Cleft Lip and Palate Ch 22

8/12/2019 Cleft Lip and Palate Ch 22

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Cleft Lip and Palate

Introduction

Cleft lip and palate are the most commonly foundfacial congenital anomaly, it constitute 80 percent of

orofacial cleft. Cleft of the lip and palate may occur inisolation or involve both lip and palate together. Theincidence of cleft lip among Caucasians is higher thanthe Africans, Europeans and Japanese. Incidenceamong the Asians varies from 1 in 400 to 500 life-birthsand 1 in 1500 to 2000 among the Americans. However,incidence of isolated cleft palate is similar among theraces (0.50 in 1000 life-births). The left-sided cleft liphas higher incidence than the right. But, the etiologyis unknown. The cleft lip and palate have a higherincidence among close relatives (Figs 22.1 to 22.11).

Classification

There are different methods of classification of cleftlip and palate. Cleft lip may be complete or incompleteand may be associated with cleft of the alveolus andpalate (figure). The cleft of the lip anterior to incisiveforamen is called the cleft of the primary palate. Thisprimary cleft palate may be unilateral or bilateral andmay be complete or incomplete (figure). The cleftpasses entirely between the lateral incisor and canineteeth and passes in a V-shaped manner. Midline cleftlip is a rare occurrence. The cleft of the palate posteriorto incisive foramen is called secondary palate. The cleftof the secondary palate may involve both hard andsoft palate. However, classification based on Kernahanand Stark is simple and generally acceptable.

Kernahan and Starks Classification

1. Cleft of Primary PalateUnilateralComplete

Incomplete

BilateralCompleteIncomplete

MidlineComplete

Incomplete2. Cleft of Secondary PalateCompleteIncompleteSubmucus

3. Cleft of both Primary and Secondary PalateUnilateralComplete

IncompleteBilateralComplete

IncompleteMidlineComplete

Incomplete

Embryology of Cleft Lip and Palate

The face is developed from five processes as a resultof migration and proliferation of neural crestmescenchyme. The frontonasal process, a pair ofmaxillary process and a pair of mandibular archescoalesce together around the stomodeum near the fifthweek of embryo to form the face. The formation ofolfactory pits divides the frontonasal process intomedian nasal process and lateral nasal process. Thelateral nasal process forms the alae of the nose. Mediannasal process extends caudally and forms bilateral

elevations called globular process. The fusion ofglobular process and maxillary process gives rise tothe formation of upper lip. The globular process fusein the midline to form philtrum. The triangular shapedarea in front of incisive foramen and between the fourincisor teeth constitute the premaxilla (primarypalate). The failure of fusion of the globular processwith the maxillary process or mesenchymal dehiscencegives rise to different types of cleft lip.

Introduction Classification Embryology Etiology Anatomy ClinicalPresentation Management Neonatal Care Surgical Timing PreoperativePreparation Anesthesia Surgery Secondary Surgery Palatal Revision

TWENTY-TWO

This chapter written by Prof D Basak, MS and Mch.


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Cleft Lip and Palate153

Fig. 22.1: Incomplete cleft lip

(unilateral)

Fig. 22.2:Complete cleft lip (unilateral) Fig. 22.3: Bilateral cleft lip and

alveolus

Fig. 22.4: Incomplete cleft palate Fig. 22.5: Incomplete cleft palate Fig. 22.6: Complete cleft palate

and alveolus

Fig. 22.7: Complete cleft palate Fig. 22.8: Bilateral complete cleft palate with

premaxilla and nasal septum

Fig. 22.9A: Millard repair of cleft lip

showing the incision line

Fig. 22.9B: Repair completed with

rotation of flap

Fig. 22.10A: VY repair of cleft

palate-showing the incision line

Fig. 22.10B: Repair of the palate

in two layers with posterior

reposition of flap


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Synopsis of Oral and Maxillofacial Surgery154

The palate develops from the primary palate (pre-palate) and secondary palate (palate). The wedge-shaped primary palate developed from globularprocess gives rise to parts of the pre-maxilla, nasal tipcartilage, nasal floor, lip, alveolus and triangular-shaped anterior palate. The lack of mesenchymaldevelopment of the central or lateral processes leadsto different varieties of prepalatal cleft. This is alsoassociated with hypoplasia of the maxillary structure.The structure posterior to incisive foramen gives riseto secondary palate. In a seven-week embryo, palatalprocess develops from maxillary process, extendsfrom primary palate to tonsilar fossa, and hangsvertically. Between the 8 to 9th weeks palatal shelverotates from vertical position to horizontal positiondue to straightening of the neck from flexed position

and dropping down of tongue, thereby separating theoral from nasal cavity. The fusion of primary palateand secondary palate takes place in a Y shaped mannerand the limbs of Y passes anteriorly between theincisor and canine teeth. The ventral 3/4th of thesecondary palate ossified to form the hard palate andfuses with the nasal ceptum. Dorsal 1/4th of thesecondary palate does not ossify and hangs like acurtain to form soft palate. The cleft of the palate occursdue to failure of fusion of palatal processes orsubsequent breakdown of mesenchymal structures.The clinical sequence of cleft palate, glossoptosis and

mandibular hypoplasia as described by Robin is amanifestation of early embryological defect.

Etiology

The etiology of the cleft lip and palate is multifactorialinvolving both genetic and environmental factors. Nosingle gene has been implicated to the causation offacial cleft. The facial cleft has been associated withvarieties of genetic syndrome. Because of syndromic

association, it is imperative to search for othercongenital anomalies associated with cleft lip andpalate specially, in the head and neck. Chromosomeabnormality Trisomy D syndrome may cause cleft lipand Wander Woud syndrome a genetic defect isassociated with lower lip defect. Experimentally, clefthas been produced in varieties of condition due todeficiency of vitamin A, Folic acid, Pantothenic acid,Riboflavin and Nicotinic acid. Cleft has also beenproduced by excess of vitamin A, hypoxia andingestion of various drugs like Nitrogen Mustard,Nucleic Acid Antagonist, Corticosteroid andIrradiation during pregnancy. Maternal smoking andalcoholism have also been implicated for theoccurrence of cleft lip and palate. The unilateral cleftlip in males has a higher hereditary background than

the incomplete cleft palate, which is more common infemales and has a low hereditary backgroundsuggesting different causes in the development of cleftlip and palate.

Anatomy of Cleft Lip and Palate

Cleft lip and palates are separate entities. However,cleft lip may be associated with cleft alveolus and cleftpalate. Severity of the deformity depends on theabnormal development of median nasal process andmaxillary process. In minor degree of cleft lip the

deformity, involve the front nasal process. Due toabsence of restraining force of orbicularis oris alveolarsegment is displaced outwards and pre-maxilla isflaired anteriorly. The alveolar gap varies from mildto severe with varying degrees of collapse of alveolararch. In bilateral cleft lip the pre-maxilla showsmarked protrusion giving a grotesque appearance.The teeth adjacent to the cleft are angled, distortedand lateral incisor may be absent. Nasal tip and

Fig. 22.11A: Furlows Z-plasty cleft palate

repairshowing incision line

Fig. 22.11B: Mobilization and reconstruction of flap by Z-plasty


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columella are short with flattening of the alar cartilagemaxillary hypoplasia. In incomplete unilateral cleftpalate, nasal septum (Vomer) is attached to the uncleftside of hard palate. In bilateral complete cleft lip and

palate, Vomer is free and septum hangs freely. Themusculature of soft palate is distorted. Levator palatimuscle is attached to the posterior age of the hardpalate instead of being directed towards the midline.Both the tensor and levator palati are attached to theeustachian tube causing malfunctioning of the tube.The cleft palate is occasionally associated withretroposition of tongue and mandibular hypoplasia,thus causing obstruction in the air passage andabnormality in swallowing reflex as described inPeirre Robins sequence.

Clinical Presentation

Cleft lip and palate presents with multiple clinicalproblems:1. Facial deformity is the immediate concern to the

parents and causes psychological problem to themother. Parent should be properly guided and beassured that the defect in her child is curable.

2. Sucking and eating Sucking of breast is not greatlyaffected in isolated cleft lip deformity as the infanttakes the nipple and areola inside the mouth during

breast-feeding. However sucking is affected in caseof cleft palate as tongue cannot compress the nippleagainst the cleft palate and negative pressure is notcreated during sucking. There will be regurgitationof feeds through the cleft palate.

3. Respiratory obstruction Isolated cleft palatedeformity may cause airway obstruction inpresence of Pierre Robins sequence due to thefalling back of the tongue and retrognathia andmay need immediate interference for oropharyn-geal reflex to develop.

4. Speech and phonation The complete speechmechanism is ensured by velopharyngeal closure.Voluntary contraction of soft palate aided by tensor

and levator muscle compress the soft palate againstthe nasopharynx and helps in the production ofspeech. Incomplete velopharyngeal closure is thehallmark of the cleft palate. Nasal intonation isacquired during production of vowel sounds in-patients with velopharyngeal incompetence andconsonant sounds are distorted.

5. Teeth Alveolar cleft interfere with the develop-ment of incisor and canine teeth. The incisor may

be absent or even duplicated. Maxilla is hypoplasticand smaller and alveolus on the lateral side is at alower level than the medial segment. Teeth on themaxillary side becomes crowded and there may

be occlusion diff iculties due to mandibularprognathism.

6. Respiratory tract Nasal tip is depressed andcolumella is short in cleft lip. Baby suffers fromrecurrent upper respiratory tract infection due tothe nasal regurgitation. Otitis media is commondue to the malfunctioning of eustachian tube andhearing may be affected.

Management

The aim of treatment of cleft lip and palate is to achievea. Normal appearance

b. Normal swallowing of feeds withoutregurgitation

c. Free airway passaged. Normal phonation ande. Alignment of teeth.

The general care should be started in neonatalperiod to achieve the goal.

Neonatal Care

Feeding

There is not much problems of feeding in babies withcleft lip though parents are worried about it. Feedingin a child with cleft palate is a definite problem asinfant is unable to suck properly due to the palatalgap and there is regurgitation of feeds duringswallowing. However, the feeding can be maintainedif milk is delivered at posterior part of the oral cavity

by specially created artificial nipple or spoon. Specialtype of feeding bottle or plastic bottle can be squeezedto deliver the food at the back of the oral cavity. Babyalso is to be held in 450 to prevent regurgitation offeed.

An orthodontist who can prepare a plate to coverthe gap in the palate, which helps in facilitating the

feeding, should examine the baby. The base plate canbe secured in position to help in the growth of thehard palate. Apart from this intra, oral or extra oralorthodontic appliances will be of great help to mouldthe growth of alveolus and maxilla. Elastic head captraction with elastic strapping for the projecting pre-maxilla is of utmost important procedure in new bornperiod. This makes the lip repair easy by decreasingthe gap between the lip and alveolus. The dynamic


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prolabium for central position. The Millard techniqueor modified Manchester technique (Straight lineclosure) can be used satisfactorily if the bilateralclosure is contemplated. The alveolar closure and

repair of the base of the nostril is done concurrently.In case of short columella, lengthening of columella isundertaken by V-Y Plasty at a later period.

Repair of Cleft Palate

Controversies exist in the surgical treatment of the cleftpalate repair regarding timing and technique of stagedversus complete repair. Early repair has a betterinfluence on the speech and skeletal deformities. The

basic goal of treatment is muscular closure of softpalate and closure of gap in the hard palate. This can

be achieved by side-to-side closure across the cleft intwo layers by mobilizing the mucoperiostial flap andrelaxation incision along the alveolar margin to releasethe tension in the suture line. The time old techniqueof Von Langenbeck is a straightforward closure butadequate palatal lengthening is not achieved. Toachieve the lengthening of the palate oblique anteriorincisions are made on both side of the alveolar grooveand mucoperiosteal flaps are mobilised from the hardpalate, the levator muscles are separated from free ageof hard palate and the muscles are closed in themidline. The palatal flaps are pushed back andrepaired in V-Y arrangement. This technique of Veau-Wardill Kilner repair helps in achieving the length ofthe short palate. The addition of Z plasty in the shortpalate helps in the gain of additional length. Two Zplasties described by Furlow are made in the oral andnasal side of the muscular and mucus tissue. Thepalatal length is gained with the reconstitution ofmuscle and mucosa of the soft palate. In huge gap thepalate can be repaired taking flap from the tongue andBuccal mucosal flap.

Secondary Surgery

Alveolar Gap

The alveolar gap is generally repaired concurrentlywith the cleft lip repair. But in case of huge gap bone,grafting is required to fill up the gap. The autologus

bone graft (Rib) or costal cartilage is undertaken atfive to six years of age. This procedure helps in theclosure of residual oronasal fistula and providessupport for the subsequent eruption of teeth.

Lip and Nose

In spite of different methods of techniques of the repairof cleft lip revisional surgery is required for scarcontracture, vermillion realignment, philtrumlengthening. Depressed nasal tip can be corrected bycartilage graft. columellar lengthening can be done byV-Y plasty in cases of short columella.

Palatal Revision

The incidence of post-operative fistula is very high 10percent to 20 percent even in experienced hands. Thecommon site of fistula formation is anteriorly at the

junction with the pre-maxilla and posteriorly at thejunction of the soft and hard palate. Different localflaps are created to close the fistula. However, buccal

mucosal flap or other distant flaps may be useddepending on the site and size of the fistula. In casesof short palate with velopharyngeal incompetence

baby suffers from nasal intonation. Musculomucosalflaps are taken from the posterior pharyngeal wall tolengthen the palate and to diminished the nasopharyn-geal openings. This pharyngeal flaps can be takeneither superiorly or inferiorly based to repair with theposterior palatal margin. This technique is improvedthe nasopharyngeal incompetence and diminished theair-leak via the nasopharyngeal openings.

FURTHER READING

1. American Cleft Palate-Craniofacial Association. Parametersfor the evaluation and treatment of patients with cleft lip/palate or other craniofacial anomalies. Cleft PalateCraniofacial J 1993;30(Suppl 1):4.

2. Brauer RO, Cronin TD. The Tennison lip repair revisited.Plast Reconstr Surg 1983;71:633.

3. Byrd HS. Cleft Lip. In: Smith JW, Aston SJ (Eds). Grabb &Smiths Plastic Surgery (4th edn) Boston, Little Brown andCompany. 1991;271.

4. Clarren SK, Anderson B, Woef LS. Feeding infants with cleftlip, cleft palate or cleft lip and palate. Cleft Palate J1987;24:244.

5. Cohen MM, Jr. Syndromes with cleft lip and palate. Cleft

Palate J 1978;15:306.6. Davis D. The one stage repair of unilateral cleft lip and

palate. Plast Reconstr Surg 1966;38:129.7. Dorf DS, Curtin JW. Early Cleft palate repair and speech

outcome. Plast Reconstr Surg 1982;70:74.8. Fara M, Dvorak J. Abnormal anatomy of the muscles of the

palato pharyngeal closure in cleft palate. Plast Reconstr Surg1970;46:488.

9. Fraser FC. Etiology of the cleft lip and palate. Am J HumGenet 1970;22:125.


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Synopsis of Oral and Maxillofacial Surgery158

10. Furlow LT Jr. Cleft Palate repair by double opposing ZPlasty. Plast Recnostr Surg 1986;78:724.

11. Furlow LT. Double reversing Z plasty for cleft palate. In:DR Millard (Ed), Cleft Craft, Vol. 3: Alveolar and palataldeformities. Boston: Little Brown, 1980

12. Jone MC. Facial clefting; etiology and developmentalpathogenesis. Clin Palst Surg 1993;20;599.

13. Kernahan DA, Bauer BS. Functional cleft repair: Asequential, layered closure with orbicularis musclealignment. Plast Recnostr Surg 1983;72:459.

14. Kernahan DH, Stark RB. A new classification for cleft lipand cleft palate. Plast Reconstr Surg 1958;22:435.

15. Kraus O. Anatomy of the velopharyngeal area in cleft palate.Clin Plast Surg 1975;2:261.

16. LeMesurier AB. Method of cutting and suturing lip incomplete unilateral cleft lip. Paslt Reconstr Surg 1949;4:1.

17. Millard DR, Jr Cleft craft: The evolution of its surgery, Thebilateral and Rare deformities. Boston: Little Brown 1977;2.

18. Millard DR. Bilateral cleft lip and a primary forked flap. In:Millard DR (Ed) Cleft Craft, the Bilateral Deformity. Boston:Little Brown, 1980;3.

19. Nicolau PJ. The orbicularis oris muscle. A functionalapproach to its repair in the cleft lip. Brit J Plast Surg1983;36:141.

20. Randall P, LaRossa D. Cleft Palate. In: Smith JW, Aston SJ(Eds) Grabb and Smiths Plastic Surgery (4th edn). Boston,

Little Brown and Company. 1991;281.21. Sadove AM, Eppley BL. Cleft lip and palate. In: ONeil JA

(Jr), et al. Pediatric Surgery (5th edn). St. Louis: Mosby,1998;693-700.

22. Sedove AM, Eppley BL. Timing of alveolar bone grafting: asurgeons view points. Prob Plast Recnostr Surg 1992;2:39.

23. Stark RB. The pathogenesis of hare lip and cleft palate.Plast Reconstr Surg 1954;13:20.

24. Tennison CW. The repair of unilateral cleft lip by stencilmethod. Plast Reconstr Surg 1952;9:115.

25. Watson ACH. Classification of cleft palate. In: Edward Mand Watson ACH (Eds), Advances in the management ofcleft palate. Edinburgh, Churchill Livingstone, 1980.

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Cleft Lip and Palate Ch 22