has been an eye witness to the entire 'in utero show' as yet and thus it is impossible to propose and prove what exactly is happening.
  55. 55. The theory that separate facial processes fuse to form the mid portion of the face was popularized by Wilthelm His. It was Richard B Stark of St. Luke Hospital, New York, in 1954, who wrote extensively explaining mesodermal migration.
  56. 56. There are five principal stages in craniofacial development:Stage – I Germ layer formation and initial organization of craniofacial structures. Stage – II Neural tube formation and initial formation of the oropharynx.
  57. 57. Stage – III Origins, migration and interactions of cell populations, especially neural crest cells and their derivatives. Stage – IV Formation of organ systems, especially the pharyngeal arches and the primary and secondary palates, and Stage – V Final differentiation of tissues (skeletal, muscular, and nervous elements).
  58. 58. EMBRYOLOGY OF FACE, NOSE AND PALATE After the formation of the head fold, the developing brain and the pericardium form two prominent bulgings on the ventral aspect of the embryo. These bulgings are separated by the stometodaeum. The floor of the stomatodaeum is formed by the buccopharyngeal membrane, which separates it from the foregut. Soon, mesoderm covering the developing forebrain proliferates, and forms a downward projection that overlaps the upper part of the stomatodaeum.
  59. 59. This downward projection is called the frontonasal process. The pharyngeal arches are laid down in the lateral and ventral walls of the cranialmost part of the foregut. These are also, therefore, in very close relationship to the stomatodaeum. It will now be readily appreciated that the face is derived from the following structures that lie around the stomatodaeum:  the frontonasal process and  the first pharyngeal (or mandibular) arch of each side.
  60. 60. At this stage each mandibular arch forms the lateral wall of the stomatodaeum. This arch gives off a bud from its dorsal end. This bud is called the maxillary process. It grows ventromedially cranial to the main part of the arch which is now called the mandibular process.
  61. 61. The ectoderm overlaying the frontonasal process soon shows bilateral localized thickenings, that are situated a little above the stomatadaeum. These are called the nasal placodes. These placodes soon sink below the surface to form nasal pits. The pits are continuous below with the stomatodaeum. The edges of each pit are raised above the surface; the medial raised edge is called the medial nasal process and the lateral edge is called the lateral nasal process. We are now in a position to study the formation of the various parts of the face.
  62. 62. Lower lip The mandibular processes of the two sides grow towards each other and fuse in the midline. They now form the lower margin of the stomatodaeum. If it is remembered that the mouth develops from the stomatodaeum. It will be readily understood that the fused mandibular processes give rise to the lower lip, and the lower jaw.
  63. 63. Upper lip  Each maxillary process now grows medially and fuses, first with the lateral nasal process, and then with the medial nasal process. The medial and lateral nasal processes also fuse with each other. In this way the nasal pits (now called anterior nares) are cut off from the stomatodaeum.  The maxillary processes undergo considerable growth. At the same time the frontonasal process becomes much narrower from side to side, so that the two anterior nares come closer together.
  64. 64.  The stomayodaeum is now bounded above by the upper lip which is derived as follows: ♦ The mesodermal basis of the lateral part of the lip is formed the maxillary process. The overlaying skin is derived from ectoderm covering this process. ♦ The mesodermal basis of the lip (called philtrum) is formed from the frontonasal process. The ectoderm of the maxillary process, however, overgrows this mesoderm to meet that of the opposite maxillary process in the midline. As a result, the skin of the
  65. 65. TThe muscles of the face (including the lips) are derived from mesoderm of the second branchial arch and are, therefore, supplied by the facial nerve.
  66. 66. Nose We have seen that the anterior nares are formed when the nasal pits are cut off from the stomatodaeum by the fusion of the maxillary process with the medial nasal process. We have also noted that the anterior nares gradually approach each other. This is a result of the fact that the frontonasal process becomes progressively narrower and its deeper part ultimately forms the nasal septum. Mesoderm becomes heaped up in the median plane to form the prominence of the nose. Simultaneously, a groove appears between the region of the nose and the bulging forebrain (which may now be called forehead). As the nose becomes prominent the anterior nares come to open downwards instead of forwards. The external form of the nose is thus established.
  67. 67. Cheeks After formation of the upper and lower lips the stomatodaeum (which can now be called the mouth) is very broad. In its lateral part, it is bound above by the maxillary process and below by the mandibular process. These processes undergo progressive fusion with each other to form the cheeks.
  68. 68. We have already seen that (during the formation of upper lip) the maxillary process fuses with the lateral nasal process. This fusion occurs, not only in the region of the lip, but also extends from the stomatodaeum to the medial angle of the developing eye. For some time this line of fusion is marked by a groove called the naso-optic furrow or nasolacrimal sulcus. A strip of ectoderm becomes buried along this furrow and gives rise to the nasolacrimal duct.
  69. 69. Development anomalies of the face It has been that the formation of various parts of the face involves fusion of diverse components. This fusion is occasionally incomplete and gives rise to various anomalies.
  70. 70. Hare-lip: The upper lip of the hare normally has a cleft. Hence the term hare-lip is used for clefts of the lips. ♦ When one or both maxillary processes do not fuse with the medial nasal process, this gives rise to defects in the upper lip. These may vary in degree and may be unilateral or bilateral. ♦ Detective development of the lowermost part of the frontonasal process may give rise to a midline defect of the upper lip. ♦ When the two mandibular processes do not fuse with each other the lower lip shows a defect in the midline. The defect usually extends into the jaw.
  71. 71.  Oblique facial cleft: Non-fusion of the maxillary and lateral nasal processes gives rise to cleft running from the medial angle of the eye to the mouth. The nasolacrimal duct is not formed.
  72. 72. DEVELOPMENT OF NASAL CAVITIES The nasal cavities are formed by extension of the nasal pits. We have seen that these pits are at first in open communication with the stomatodaeum. Soon the medial and lateral nasal processes fuse, and from a partition between the pit and the stomatodaeum. This is called the primitive palate, and is derived from the frontonasal process.
  73. 73. The nasal pits now deepen to from the nasal sacs which expand both dorsally and caudally. The dorsal part of the sac is, at first, separated from the stomatodaeum by a thin membrane called the bucconasal membrane (or nasal fin). This soon breaks down. The nasal sac now has a ventral orifice that opens on the face (anterior nares), and a dorsal orifice that opens into the stomatodaeum (primitive posterior nares).
  74. 74. The two nasal sacs are at first widely separated form one another by the frontonasal process. We have seen, however, that the frontonasal process becomes progressively narrower. This narrowing of the frontonasal process, and the enlargement of the nasal cavities themselves, brings them closer together. The intervening tissue becomes much thinned to from the nasal septum. The ventral part of the nasal septum is attached below to the primitive palate. More posteriorly, the septum is at first attached to the bucconasal membrane, but on disappearance of this membrane it has a free lower edge. The nasal cavities are separated from the mouth by the development of the palate, as described below.
  75. 75. The lateral wall of the nose is derived on each side from the lateral nasal process. The nasal conchae appear as elevations on the lateral wall of each nasal cavity. The original olfactory placodes form the olfactory epithelium that lies in the roof and adjoining parts of the walls, of the nasal cavity.
  76. 76. Development of the palate To understand the development of the palate let us have another look at the maxillary processes. From ------- it will be seen that these processes not only from the upper lip but also extend backwards on either side of the stomatodaeum. They can, therefore, be diagrammatically illustrated as in fig…. If we cut a coronal section through the region (along the line X-Y) the maxillary processes will be seen as in……… Finally, if we now correlate …. with …… , the relationship of the maxillary processes to, the developing nasal cavity and mouth is easily understood.
  77. 77. From each maxillary process a palate like shelf grows medially. This is called the palatal process. We now have three components from which the palate will be formed. These are: (a) the two palatal processes and (b) the primitive palate formed from the frontonasal process. The definitive palate is formed by the fusion of these three parts as follows: (i) Each palatal process fuses with the posterior margin of the primitive palate. (ii) The two palatal processes fuse with each other in midline. Their fusion begins anteriorly and proceeds backwards. (iii) The medial edges of the palatal processes fuse with the free lower edge of the nasal septum, thus separating the two nasal cavities from each other, and from the mouth.
  78. 78. At a later stage the mesoderm in the palate undergoes intramembranous ossification to form the hard palate. However, ossification does not extend into the posterior-most portion, which remains as the soft palate. The part of the palate derived from the frontonasal process the premaxilla, which carries the incisor teeth.
  79. 79. Cleft palate Defective fusion of the various components of the palate gives rise to clefts in the palate. These vary considerably in degree as illustrated. Clefts on the palate that extend to its anterior end are associated with cleft-lip, as both the upper lip and the palate are formed by fusion of the maxillary processes with the frontonasal process. Clefts of the palate result in communications between the mouth and the nose. These may be unilateral, or bilateral.
  80. 80. ETIOLOGICAL FACTORS IN CLEFT LIP AND PALATE Following are the probable etiological factors causing cleft lip and palate:        Monogenic or single gene disorder Polygenic or multifactorial inheritance Teratogenic Chromosomal abnormalities Familial Heterogeneity Sex predominance
  81. 81. Monogenic or Single Gene Disorders Approximately half of the recognized syndromes associated with cleft lip and palate are due to single gene disorders with equal distribution autosomal dominant and autosomal recessive. Single gene defects may give rise to Mendelian patterns of inheritance, either of isolated cleft lip (palate) or in multiple malformations associated with cleft lip with or without cleft palate.
  82. 82. Teratogenic Drugs Anticonvulsants (a) Diphenylhydantoin: CL (CP) is part of the fetal hydantoin syndrome related to the drug and its analogues. The association between diazepam and cleft lip (palate) has also been reported. Material use of alcohol is potentially teratogenic. Rubella in the first few weeks of gestation is associated with clefting.
  83. 83. Chromosomal Abnormalities Chromosomal abnormalities account for 18% of the clefting syndromes and would invariably be associated with other malformations. Chromosomal abnormalities notably trisomy D and also less frequently trisomy E, may cause multiple malformations including CL (P). There are multiple deletions and translocations where individual case reports included clefting. Clefting syndromes are three times more frequent in Down's syndrome.
  84. 84. Familial Familial aggregation has been observed in CL (P). Thus it is inferred that someone with a relative affected by one of these disorders is more likely to be affected than if that person had no affected relatives. The relatives of the index patient shares genes in common in direct proportion to the closeness of their relationship. According to the multifactorial model, the relatives will share some of the disease predisposing genes and hence will be shifted towards the threshold for disease.
  85. 85. Analysis of family studies indicate degrees of susceptibility. Fogh-Anderson's family studies shows that siblings of patients with cleft lip had increased frequency of cleft lip and cleft palate, but no increased frequency of cleft palate alone. Siblings of patients with cleft palate had increased frequency of cleft palate, but not CL and CP. A cleft uvula, submucous cleft of the palate, and velopharyngeal insufficiency (microforms of clefts) when noted in a parent, is a guideline in discerning autosomal dominant varieties of genetically heterogenous clefting syndromes.
  86. 86. Of first degree relatives, the incidence in the sibling of index cases of CL (P) is approximately 40 times the incidence in the general population. The incidence in 2nd degree relatives is approximately seven times and that of the 3rd degree relatives is three times that of general.
  87. 87. Heterogeneity Genetic heterogeneity means what appears first to be one disease turns out to be many diseases with different genetic and non-genetic etiologics. Thus differing genotypes and/or environmental causes may produce a single or similar phenotypes.
  88. 88. Sex Predominance More males are born with cleft lip and cleft palate than females and more females than males have cleft palate alone.
  89. 89. CLASSIFICATION Any disease or anomaly has to be defined and classified for the sake of understanding and treating the anomaly. Classification also as a language for communicating with other professionals colleagues. Symbolic representations of cleft lip and palate anomalies serve the clinician as a convenient short hand method for recording conditions. Surgeons, in particular, find it appealing to see at a visual summary of anatomic malformations, particularly congenital anomalies.
  90. 90. Numerous classifications have been proposed by various authors. Each has its own advantages and disadvantages. Only those classification systems which have found wide clinical acceptance will be described. They are:      Davis and Ritchie classification (1922) Veau's classification (1931) Keranhan's striped 'Y' classification (1971) Spina's classification (1974) Millard's classification (1977).
  91. 91. Davis & Ritchie Classification (1922) They classified the congenital clefts into three groups according to the position of the cleft in relation to the alveolar process. Group – I Prealveolar clefts: ♦ Unilateral ♦ Median ♦ Bilateral
  92. 92. Group – II Postalveolar clefts: ♦ Soft palate only ♦ Soft & hard palate ♦ Submucous cleft Group – III Alveolar clefts: ♦ Unilateral ♦ Bilateral ♦ Median.
  93. 93. Veau's Classification (1931) He divided clefts into four groups: Group – I Cleft of soft palate only. Group – II Cleft of the hard and soft palate extending no further than the incisive foramen, thus involving the secondary palate alone.
  94. 94. Group – III Complete unilateral cleft, extending from the uvula to incisive foramen in the midline, then deviating to one side and usually extending through the alveolus at the position of the future lateral incisor tooth.
  95. 95. Group – IV Complete bilateral cleft, resembling group –III, with two clefts extending forward from the incisive foramen through the alveolus. When both clefts involve the alveolus, the small anterior element of the palate commonly referred to as the premaxilla, remains suspended from the nasal septum.
  96. 96. Kernahan's Striped 'Y' Classification (1971) In this classification incisive foramen is the reference point and it is based on embryology rather than morphology. The two arms of the stripes Y Logo are each divided into three sections, representing the lip, the alveolus, and the hard palate as far back as the incisive foramen. The stem of Y is also divided into three parts, representing varying degrees of clefting of the hard and soft palates. Over clefting is represented by stippling and submucous clefting by cross-hatching.
  97. 97. The advantage of the diagrammatic representation of clefting is that it gives as immediate and readily recognized account of the original extent of the cleft at any time in the long progress of the patient's treatment.
  98. 98. Spina's Classification (1974) He divided clefts into four groups: Group – I Preincisive foramen clefts (clefts lying anterior to the incisive foramen). Clefts of the lip with or without an alveolar cleft.
  99. 99.  Unilateral: ♦ Right or left: Total when they reach the alveolar arcade or partial.  Bilateral: ♦ Total: (On one or both sides). ♦ Partial  Median: ♦ Total ♦ Partial
  100. 100. Group – II Transincisive foramen clefts. (clefts of the lip, alveolus and palate). Unilateral :  Bilateral Right or Left.
  101. 101. Group – III Postinisive foramen clefts  Total  Partial Group – IV Rare facial clefts.
  102. 102. Millard's Classification (1977) Millard's symbolic representation is basically a modification of Kernahan's striped 'Y' classification. He has added inverted triangles atop the upright triangular segment which in turn stand on the 'Y' proposed by Kernahan. The inverted triangles represent the nasal arch and the upright triangles represent the nasal floor.
  103. 103. Leader in continuing dental education