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cleft lip and palate part 1
- 3. CONTENTS
- 5. Fissure or elongated opening, especially one occuring in the embryo or derieved from failure of parts to fuse during embryonic development
- 8. Hippocrates (400 BC) and Galen (150 AD) mention cleft lip, but not cleft palateHippocrates (400 BC) and Galen (150 AD) mention cleft lip, but not cleft palate in their writingsin their writings First documented cleft lip repair – 390 ADFirst documented cleft lip repair – 390 AD Harelip has been reported back to 1000ADHarelip has been reported back to 1000AD Jehan Yperman first described unilateral and bilateral cleft lip repairJehan Yperman first described unilateral and bilateral cleft lip repair Cleft palate was not recognized as a congenital disorder until 1556, by Fanco.Cleft palate was not recognized as a congenital disorder until 1556, by Fanco. The first successful closure of a soft palate defect was reported in 1764 byThe first successful closure of a soft palate defect was reported in 1764 by LeMonnier, a French dentist.LeMonnier, a French dentist. Dieffenbach (1826) : separation of soft tss of palate from underlying bone.Dieffenbach (1826) : separation of soft tss of palate from underlying bone. History ….History ….
- 9. Fergusson 1844 & von lagenback 1862 : laid emphasis on creatingFergusson 1844 & von lagenback 1862 : laid emphasis on creating mucoperiosteal flapsmucoperiosteal flaps Passavant : considered surgical methods to assist speechPassavant : considered surgical methods to assist speech 1943: American cleft palate association formed.1943: American cleft palate association formed. 1950 – concept of bone grafting by Schmid1950 – concept of bone grafting by Schmid 1960 : Blakely fitted palatal obturator to improve consonant articulation1960 : Blakely fitted palatal obturator to improve consonant articulation 1963 : Fenton Braithwaite gave possible causes of CLP , action of1963 : Fenton Braithwaite gave possible causes of CLP , action of levators & palatopharyngeuslevators & palatopharyngeus 1970s – Bell pioneered maxillary osteotomies1970s – Bell pioneered maxillary osteotomies
- 12. 5 wk 6 wk
- 13. Medial nasal prominence Lateral nasal prominence Nasolacrimal groove stomodeum Maxillary prominence
- 14. 7 wk
- 15. 10 wk
- 16. Nasal pit Medial nasal prominence Mandibular prominence Maxillary prominence Lateral nasal prominence eye
- 24. Higher incidence of cleft palate in females may be due to lateral Palatine process fusing about a week later – prolongs sensitive period
- 27. Native Americans : 3.74 per 1000 live births Europeans :1:600 to 1:700 live births. (Highest )Asians : (0.82-4.04 per 1000 live births), (Intermediate) Caucasians : (0.9-2.69 per 1000 live births) (Low)Africans : (0.18-1.67 per 1000 live births) Isolated clefts : 25% of all clefts Combined cl/p : 45% Boys > girls Unilateral > bilateral : 4:1 Left > right Syndromic clefts : 50% of total
- 28. IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) Volume 13, Issue 5 Ver. II. (May. 2014), PP 78-81 Sah & powar et al , 55.7% : males 44.3% : females. Age : 1 day to 71 years( median age of 2 years). Cleft Lip : 21.7%, Cleft Lip-Palate : 61.1% Cleft Palate : 16% and rare clefts : 1.1% Family history : 1.3% cases. Consanguineous marriage was noted in 48.9% parents. 54.6% : income< Rs 5000 per month. 77% cases were first and second born child. Prevalence of CL±P was significantly higher in children of consanguineous parents (p<0.0001).
- 29. Indian J Plast Surg. 2010 Jul-Dec; 43(2): 184–189. Reddy et al 65% : males. 33% : CL, 64%:CLP, 2% :CP 1% : rare craniofacial clefts. Unilateral cleft lips : 79% of the patients.( 64% were left sided.) significant correlation of children with clefts being born to parents who shared a consanguineous relationship and those who were illiterate with the odds ratio between 5.25 and 7.21 for consanguinity and between 1.55 and 5.85 for illiteracy, respectively. urban Semi-urban rural 2001
- 31. MENDELIAN ▫ Van der Woude syndrome ▫ Stickler syndrome ▫ Oral-facial- digital syndrome ▫ Treacher Collins syndrome ▫ Ectodactyly- ectodermal dysplasia- clefting syndrome ▫ Gorlin syndrome ▫ Smith-Lemli- Opitz syndrome Non syndromic causes • Over 90% of isolated cleft palate cases are nonsyndromic• Localization of contributory genetic sequences is ongoing program, ▫ Transforming growth factor - Alpha (TGF-A) ▫ MSX -1 gene. ▫ Barx – 1 gene ▫ Several other genes involvement is also suspected like • MSX2, RARA, TGFA, TGFB3, TGFB2, MTHFR, GABRB3, FOXE1, GLI2, JAG2, LHX8, PHF8, SATB2, SKI, SPRY2, AND TBX10 TERATOGENS VIRAL INFECTIONS German measles ▫Toxoplasmosis ▫cytomegalo inclusion diseases Prescription drugs Anticonvulsants Thalidomide ▫Alkylating agent •Chlorambucil •Selenium •Salicylic acid •boric acid •Recreational drugs ▫Alcohol, ▫Cigarette smoke •Toxins ▫Dioxin, organic solvents, plant toxins, food contaminants ▫Nutritional deficiencies •Vitamin A •Folic acid •Riboflavin •Magnesium •Pantothetic acid Endocrine abnormalities •Steriod hormones •Sex steriod •Maternal diabetes •Maternal adrenalectomy •Thyriod •Maternal thyroidectomy Propylthiouracil Iodine compounds Intrauterine factors Hypoxia Radiation Foetal constraint Amniotc bands Maternal factors
- 34. Recurrence rate of cleft conditions
- 35. Nyberg et al : ultrasound classification Type 1: isolated cleft lip without palate Type 2: unilateral cleft lip and palate Type3 : bilateral cleft lip and palate Type 4 : clefts associated with amniotic bands or limb- body-wall complex
- 38. Davis & Ritchie's classification (1922)Davis & Ritchie's classification (1922) Group 1 : Pre Alveolar CleftsGroup 1 : Pre Alveolar Clefts lip cleftslip clefts unilateral, median and bilateralunilateral, median and bilateral Group 2 : Post Alveolar CleftsGroup 2 : Post Alveolar Clefts Degree of involvement of the soft and hard palate - specified , UpDegree of involvement of the soft and hard palate - specified , Up to the alveolar ridgeto the alveolar ridge Group 3 :Alveolar CleftGroup 3 :Alveolar Cleft Complete clefts of palate, alveolar ridge & lip with subdivisionComplete clefts of palate, alveolar ridge & lip with subdivision unilateral, median & bilateralunilateral, median & bilateral CLASSIFICATIONSCLASSIFICATIONS
- 39. Grp 1: soft palate Grp 2: Hard & Soft palate to the incisive foramen Grp 3: complete unilateral cleft of the soft hard palate & lip & alv ridge on one side Grp4 : complete bilateral cleft of the soft & hard palate & lip & alveolar ridge on both side VEAU CLASSIFICATION (1931)VEAU CLASSIFICATION (1931)
- 40. Group1- clefts of lip – unilateralGroup1- clefts of lip – unilateral - bilateral- bilateral Group 2- clefts of lip & palate - unilateralGroup 2- clefts of lip & palate - unilateral - bilateral- bilateral Group 3- clefts of palate extending up to incisive foramenGroup 3- clefts of palate extending up to incisive foramen Fogh –Anderson classificationFogh –Anderson classification (1942)(1942)
- 41. American Cleft Plate AssociationClassification (1962) :
- 42. Pfiefer classification (1964)Pfiefer classification (1964):: GRAPHICAL METHODS OF RECORDING CLEFTSGRAPHICAL METHODS OF RECORDING CLEFTS
- 43. Kernahan’s stripped ‘Y’ classification :Kernahan’s stripped ‘Y’ classification : (1971)(1971)
- 44. MODIFIED STRIPED-Y ELSAHY CLASSIFICATION: (1973)MODIFIED STRIPED-Y ELSAHY CLASSIFICATION: (1973) nostrils
- 46. Millard’s modification of the ‘striped Y’ classification (1977)
- 47. LAHSHAL classification: (Okriens 1987)
- 48. Clefts – 0-14Clefts – 0-14 Orbit - landmarkOrbit - landmark Clefts above the upper eyelid –Clefts above the upper eyelid – cranial clefts.cranial clefts. Clefts below the lower eyelid –Clefts below the lower eyelid – facial clefts.facial clefts. Combination of clefts –Combination of clefts – craniofacial clefts.craniofacial clefts. Tessier system ofTessier system of classificationclassification
- 52. monozygous twins: 35% concordance ratemonozygous twins: 35% concordance rate dizygous twins < 5% concordancedizygous twins < 5% concordance Reasons for the failure to resolve the question of a hereditary basis forReasons for the failure to resolve the question of a hereditary basis for cleftsclefts:: (1) nongenetic origin(1) nongenetic origin (2) individuals who have increased genetic liability for having a child(2) individuals who have increased genetic liability for having a child with CLP often fail to be recognized(nonpenetrance for genes that controlwith CLP often fail to be recognized(nonpenetrance for genes that control CLP)CLP) (3) complex of diseases with different etiologies lumped together(3) complex of diseases with different etiologies lumped together because of clinical disease resemblance (they all show clefting).because of clinical disease resemblance (they all show clefting). CPO : 1 per 1500 to 2000 births in Caucasians males : CL(P)CPO : 1 per 1500 to 2000 births in Caucasians males : CL(P) CL(P) : 1 or 2 per 1000 births.CL(P) : 1 or 2 per 1000 births. females: CPO.females: CPO. GENETICS OF CLEFT LIP ANDGENETICS OF CLEFT LIP AND PALATEPALATE
- 53. Approximately 30% of CL(P) and 50% of CPO patients have one of theApproximately 30% of CL(P) and 50% of CPO patients have one of the more than 400 syndromes.more than 400 syndromes. CL(P) and CPO are heterogeneous diseases ( there are multiple causes forCL(P) and CPO are heterogeneous diseases ( there are multiple causes for the single phenotypes).the single phenotypes). Accepted hereditary basis for CL(P) and CPO can be summarized asAccepted hereditary basis for CL(P) and CPO can be summarized as follows: Single, nonsyndromic cases of CL(P) and CP,follows: Single, nonsyndromic cases of CL(P) and CP, sporadic clefts (result of a complex interaction between multiple geneticsporadic clefts (result of a complex interaction between multiple genetic and environmental factors)and environmental factors) Familial : true genetic cases(action of single major gene)Familial : true genetic cases(action of single major gene) Environmental (dietary) : maternal intake of folateEnvironmental (dietary) : maternal intake of folate multifactorial
- 54. T-box transcription factor-22 (TBX22), poliovirus receptor- like-1 (PVRL1) interferon regulatory factor-6 (IRF6) 7.16 times 1.5- 4.7
- 55. SYNDROMES Van der woude Treacher collins Cleidocranial dysplasia Ectodermal dysplasia Sticklers Robert Bowen – Armstrong Appelt Christian Cerebro-costo-mandibular Micrognathic dwarfism Meckels Autosomal dominant Autosomal recessive
- 57. CLEFTS OF LIP
- 63. I I
- 65. VARIATIONS IN ISOLATED CLEFT PALATEVARIATIONS IN ISOLATED CLEFT PALATE
- 67. SPERBER Oblique facial cleft Midline mandibular cleft Median cleft lip
- 68. CLEFT LIP ANATOMY UNILATERAL BILATERAL
- 70. 11
- 73. CLINICAL FEATURES OF UNILATERAL CLEFT LIP Asymmetric structure V shaped defects White roll disappears Absence of philtrum dimple Orbicularis buldge Lateral side of cleft repositioned relative to medial side Overall tissue deficiency Vertical height of lip diminished on cleft side Medial cleft margin shorter Deflection of nasal tip to non cleft side Larger nares
- 76. CLINICAL FEATURES OF UNILATERAL CLEFT PALATE • Malalignment of central structures of nose • Palatal shelf on cleft side is smaller in width & length than noncleft shelf & retroplaced • Posterior palatal arch wider than normal • Greater palatine artery and its grooves located more laterally • Soft palate: overall muscle deficiency • Hypoplastic tensor veli paltini & levator muscles • Both intrinsic & extrinsic muscles are hypoplastic : short palate
- 77. CLINICAL FEATURES OF BILATERAL CLEFT LIP & PALATE
- 79. DENTAL AND OCCLUSAL MANIFESTATIONS Congenitally missing teeth Supernumeraray teeth Fused, irregularly shaped Malpositioned teeth Delayed eruption of teeth
- 82. Middle ear diseases Pterygoid hamulus Tensor veli palatini Middle ear
- 83. FEEDERS
- 84. Mead – Johnson cleft palate nurser: Soft squeezable Short nipple Practice squeezing Haberman feeder: For premature infants with only cleft palate One-way valve – keeps milk in nipple Milk expressed – when placed against palate Sucking not required Less tiring for mother Oral Maxillofacial Surg Clin N Am 28 (2016) 153–159
- 85. Eur Arch Paediatr Dent (2014) 15:1–9Eur Arch Paediatr Dent (2014) 15:1–9
- 86. Craniofacial Development - SperberCraniofacial Development - Sperber Langman’s Medical Embryology – 11Langman’s Medical Embryology – 11thth EditionEdition Cleft Lip and Palate, Diagnosis and Management – Samuel BerkowitzCleft Lip and Palate, Diagnosis and Management – Samuel Berkowitz 2nd edition2nd edition Oral anatomy, histology and embryology – Berkovitz, Holland, Moxham,Oral anatomy, histology and embryology – Berkovitz, Holland, Moxham, 3rd Etd3rd Etd Oral & maxillofacial surgery – PetersonOral & maxillofacial surgery – Peterson Dentistry - infancy through adolesence - Mc DonaldDentistry - infancy through adolesence - Mc Donald Textbook of Oral Pathology – Shafers 7th EtdTextbook of Oral Pathology – Shafers 7th Etd REFERENCES
Editor's Notes
- Having a child born with a cleft can seem like a major crisis..&quot; Most parents go through pregnancy looking forward to the delivery of the &quot;perfect child.Giving birth to a child with cleft lip and palate (CLP) can be emotionally traumatic for parents. The facial appearance awakens feelings and reactions in the family. When this is not the case, it can at first feel like a major loss, bringing with it many emotions: fear, guilt, happiness, sadness, anxiety, uncertainty and hope. d other people. After the initial shock, the parents
- Cleft – split / fissure A cleft lip is a sep[aration in the upper lip.A cleft palate is an opening in the roof of the mouth In craniofacial region – cleft is associated with various regions of face involving soft as well as hard tissues. A cleft is a separation of the parts of the lip or palate (roof of the mouth), which usually fuse together during the first three months of development. If the parts, which make up the lip and palate fail to meet and fuse, there will be a space between the parts, which is called a cleft
- For centuries, perforations of the palate were considered to be secondary to syphilis,
- Schematic drawing of the intermaxillary segment and maxillary processes. B, The intermaxillary segment gives rise to the philtrum of the upper lip; the median part of the maxillary bone and its four incisor teeth; and the triangular primary palate.
- From innr aspect of max processes on each side r already developing a pair of shelves which are destined to fuse in midline to complete palate. Becoz this part of palate forms after ant part it is known as sec palate. At first tongue which is also developing at this time, lies btwn 2 palatal shelves which hang vertically down on either side as neck begins to extend during 8th week tongue moves dwnwrds & palatal shelves spring upwarsds above it to horizontal level where they can grow towards each other.
- By this time shelves have made contact with each other and with primary palate anteriorly and free margins fuse together. Incisive foramen comes to lie at jxn of pri and sec palate in midline. Recent research suggest that edges of advancing palatal shelves become sticky when they come in contact with opposite shelf.epithelial layersadhere to one another. There is some evidnce that sp may not fuse . Bt develop by inflow of mesoderm from hardpalate and merging of two halves, as in face. In weeks aftr fusion, mesoderm in ant half of sec palate develops centers of ossification and gives rise to hard palate, while posteriorly it differentiates into muscles of soft palate, forming sling across midline. Cleft of sec palat may occur if fusion doesn’t take place. This may be due to failure of descent of tongue, keeping two palatal shelves apart. May be due to failure of mesodermal migration into palatal shelves so they fail to reach midline, resulting in wide cleft with tissue deficiency or due to delay in migration.. Abnormal differentiation of bone centers or outgrowths can cause secondary defects.these defects show imperfect dev of soft tss, conn tss or those of ectodermal origin. Primary or true clefts result from fusion defects btw swellings….sec defects r reslt of differentiation defects in bone centers in combo with ectodermal defects.
- READ FROM LD
- In India alone the number of infants born every year with CLP is 28,600, which means 78 affected infants are born every day, or 3 infants with clefts born every hour.
- Cleft Lip and/or Palate (CL±P) is the most common congenital malformation of the face and its pattern varies with geography worldwide. Pattern and magnitude remains uncertain due to very few studies. This study has been conducted to study the epidemiological profile of CL±P patients attending a Tertiary Care Hospital & Medical Research Centre, Karnataka, India. This study includes 2453 CL±P Patients registered from 2007-2012 in the Department of Plastic and Reconstructive Surgery of this Hospital & Medical Research Centre. Data were collected from January to September 2013. Variables like age, sex, socioeconomic status, cleft pattern, birth order and consanguinity were noted. Associations of clefts with different variables were studied using Chi-square test. Among all cleft cases, 55.7% were males and 44.3% were females. Age of patients at presentation ranged from 1 day to 71 years with a median age of 2 years. Cleft Lip, Cleft Lip-Palate, Cleft Palate and rare clefts were noted in 21.7%, 61.1%, 16% and 1.1% cases, respectively. Family history was present in 1.3% cases. Consanguineous marriage was noted in 48.9% parents. 54.6% had income of less than Rs 5000 per month. 77% cases were first and second born child. Prevalence of CL±P was significantly higher in children of consanguineous parents (p&lt;0.0001). Key Words: Cleft Lip, Cleft Lip-Palate, Cleft Palate, 1) In this study patients came from local area (i.e. Belgaum) Karnataka and also 2014 km farther from the service centre (i.e. KLE’s Dr.PKH & MRC, Belgaum). Most of the cases 2053 (83.7%) had come from Karnataka followed by Maharashtra 320 (13%), Goa 67(2.7%), Andhra Pradesh 9 (0.37 %,), Bihar 3(0.1%), West Bengal 1(0.04%). 2) Among all cases, 1500 (61.1%) had CLP, 532 (21.7%) had CL, and 393 (16%) had CP and only few 28 (1.1%) had rare clefts. 1367 (55.7%) were males and 1086 (44.3%) were females with the sex ratio of 1.26:1.Out of 1376 male cases, CLP was more common contributing 842 (61.6%) followed by CL 305 (22.31%), CP 208 (15.22%) and rare clefts 12(0.87%). In 1086 females cases, CLP was more frequent contributing 658 (60.6%) followed by CL 227 (20.9%), CP 185 (17%) and rare clefts 16 (1.5%).The sex ratio in CL, CLP, CP and rare cleft were 1.34:1,1.28:1,1.12:1 and 0.75:1 respectively. In both sex, CLP was more prevalent followed by CL, CP and rare clefts. There was no significant association between sex and cleft deformities were observed. ( 3) Of the 2453 cases, parents of 1199 (48.9%) cases had consanguineous marriage, whereas parents of 1254 (51.1%) cases did not have consanguineous marriage. In non consanguineous marriage, CLP was more frequent representing 739 (58.9%) followed by CP 294 (23.4%), CL 208 (16.6%) and rare clefts 13 (1%), whereas in consanguineous marriage, CLP was more common contributing 761 (63.5%) followed by CL 324 (27%), CP 99 (8.3%) and rare clefts 15 (1.3%). In both children of consanguineous as well as non consanguineous parents, CLP was more prevalent, whereas CL was more prevalent among children of consanguineous parents as compared to CP and rare clefts but CP was more frequent among children of non consanguineous parents as compared to CL and rare clefts. The significant association was observed between consanguineous marriage and cleft deformities. 1)
- The study was conducted in 2001 in the state of Andhra Pradesh, India. The state has a population of 76 million. Three districts, Cuddapah, Medak and Krishna, were identified for this study owing to their diversity. They were urban, semi-urban and rural, respectively. Results: The birth rate of clefts was found to be 1.09 for every 1000 live births. This study found that 65% of the children born with clefts were males. The distribution of the type of cleft showed 33% had CL, 64% had CLP, 2% had CP and 1% had rare craniofacial clefts. Unilateral cleft lips were found in 79% of the patients. Of the unilateral cleft lips 64% were left sided. There was a significant correlation of children with clefts being born to parents who shared a consanguineous relationship and those who were illiterate with the odds ratio between 5.25 and 7.21 for consanguinity and between 1.55 and 5.85 for illiteracy, respectively. Conclusion: The birth rate of clefts was found to be comparable with other Asian studies, but lower than found in other studies in Caucasian populations and higher than in African populations.
- Vit A :↓in folic acid supplem ↑ neural tube def. Mills et al – prev of mutation in methylene tetrahydrofoliate reductase & is 3 times &gt; ICP , CLP Impairment of foliate metabolism - orofacial clefting Riboflavin - organogenesis. Cortisone - inhibition of palatal elevation. 1. inhibits synthesis of sulfomucopolysac 2. ↓ amniotic fluid 3. ↑ water retention 4. ↓ inhibit RNA synthesis
- Recently, CDC reported on important findings from research studies about some factors that increase the chance of having a baby with an orofacial cleft: Smoking―Women who smoke during pregnancy are more likely to have a baby with an orofacial cleft than women who do not smoke.2-3 Diabetes―Women with diabetes diagnosed before pregnancy have an increased risk of having a child with a cleft lip with or without cleft palate, compared to women who did not have diabetes.5 Use of certain medicines―Women who used certain medicines to treat epilepsy, such as topiramate or valproic acid, during the first trimester (the first 3 months) of pregnancy have an increased risk of having a baby with cleft lip with or without cleft palate, compared to women who didn’t take these medicines.
- Prenatal diagnosis – targetted level II ultrasound designed to check baby’s anatomy therefr bst for diagnosis Amniocentesis and chorionic villus sampling
- Prenatal diagnosis of orofacial cleft gives the parents the possibility to prepare themselves in an emotional and practical way [20]. A frank discussion of the cost of care and strategies for obtaining coverage for care can also relieve unspoken anxiety [4]. Knowing the diagnosis before delivery also allows the cleft team to discuss the plans, for example, feeding issues, type and geographical location of the cleft [2]. This would make delivery much less traumatic, especially for the parents and the extended family Advanced imaging modalities – 3D ultrasonography and MRI 3D ultrasonography and prenatal MRI would improve the accuracy of prenatal diagnosis of orofacial clefts. 3D ultrasonography can provide more precise image of the defect and it has shown to enhance 2D examination significantly [5, 15–17]. While 3D ultrasonography can achieve a reliable diagnosis of fetal CL ± CP, this does not rule out cases of CP only [5]. Magnetic resonance imaging (MRI) has been useful for prenatal diagnosis of fetal deformities compared with US, adding valuable information or supplying higher diagnostic accuracy [12]. The use of MRI is increasing for evaluation of fetal abnormalities that are difficult to identify on ultrasonography alone
- Bony str is formed by max bones in ant 3 quarters and by palatine bones posteriorly.. Most ant part of maxilla is derieved from premaxilla and incisive bone. In mid anteriorly , behind alv ridge , is incisive frmn.. Post & laterally in palatine bone is gp frmn.. Further back pterygoid hamulus is located. Most post end of HP is extended in midline and this process is called PNS..
- Drawback: he ignored clefts involving lip & alveolus, or alveolus alone. Clefts of HP & SP are included seperately though embryologiclly they are single structure..
- CLASSIFICATION BASED ON EMBRYOLOGY
- Studies of the CLP phenotype in twins indicate that monozygous twins have a 35% concordance rate, whereas dizygous twins show less than 5% concordance.133 Information from two sources (families and twins) establishes a genetic basis for CLP, but despite many extensive investigations, no simple pattern of inheritance has been demonstrated. This has led to proposals for a variety of genetic modes of inheritance for CLP, including dominance, recessiveness, and gender linkage, and has led ultimately to the documentation of modifying conditions that may be present, such as incomplete penetrance and variable gene expressivity.134 There are three important reasons for the failure to resolve the question of a hereditary basis for clefts: (1) some clefts are of a nongenetic origin and should not be included in a genetic analysis (such cases are seldom recognized and are difficult to prove); (2) individuals who have increased genetic liability for having a child with CLP often fail to be recognized, but because they do not have CLP themselves, they cannot be identified with certainty (this latter situation defines the problem of nonpenetrance for genes that control CLP)107; and (3) CLP, although sometimes appearing to be relatively simple in origin, is undoubtedly a complex of diseases with different etiologies lumped together because of clinical disease resemblance (they all show clefting). There are two clearly recognized groups of etiologically different clefts: cleft lip either with or without cleft palate, CL(P), and isolated cleft palate (cleft palate only, CPO). These two entities, CL(P) and CPO, can occur as both single and multiple cases in a family. In the former they are called sporadic, and in the latter they are called familial or multiplex. Some researchers refer to multiplex cases as those individuals with findings in addition to an oral cleft, even if a specific syndrome is not recognized. It should also be noted that CPO that occurs without a cleft of the lip is different from the palatal cleft that occurs as a part of CL(P). The embryology and developmentaltiming are different, and CPO is more commonly part of a syndrome than is CL(P). CPO is less common, with a prevalence of approximately 1 per 1500 to 2000 births in Caucasians, whereas CL(P) is more common, with 1 or 2 per 1000 births. The prevalence of CPO does not vary in different racial backgrounds, but the prevalence of CL(P) varies considerably, with Asians and Native Americans having the highest rate and Africans the lowest. There are also gender ratio differences, with more males having CL(P) and more females having CPO. Except in a small number of syndromes such as Van der Woude syndrome, families with one type of clefting segregating in the family do not have the other cleft type occurring at a rate higher than the population prevalence. When all potential study groups for CL(P) and CPO are considered, the minimum number is six: three subgroups for CL(P) and three for CPO. These three for each type of cleft are the sporadic and the familial groups, and a group of syndromes that feature CL(P) and/or CPO. Approximately 30% of CL(P) and 50% of CPO patients have one of the more than 400 syndromes described.135 As noted earlier, it is probable that minor and subtle facial changes are more likely to produce the best-correlated phenotype needed to pinpoint the cleft genotype. Part of the reason for this view is the suspicion that certain facial shapes are more predisposed to developing CL(P) than others,136,137 and that subepithelial defects of the upper lip musculature are part of the phenotypic spectrum of oral clefts and may represent an occult, subclinical manifestation of the anomaly.138 Although this approach seems best for producing an accurately generated clefting phenotype, further study of the developmental anatomy of the head and face is needed. The published data on nonsyndromic cleft populations come from around the world (Japan, China, Hawaii, Denmark, Sweden, the United Kingdom, and North America). These studies make it clear that both CL(P) and CPO are heterogeneous diseases; that is, there are multiple causes for the single phenotypes. The generally accepted hereditary basis for CL(P) and CPO can be summarized as follows: Single, nonsyndromic cases of CL(P) and CP, or sporadic clefts, are believed to be the result of a complex interaction between multiple genetic and environmental factors. Hence, their etiology is multifactorial in the true sense of the word, and the chance that these multiple factors would interact to produce a cleft phenotype in relatives is small, probably less than 1%. The other nonsyndromic group consists of multiple cases of clefts that occur in a single family. These are called familial (or multiplex) and have been viewed by researchers as the “true” genetic cases. Familial occurrences of CL(P) and CPO seem most likely to be attributed to the action of a single major gene, but the influence of multifactorial (complex) trait factors is difficult to rule out. Thus we are left with the idea that both multifactorial and single major gene elements may have a role in producing sporadic and familial cases of CL(P) and CPO. (For an overview of genetic and other factors in orofacial clefting, the reader is referred to the paper by Leslie. An example of an environmental (dietary) factor associated with a decrease in neural tube defects such as spina bifida, as well as orofacial clefting, is the maternal intake of folate (folic acid), now a common component in prenatal vitamins. To be effective, such vitamins or other dietary supplements must be used at least around the time of conception because of the embryologic timing of neural tube closure, and lip and palate formation. Because of the public health importance and critical need before a woman may realize that she is pregnant, folic acid fortification of grains in the United States became mandatory January 1, 1998, specifically to reduce the occurrence of neural tube defects, which has been successful. This has also, to a lesser degree, reduced the occurrence of orofacial clefting. Interestingly, however, it did not decrease the occurrence of orofacial clefting in children whose mothers smoke cigarettes.140 Although some genetic and environmental risk factors for CL(P) have been identified, many nonsyndromic clefts are not linked to any of these factors. Furthermore, there is a paucity of information available on the longterm consequences for children born with CL(P) or CPO. To address these concerns, the National Center on Birth Defects and Developmental Disabilities at the Centers for Disease Control and Prevention conducted a workshop entitled “Prioritizing a Research Agenda for Orofacial Clefts.” Experts in the fields of epidemiology, public health, genetics, psychology, speech pathology, dentistry, health economics, and others participated in this workshop to review the state of knowledge on orofacial clefts, identify knowledge gaps that need additional public health research, and create a prioritized public health research agenda based on these gaps. Their report is recommended to the reader as an excellent summary of the current knowledge and future research priorities for orofacial clefting.
- van Rooij et al.[49] found that a maternal glutathione s-transferase (GSTT1) genotype, when combined with smoking, could significantly increase the risk of CLP (odds ratio=4.9). Beaty et al.[50] reported that maternal smoking and infant MSX1 genotypes acted together to increase the risk for CLP by 7.16 times. maternal drinking increased the risk for CLP by 1.5–4.7 times in a dose-dependent manner. The results were supported by Shaw and Lammer[52] who showed that mothers who consumed more than five drinks per occasion had 3.4 times increased risk of delivering an infant with CLP. Use of folic acid and multivitamins Shaw et al.[54] reported that if vitamin supplements namely folic acid and cobalamins were not taken during early pregnancy the risk for CLP could be tripled. Folic acid deficiency with a pre-existing TGFA TaqI C2 genotype was also found to increase the risk of CLP. very high dose of supplementary folic acid (10 mg/day) could reduce the risk. Three of them—T-box transcription factor-22 (TBX22), poliovirus receptor-like-1 (PVRL1), and interferon regulatory factor-6 (IRF6)—are responsible for causing X-linked cleft palate, cleft lip/palate–ectodermal dysplasia syndrome, and Van der Woude..
- The figure above shows the middle portion of the lip in blue and the side portion of the lip in green. The two side portions of the lip merge with the middle part of the lip by the 10th week of pregnancy. If the middle and one of the side portions of the lip don&apos;t merge it causes a unilateral cleft lip.
- a Complete unilateral cleft lip and palate. The distorted nostril is caused by the aberrant lip muscle forces. b Complete bilateral cleft lip and palate with a widely separated lateral palatal segment. The protruding premaxilla extends forward of the lateral palatal segments and is attached to the vomer. The prolabium (central portion of the lip) overlies the premaxilla
- Incomplete clefts of the lip. a Unilateral b Bilateral(LAST PIC)
- Fig. 6.1. The balance between facial and tongue muscle forces. a The outer muscles, the orbicularis oris, buccinator, and superior constrictor muscles, form a ring which acts to compress the palatal and mandibular arches with the teeth. b The tongue force acts to expand the dental arches and teeth.Whether the teeth and arches align is determined by the resultant of these Forces. Fig. 6.2 a–c. Effects of complete clefts of the lip and palate at birth. In complete cleft lip and palate, with a separation in the orbicularis oris (a) -buccinator (b) -superior constrictor (c) muscle ring, the aberrant muscle forces plus the plunger action of the tongue causes the palatal segments to be pulled and pushed apart. a In bilateral clefts of the lip and palate, the premaxilla may be laterally or ventrally flexed with the fulcrum at the premaxillary vomerine suture. b Complete unilateral clefts of the lip and palate at birth. The cleft’s lesser segment and the premaxillary portion of the larger segment (d) are pulled outward. c Cleft of the lip and alveolus. The bony distortion is determined by the extent of alveolar involvement. (Courtesy of Action of Intact Facial Muscular Forces an the Maxilary Arch In a normal jaw with intact lips and palate, the muscles of the lip, cheek, and pharynx exert their normal sphincter-like actions against the developing maxillary and mandibular arches. The compressive external muscular forces neutralize the expansion forces of the tongue (Fig. 6.1). The neonatal arch form changes as these forces change with growth and maturation; yet the opposing muscles always maintain a precise and dynamic balance with each other.When this muscular balance is upset, the arch form and teeth relatnshp changes. Aberrant Muscle Forces in Clefts of the Lip and Palate A cleft of the lip and palate is the result of the failure of lip elements, and right and left palatal segments, to come together within the first 9 weeks of fetal life. The loss of muscular continuity of the orbicularis orisbuccinator- superior constrictor ring in complete unilateral and bilateral clefts changes the normal muscular force diagram. The aberrant muscular forces act to displace tissue masses. In complete clefts of the lip and palate, if the lateral palatal cleft segments are detached from the vomer, they will be pulled laterally by the external aberrant lip-cheek muscular forces, as well as spread apart by the tongue pushing into the cleft space (Fig. 6.2).Because clefts differ in their location and extent, lip and palate clefts can vary
- Variations in unilateral clefts of the lip and palate at birth. The palatal segments may be complete (a, c, e and f) or incomplete (b and d); the cleft segment may be almost of the same length or shorter. The cleft space may be relatively narrow (b and d) or wide (a, c, e and f).
- Variations in bilateral cleft lip and palate. The size of the premaxilla varies with the number of teeth it contains. Classification is dependent on the completeness of clefting of the lip and alveolus and whether there is a cleft of the hard and soft palate.Yet one or both sides of the hard palate may or may not be attached to the vomer. If it is attached to the vomer, it is classified as being incomplete. Even in complete clefts of the lip and alveolus the extent of premaxillary protrussion will vary. a Incomplete bilateral cleft lip and palate.Complete cleft lip and palate – left side. Incomplete cleft lip and palate – right side. b Complete bilateral cleft lip and palate.Complete cleft palate – both sides. c Incomplete bilateral cleft lip and palate. Incomplete palatal clefts – both sides. d Complete bilateral cleft lip and palate. Incomplete right and complete left palate. e Incomplete bilateral cleft lip and palate. Incomplete left palate and complete right palate. f Complete bilateral cleft of the lip and palate. incomplete right and left palatal segments. g Complete bilateral cleft of lip and palate. Incomplete left palate and complete right palatal segment. h Complete bilateral cleft lip and palate. incomplete left palate and complete right palate. i Incomplete bilateral cleft lip and alveolus.Normal palate
- Fig. 6.10 a–h. Variations in isolated cleft palate. The length and width of the cleft space is highly variable. The cleft extends anteriorly to various distances but not beyond the incisal canal
- Submucous cleft palate. This cleft is characterized by a bifid uvula, lack of muscle continuity across the soft palate, and a pink zone of mucosa (zona pellucida) across the cleft in the hard palate.A palpable notch in the posterior border of the hard palate is always indicative of the presence of a cleft
- OBLIQUE facial :persistance of groove btwn max prominence and lnp running from medial canthus of eye to ala of nose Midline mand cleft : pesistance of furrow btwn two mand prominences Median cleft lip : true hairlip due to incmplete fusion of 2 merging mnp…deep fissure, groove,,,mental retardation..holoprosencephaly…
- 1FIG) A baby’s upper lip, nose and roof of the mouth (palate) have completed formation by only 10 weeks into the pregnancy. The philtrum is the normal feature found in the middle portion of the upper lip seen below. The lip forms from three parts merging during pregnancy– the center part and the left and right side parts. The center and side parts of the upper lip meet at the philtral columns. Normally the side portions of the lip meet and fuse with the center portion of the lip. The places where the center and side portions of the lip merge become the philtral columns (in blue above). These are the raised ridges extending the vertical length of the lip from the mouth up to the nose. The Cupid’s bow is the normal shape of the lip where the red and white portions of the lip meet (in red above). The philtral columns meet the red portion of the lip at the peaks of Cupid’s bow, as illustrated in the figure above. FIG 2) During formation of the lip, if one of the side portions of the lip does not reach and connect with the center part of the lip, a unilateral cleft of the lip occurs. The X in the image on the left shows where the lip failed to merge resulting in the unilateral cleft lip. The image on the right is of an infant with a complete unilateral cleft lip and palate. The image above shows a baby a left unilateral cleft lip and palate on the right side of the figure. The philtral column and the peak of the Cupid’s bow are absent on the baby’s left side (the side of the cleft) because the cleft occurs through these structures. Note that in cleft lip and palate the cleft involves the floor of the nostril and gums. If both the left and right side fail to meet the center part of the lip a bilateral cleft lip forms as seen below. FIG 3) In bilateral cleft lip, both the left and right philtral columns and the peaks of the Cupid’s bow are absent. The X in the image on the left shows where the lip failed to merge resulting in the bilateral cleft lip. The image on the right is of an infant with a complete bilateral cleft lip and palate. In complete cleft lip and palate the cleft extends up through the entire height of the lip and through the floor (bottom) of the nostril. This can be seen in both the unilateral and bilateral cleft lip images above. The cleft continues back through the bone in the upper jaw including the gums and the entire length of the palate. While the features of the cleft lip alone are striking, the involvement of the underlying facial bones including the gums and palate creates significant changes in the facial skeleton that adds to the uneven appearance of the face.
- The orbicularis oris muscle is the primary muscle of the lip and can be divided functionally and anatomically into 2 parts (see the image below).[4] The deep component, in concert with other oropharyngeal muscles, works in swallowing and serves as a sphincter. The superficial component is a muscle of facial expression and inserts into the anterior nasal spine, sill, alar base, and skin to form the philtral ridges. Muscular defects in unilateral deformity. In a complete cleft lip (CL), the deep fibers of the orbicularis oris muscle are interrupted by the cleft and end on either side of the defect instead of making their way around the mouth. In addition, the superficial component of the orbicularis oris turns upward, along the margins of the cleft and ends beneath the ala or columella.[4] Incomplete cleft lip behaves in a similar manner, except when the cleft is less than two thirds of the height of the lip.[4] In this case, the fibers of the muscle run along the margins of the cleft, then change direction and run horizontally over the top of the cleft. These muscle fibers are interspersed with connective tissue. The blood vessels parallel the course of the muscle fibers and run along the margins of the cleft toward the columella or alar base, where they form anastomoses with nearby vessels. In the bilateral deformity, the anatomic characteristics are determined by the degree of completeness of the cleft and its symmetry. The cleft may involve the primary palate alone or in conjunction with the secondary palate. Although the prolabium varies in size, it is usually retracted and lacks muscle fibers. In addition, the columella is absent and the prolabium appears attached to the top of the nose in some cases. The size and position of the premaxilla vary and effectively can be excluded with a collapse of the alveolar arch.
- 1) This figure shows the range in severity of unilateral cleft lip. Note that involvement of the gums or palate results in a much wider gap in the lip and more severe changes on the shape of the nose. In the least severe form of unilateral cleft lip, the cleft involves only a minor irregularity of the lip. This can range a ridge or intact skin irregularity along the philtral column or a notch in or just above the red portion of the lip. This is called a microform cleft lip (seen on the left side of the figure above). An incomplete cleft lip involves a separation of the lip through all three layers of the lip - the skin, muscle and pink intraoral part (mucosa). This cleft extends up to but does not involve the floor of the nostril. Complete cleft lip involves an extension of the cleft through the entire height of the lip and floor of the nostril. This cleft usually extends through the gums (alveolus) and the palate, but occasionally involves only the gums with an intact palate. Patients with complete cleft lip and palate have the most severe changes in the appearance of the lip and nose because the separation in the bones of the gums and palate creates a wide gap that separates the lip segments and drastically changes the shape of the nose. 2) The degree to which the nose is deformed by the cleft of the lip relates to the severity of the cleft of the lip. For the nose, the involvement of the gums and palate has the most dramatic effect on the nasal shape. The impact of the cleft involving the floor of the nostril and the underlying facial skeleton is visible with these views of the nose. The changes in the nasal shape of the infants with clefts of the alveolus (gums) alone and combined alveolus and palate are significantly more severe than those of the incomplete clefts.
- Note that while the nose has a fairly normal appearance in the bilateral incomplete cleft lip the central lip is very short. This central lip segment does not have the normal lip muscle in it. This leads to less bulk and a shorter lip because the muscle has not been stretching this part of the lip out. Note how the lateral lip segments are much taller than the central segment. The images on the left side of the figure above shows the significant shortening that occurs of the central portion of the lip (prolabium) that occurs with bilateral cleft lip. Note that the intact nasal floor preserves the nasal shape. The most defining feature of the bilateral cleft nasal deformity is the severe shortening of the columella which is the skin and cartilage between the nostrils. In the figure above the images on the central and right side show the severe change in the nasal shape that occurs with complete bilateral cleft lip. It is extremely challenging to lengthen the columella without nasoalveolar molding (NAM) or lip adhesion.
- The figure above shows the normal nasal tip which is symmetric and round. The ala (outer part of the nostril) is also rounded and symmetric. The nasal floor is skin at the bottom of the nostril where the lip and nostril meet. The columella is the skin and cartilage that separates the two nostrils and supports the nasal tip. The ala (outer part of the nostril) is also rounded and symmetric. In complete cleft lip and palate the cleft extends up through the entire height of the lip and through the nasal floor (bottom of the nostril). The cleft continues back through the bone in the upper jaw including the gums and the entire length of the palate. While the features of the cleft lip alone are striking, the involvement of the underlying bone including the gums and palate creates significant changes in the facial skeleton that adds to the asymmetry of the face. These facial bones are the foundation that the nose sits on. Clefts involving the gums (alveolus) and palate create a large step off in the bony foundation causing deformity of the nose. In the figure above the location of the location where unilateral cleft lip and palate occurs is marked in blue. Complete unilateral cleft lip and palate causes a separation in the lip gums and palate. The wide separation of the gums and the significant affect on the nasal shape can be appreciate in the photo on the right. As you can see in the figure above, the degree to which the nose is deformed by the cleft of the lip relates to the severity of the cleft of the lip. For the nose, the involvement of the gums and palate has the most dramatic effect on the nasal shape. This is because the cartilage and skin of the nostril (ala) is shaped like an upside down U with the ends of the U sitting on the facial bones on each side of the cleft. The farther away the ends of the cartilage are from one another, the more severe the deformity of the nostril will be. In the figure above the location of the location where unilateral cleft lip and palate occurs is marked in blue. Complete unilateral cleft lip and palate causes a separation in the lip gums and palate. The wide separation of the gums and the significant affect on the nasal shape can be appreciate in the photo on the right. As you can see in the figure above, the degree to which the nose is deformed by the cleft of the lip relates to the severity of the cleft of the lip. For the nose, the involvement of the gums and palate has the most dramatic effect on the nasal shape. This is because the cartilage and skin of the nostril (ala) is shaped like an upside down U with the ends of the U sitting on the facial bones on each side of the cleft. The farther away the ends of the cartilage are from one another, the more severe the deformity of the nostril will be.
- FROM LD. Deflection of the nasal tip towards the noncleft side Retroplacement of the cleft alar cartilage dome Obtuse angle between the medial and lateral crura of the lower lateral cartilage on the cleft side Buckling of the ala on the cleft side Absence of the alar-facial groove on the cleft side and attachment of the ala to the face at an obtuse angle Apparent or real bony deficiency of the maxilla on the cleft side Larger nares on the cleft side Shorter columella on the cleft side, positioning the entire columella at a slant toward the noncleft side Inferior displacement of the medial crus within the columella Dislocation of the caudal portion of the septum to the noncleft side from the nasal spine Downward rotation of the alar cartilage on the cleft side Bilateral deformity in which the nasal tip appears large, flat, and bifid, because both alae are rotated downward and spread apart
- The incisive foramen is the key landmark in the bony palate (see the image below). The premaxilla lies anterior to the incisive foramen and includes the 2 premaxillary bones: the alveolus and the incisors. The soft-tissue structures in the primary palate include the nasal tip and the upper central lip. The size, composition, and configuration of the premaxilla can vary from full development with the complement of teeth (4 primary and 4 secondary) to underdevelopment with only 2 incisors. If the premaxilla is unrestrained in the intrauterine and neonatal period it can protrude from the arch; the maxillary arches may then collapse and potentially exclude the premaxilla from the arch. Posterior to the incisive foramen lies the secondary palate, comprising the hard palate and soft palate. The hard palate forms from the palatine processes of the maxilla anteriorly and the palatine bones posteriorly. Posterior to the bony hard palate lies the soft palate. The soft palate plays an important role in speech and swallowing. Paired muscle on both sides of the midline (see the following image) form the musculature of the soft palate. The levator veli palatini is the most important muscle for the production of speech and velopharyngeal competence. The paired muscles of the soft palate function as a sling from their origin at the undersurface of the temporal bone to their aponeurosis across the midline, as they elevate the soft palate toward the posterior pharyngeal wall. The palatopharyngeus further supplements the posterior movement of the soft palate. Contraction of the superior pharyngeal constrictor contributes to closure of the velopharyngeal opening at the lateral and posterior pharyngeal wall. The primary function of the tensor veli palatini is to dilate the eustachian tube and to maintain its integrity. The uvular muscle is thought to have a minimal contribution to normal speech. Muscles of the soft palate. Clefts of the palate (CPs) are associated with bony, as well as soft-tissue, abnormalities. Clefts of the secondary palate may be isolated or associated with clefts of the primary palate. Although clefts of the secondary palate are midline defects (see the image below), those involving the primary palate are usually asymmetric, with the vomer attached to the noncleft side. The dental arch on the noncleft side usually splays outward due to the lack of restraining force from the lip, and the palate is foreshortened in the anteroposterior direction. In the case of complete bilateral clefts, the entire premaxilla protrudes from the adjacent alveolar ridges. Because of the collapse of the palatine shelves posterior to the premaxilla and its possible rotation, the premaxilla is prevented from rejoining the arch and is left attached solely to the vomer. ariations of cleft palate. Soft-tissue defects of the cleft palate include hypoplasia of the velar musculature in addition to anomalous insertions of its muscular components (see the following image). The normal midline insertion and transverse orientation of the levator palatini is substituted by an aberrant longitudinal orientation and insertion along the bony cleft margin and posterior palatine bones. Other palatal muscles are affected similarly. Dysfunction results in speech pathology with velopharyngeal incompetence and in eustachian-tube obstruction with resultant middle-ear effusion, infections, and possible hearing loss.
- FROM LD.
- From ld.
- The term, ‘cleft palate speech’ is used to describe phenomena such as atypical consonant productions, abnormal nasal resonance, abnormal nasal airflow, altered laryngeal voice quality, and nasal or facial grimaces. children with cleft lip and palate show delayed expressive language, evidenced by slower acquisition of sounds and words and restricted inventory of sounds in early infancy. Errors in speech sound production In individuals with cleft lip and palate, errors in speech production are noticed due to the abnormalities in oronasal structure / function, orofacial structure and growth, learned neuromotor patterns during early infancy, and / or disturbed psychosocial development. Abnormal nasal resonance is another characteristic feature in most individuals with cleft lip and palate. The resonance of speech is largely determined by the size and shape of the oral, nasal, and pharyngeal cavities, and the functioning of the velopharyngeal valve. The abnormal nasal resonance in cleft lip and palate could involve either hypernasality or hyponasality. Hypernasality refers to an excessive nasal resonance that is perceived for vowels and oral consonants. Hyponasality indicates a decreased nasal resonance for nasal consonants and vowels. In some individuals with cleft, hypernasality and hyponasality co-exist, resulting in a mixed nasality. Abnormal resonance can be caused by structural disturbances such as obstructions in the nasopharynx due to adenoid hypertrophy, swelling of the nasal passages secondary to allergic rhinitis, or hypertrophic tonsils (causing hyponasality), large oronasal fistula, and velopharyngeal dysfunction (causing hypernasality). VELOPHARYNGEAL VALVING The velopharyngeal valve is a tridimensional muscular valve that is located between the oral and nasal cavities. It consists of the lateral and posterior pharyngeal walls as well as the soft palate. The role of the velopharyngeal valve is to separate the oral and nasal cavities during speech and swallowing. During speech production, the air is directed through the mouth for oral sounds and through the nose for nasal sounds. VPD is the inability to separate the oral and nasal cavities adequately during speech production through the actions of the velum and pharynx. Effects of VPD on speech In VPD, the incompletely closed velopharyngeal valve causes an inability to effectively manage the air stream for continuous speech. This could be manifested as one or more of the following: Hypernasality Audible / visible nasal air emission during production of oral consonants like /p/b/t/d/ Facial / Nasal grimaces Weak or omitted consonants Reduced mean length of utterance Compensatory articulation errors.
- Patients with these deformities often may have associated problems including otologic disease, speech and language problems (delayed onset, articulation disorders, velopharyngeal incompetence or insufficiency), dental deformities (e.g., malocclusion; missing, malformed, or supernumerary teeth), facial growth deficiencies, and psychosocial issues. Some children have associated genetic syndromes or chromosomal abnormalities.
- Hearing and the Cleft Palate Child: The ear is composed of three parts; the outer ear, middle ear and inner ear. Soundwaves travel through the air and enter the outer ear canal. Here they strike the eardrum, the eardrum moves inwards and the sound is transmitted across the middle ear by a chain of tiny bones, three in number, which are termed ossicles. The innermost of the three ossicles is called the stirrup bone and it transmits the sound to the inner ear. Here the minute nerve endings pick up the sound and transmit it directly to the brain. The part of the ear which is usually affected in a child with a cleft palate is the middle ear. If sound cannot travel freely across the middle ear to the inner ear, the patient has a hearing loss. Normally the inner ear contains the air. It is connected to the back of the nose via the eustachian tube. This tube opens and closes approximately 1,000 time a day and permits air to enter the middle ear from the nose, thus keeping the air in the middle ear the same as the pressure in the outside world. If, for any reason, the eustachian tube does not function and air does not enter the middle ear regularly, a negative pressure develops in the middle ear, fluid is secreted into the middle ear by its lining membrane and this fluid interferes with the movement of the ossicular chain. Thus, sound cannot be transmitted efficiently across the middle ear and the patient develops what is called conductive hearing loss.. when there is a cleft in the palate, hearing problems can arise for the following reason: The soft palate at the back of the mouth has muscles connected to the eustachian tube. When we yawn, eat or swallow, the palate moves and this pulls the eustachian tube open and allows air to pass into the middle ear as mentioned above. In a child with a cleft palate, however, just as there is an abnormality of the muscles in the palate, so also is there an abnormality of these muscles where they are attached to the eustachian tube as both form a part of the same muscle group. Thus, if the eustachian tube does not work properly, air cannot get into the middle ear as it should and its place is taken by fluid. The fluid in the middle ear has the effect of dampening down the conduction of sound and producing a hearing defect. The hearing loss is frequently to the order of 3040 decibels. It is not known, without a national register of cleft palate patients, how many children may be affected in this way but it could be as high as 90%. It is therefore important that children with cleft lip and palate should have a full hearing assessment carried out at approximately 12 months of age and that their hearing should be assessed on a regular basis throughout their childhood. Eustachiantube has 3 fxn : a) ventilates middle ear space b) barrier to nasopharyngeal secretions c) drains secretions from middle ear space It is channel that extnds from middle ear cavity to nasopharynx..has carti n osseous portions.. Muscles having influence on ET are : levator veli palatini and tensor veli palatini In cleft pts, TVP muscle has reduced no. of fibers inserting into aponeurosis of SP and bulk of muscle deviates to PNS and post portion of palatine bone.. This anatomic derangemnt of TVP reduces ability to adequately open ET.. Midlle ear ds range from simple ET catarrah to choelsteatotoma.. In cleft pts, absorbed air in mid ear isnt replaced and there is constant –ve pressure in middle ear which produces middle ear effusion due to metaplasia of mucous lining ofmid ear and there is mucoid collection.. Aspiration of nasopharyngeal secretions into middle ear pressure and tubal dilatation leads to acute otitis media…which when becomes chronic with prforation of tymp memeb leads to choleosteatoma.
- Feeding Plates: If the baby has a wide cleft of the gum and front part of the palate it may be considered necessary to have a Feeding Plate made if a feeding pattern cannot be established. This is an orthodontic appliance which fits into the front of the mouth covering the cleft and aids more normal feeding. This is not routinely used due to the difficulty of fitting the plate and the uncertain longterm advantage. We do not routinely use feeding plates in The Temple Street Unit. Weaning: Your baby should wean in the normal manner. Babies can be introduced to pureed food at around four months in the normal manner and mashed foods can be introduced between 6 and 9 months. Your baby should manage to feed easier using the spoon and semi-solids, as the semi-solids, unlike fluids, do not pass up into the nasal cavity to the same extent. You should finish the feed with a drink of water to help clear any food from the cleft area.