Article of Reconstruction of cleft lip and palate defect
Reconstruction of Cleft Lip and Palate Defect Amin AbusallamahAbstract a knowledge of the embryology of the face and the classification of cleft deformity. The structuralA cleft lip and palate is a type of birth defect that abnormalities that comprise the cleft deformityaffects the top lip and the roof of the mouth (the dictate the patient management and, in particular, the (1)palate). During the 6th to 10th week of surgical procedures employed. (Fig. 1) (4).pregnancy, the bones and tissues of a baby’s upperjaw, nose, and mouth normally come together (fuse)to form the roof of the mouth and the upper lip. Ifthe tissue in the developing mouth and the palatedon’t fuse together, a baby could be born with a (2)condition called cleft lip or cleft palate. theyshould be prepared for a protracted course oftreatment to correct the cleft deformities and toallow the individual to function with them. (3)Introduction and ReviewCLEFT LIP and PALATE This is the one of the Fig. 1 Development of the face at 5 weeks. (4)commonest group of congential malformationsaffecting the head and neck. The spectrum ofdisease seventy is wide, with defects such as bifid EMBRYOLOGYuvula and submucous cleft of the soft palate To understand the causes of oral clefts, a review ofrepresenting the most minor forms. Isolated cleft nose, lip, and palate embryology is necessary. Thelip, either unilateral or bilateral. There is an entire process takes place between the fifth andassociation of cleft lip with clefts of the primary and tenth weeks of fetal life. During the fifth week, twosecondary palate, and many of the syndromes of the fast-growing ridges, the lateral and medial nasalhead and neck have palatal or facial clefts as part of (3) swellings, surround the nasal vestige (Fig. 2-1,2,3)the symptom complex. An understanding of themanagement of cleft deformities requires
The lateral swellings will form the alae of the nose;the medial swellings will give rise to four areas: (1)the middle portion of the nose, (2) the middleportion of the upper lip, (3) the middle portion ofthe maxilla, and (4) the entire primary palate.Simultaneously the maxillary swellings willapproach the medial and lateral nasal swellings bulremain separated from them by wellmarked Fig. 2.3: Scanning electron micrograph of a embryo at A. 6- week embryo. The nasal prominences are gradually separatedgrooves. from the maxillary prominence by deep furrows.. B. 7-week embryo. Maxillary prominences have fused with the medial nasal prominences. (5) During the next 2 weeks, the appearance of the face changes considerably. The maxillary swellings continue to grow in a medial direction and compress the medial nasal swellings toward the midline. Subsequently these swellings simultaneously mergeFig. 2.1: Frontal aspect of the face. A. 5-week embryo. B. 6- with each other and with the maxillary swellingsweek embryo. The nasal prominences are gradually separatedfrom the maxillary prominence by deep furrows. (5) laterally. Hence the upper lip is formed by the two medial nasal swellings and the two maxillary swellings. The two medial swellings merge not only at the surface but also at the deeper level. The structures formed by the two merged swellings are known together as the intermaxillary segament (Fig. 3) which is comprised of three components: (1) a labial component, which forms the philtrum of the upper lip; (2) an upper jaw component, whichFig. 2.2: Frontal aspect of the face. A. 7-week embryo.Maxillary prominences have fused with the medial nasal carries the- four incisor teeth; and (3) a palatalprominences. B. 10-week embryo. (5) component, which forms the triangular primary palate. Above, the intermaxillary segment is continuous with the nasal septum, which is formed by the frontal prominence.
Two shelflike outgrowths from the maxillary.swellings form the secondary palate. These palatineshelves appear in the sixth week of developmentand are directed obliquely downward on either sideof the tongue. In the seventh week, however, thepalatine shelves ascend to attain a horizontalposition above the tongue and fuse with each other,thereby forming the secondary palate. Anteriorlythe shelves fuse with the triangular primary palate,and the incisive foramen is formed at this junction.At the same time, the nasal septum grows down andjoins the superior surface of the newly formedpalate. The palatine shelves fuse with each otherand with the primary palate between the seventhand tenth weeks of development. Clefts of the FIG.3: A, Frontal section through head ( 61 /a-weeK-oldprimary palate result from a failure of me.soderm to embryo. Palatine shelves are located fn vertical position on each side of tongue, B, Ventral view of same, Notepenetrate into the grooves between the medial nasal clefls between primary triangular palate and palatine ihelves, which arc itill in vertical position. C, Frontaland maxillary processes, which prohibits their section through head of 7V2-week-old embryo, Tonguemerging with one another. Clefts of the secondary has rtiovrd downward, and palatine shelves have reached horizontal position. D, Ventral view of same.palate are caused by a failure of the palatine shelves Shelves are in horizontal position, E, Frontal sectionto fuse with one another. The causes for this arc? through head of 10-week-old embryo. Two paiatine shelves have fused with each other and with nasalSpeculative and include failure of the tongue to septum. F, Ventral view of 5ame. (5)descend into the oral cavity. (5) INCIDENCE Cleft lip = 1: l000, Cleft palate (isolated) = 0.45: l000, Complete cleft = 1:1800, Submucous cleft = 1:1200, and Bifid uvula = 1: l00. (3)
GENETICS AND ETIOLOGY CLASSIFICATIONClefts of the upper lip and palate are the most The typical classification system used clinically tocommon major congenital craniofacial abnormality describe standard clefts of the lip and palate isand are present in approximately 1 in 700 live based on careful anatomic description. Clefts can bebirths. (6) The causes of facial clefting have been unilateral or bilateral; microform, incomplete, orextensively investigated. The exact cause of clefting complete; and may involve the lip, nose, primaryis unknown in most cases. For most cleft conditions, palate, and/or secondary palates (Fig. 5). (12)no single factor can be identified as the cause.However, it is important to distinguish betweenisolated clefts (in which the patient has no otherrelated health problem) and clefts associated withother birth disorders or syndromes. (7) Instead cleftsare thought to be of a multifactorial etiology with anumber of potential contributing factors. Thesefactors may include chemical exposures, radiation,maternal hypoxia, teratogenic drugs, nutritionaldeficiencies, physical obstruction, or geneticinfluences. One prevailing theory relates the processof clefting as a threshold in which multiple factorscome together to raise the individual above a FUNCTIONAL CONSEQUENCESthreshold at which time the mechanism of fusion To regard a cleft as a deformity only is inadequate.fails.# Recently multiple genes have been This malformation causes of functional disturbancesimplicated in the etiology of clefting. Some of these that indispensably should be taken intogenes include the MSX, LHX, goosecoid, and DLX (8) (9) (10) consideration in determining appropriate treatment.genes. some Syndromes Associated with (13)Cleft Palate like: Marfan syndrome , Apert 1. Nutrition Difficulties: Nutrition in cleft infantssyndrome , Downs syndrome , Digeorge syndrome , has often been described as difficult because ofEdward syndrome , Treacher collins , Pierre Robin their incompetence in sucking. In reality, babiesand Stickler syndrome. (11) need to suck only to position the nipple, and for
drinking they “milk” the nipple with their tongue. tongue position but malposition of teeth and gaps inNutrition difficulties are caused by the tongue the dental arches may contribute. (13) Voice diseasesposition in the cleft palate, occluding the nasal may occur accidentally or following attempts toairway. Therefore, cleft babies are unable to drink compensate for speech disturbances. Myofunctionaland breathe at the same time. (13) imbalances are the result of the displaced tongue2. Hearing Disorders: By swallowing , the with its dislocated functional pressure and theEustachian tubes are opened and the secretions of dysharmonious interaction of tongue and orofacialthe middle ear can flow off. In the presence of a musculature. (20) Mimic movements are the attemptcleft palate, however, the velopharyngeal ring by cleft patients to compensate for velopharyngeal (14)muscle system is interrupted and the insertion of incompetence by grimace with labial, nasal, orthe tensor veli palatin muscles at the tube cartilage frontal muscles. Speech therapy actually starts with (15) (16)is abnormal. In consequence, the opening breast-feeding, which demands considerable effortmechanism of the Eustachian tubes cannot work. by the child and provides practice for theThe middle ear secretions become thickened and are musculature. Later on, stimulation and (17)congested. The resulting seromucotympanon myofunctional therapy create favorable generalhinders sound conduction and promotes middle ear conditions for speech development. (21) (22)infections. Speech development relies on hearing 4. Growth Disturbances: Maxillary hypoplasia(18) and in cleft infants, who usually suffer some and scarring from surgery are said to be responsiblespeech problems anyway, defective hearing will for growth disturbances. Often the influence ofmean a double handicap. Moreover, in hearing muscle action on growth of bony structures is not (23)disorders the maturation of the hearing tracts to the considered, although it plays an important role.central nervous system is retarded. (19) This was recognized in 1961 by Rosenthal, who3. Speech Problems: Nasality, articulation described the functional stimulation on the cleftedproblems, suprapalatal resonance, voice diseases, maxilla by early reconstruction of soft palatemyofunctional imbalances, and mimic movements muscles. (24)may all be manifested in the speech problems of 5. Psychological Problems A number of studiescleft patients. Nasality is the result of have examined the influence that a cleft lip and/orvelopharyngeal incompetence caused by shortness palate has on the affected children themselves, theiror inadequate activity of the soft palate, which itself families, and the general public including health careis based on the displaced muscle insertions. professionals. (13)Articulation problems originate from a posterior
RECONSTRUCTION OF and alveolar structures during the presurgical phaseCLEFT LIP AND PALATE of treatment (Fig.6). Although the use of appliances probably makes for an easier surgical repair. (26) (27)The timing of cleft lip and palate repair iscontroversial. Despite a number of meaningfuladvancements in the care of patients with cleft lipand palate, a lack of consensus exists regarding thetiming and specific techniques used during eachstage of cleft reconstruction. Surgeons mustcontinue to carefully balance the functional needs,esthetic concerns, and the issue of ongoing growthwhen deciding how and when to intervene.(12) Table .1 FIG.6 :Frontal and lateral views of the Grayson nasoalveolar molding appliance showing the nasal projections that help to theoretically mold the nasal cartilages and maxillary segments into a more appropriate configuration prior to repair. (12) Unilateral Cleft Lip Repair Clefts of the lip and nose that are unilateral present with a high degree of variability, and thus each repair design is unique (Figure 7).The repair technique is usually performed after 10 weeks ofCleft Lip and Palate Repair age. The basic premise of the repair is to create aPresurgical Taping and Presurgical Orthopedics three-layered closure of skin, muscle, and mucosaFacial taping with elastic devices is used for that approximates normal tissue and excisesapplication of selective external pressure and may hypoplastic tissue at the cleft margins. Critical inallow for improvement of lip and nasal position the process is the reconstruction of the orbicularis (25)prior to the lip repair procedure. Some surgeons oris musculature into a continuous sphincter. Theprefer presurgical orthopedic (PSO) appliances Millard rotation-advancement technique has therather than lip taping to achieve the same advantage of allowing for each of the incision linesgoals.81,82 PSO appliances are composed of a to fall within the natural contours of the lip andcustom-made acrylic base plate that provides nose. This is an advantage because it is difficult toimproved anchorage in the molding of lip, nasal, achieve “mirror image” symmetry in the unilateral
cleft lip and nose with the normal side immediately Bilateral Lip Repairadjacent to the surgical site. Primary nasal Bilateral cleft lip repair can be one of the mostreconstruction may be considered at the time of lip challenging technical procedures performed inrepair to reposition the displaced lower lateral children with clefts. The lack of quality tissuecartilages and alar tissues. Several techniques are present and the widely displaced segments areadvocated, and considerable variation exists with major challenges toachieving exceptional results,respect to the exact nasal reconstruction performed but superior technique and adequate mobilizationby each surgeon. The primary nasal repair may be ofthe tissue flaps usually yields excellentestheticachieved by releasing the alar base, augmenting the results (Figs.8,9). Additionally the columella mayarea with allogeneic subdermal grafts, or even a be quiteshort in length, and the premaxillaryformal open rhinoplasty. Since lip repair is done at segment may be significantly rotated. Adequatesuch an early point in growth and development, the mobilization of the segments and attention to theauthors prefer minimal surgical dissection due to the details of only using appropriately developed tissueeffects of scarring on the subsequent growth of these will yield excellent results even in the face oftissues. McComb described a technique that has significant asymmetry. Some surgeons have usedbecome popular, consisting of dissecting the lower aggressive techniques to surgically lengthen thelateral cartilages free from the alar base and the columella and preserve hypoplastic tissue usingsurrounding attachments through an alar crease banked fork flaps. Early and aggressive tissue flapsincision. This allows the nose to be bolstered and/or in the nostril and columella areas do not lookstented from within the nostril to improve symmetry. natural after significant growth has occurred and result in abnormal tissue contours. While surgical attempts at lengthening the columella may look good initially, they frequently look abnormally long and excessively angular later in life (Fig.11). Revision of these iatrogenic deformities is difficult and some of the contour irregularities will not be able to be revised adequately. Usually if the hypoplastic tissue is excised and incisions within the medial nasal base and columella are avoided,FIG. 7: A complete unilateral cleft of the lip is shownhighlighting the hypoplastic tissue in the cleft site that is not the long-term esthetic results are excellent.used in the reconstruction. (12)
(2) the anatomic repair of the musculature within the soft palate that is critical for normal creation of speech (Fig.10). FIG.10:A, Presurgical frontal view of a wide bilateral cleft lip and palate with significant asymmetry and lack of columellaFIG.8:The bilateral cleft of the lip and maxilla shown here is length. B, Presurgical left lateral view of a wide bilateral cleftcomplete and highlights the hypoplastic tissue along the cleft lip and palate with a protrusive premaxillary segment. Noteedges. the short columella length. C, The same child at 10 months of age after repair of her bilateral cleft lip and palate. No presurgical taping or orthopedic appliances were used. The exact timing of repair of a palate cleft is controversial. Generally the velum must be closed prior to the development of speech sounds that require an intact palate. (12) On average this level of speech production is observed by about 18 months of age in the normally developing child. If the repair is completed after this time, compensatory speechFIG.9: A, Presurgical appearance of the incomplete bilateral articulations may result. Repair completed prior tocleft lip of a 3-month-old boy. B, Surgical markings forexcision of the hypoplastic tissue and the planned creation of this time allows for the intact velum to closea new philtrum. Advancement flaps from the lateral lipsegments bring good white-roll to the midline via small effectively, appropriately separating thecutbacks. C, The same child at 1 year of age after the repair ofhis bilateral cleft lip. nasopharynx from the orophayrynx during certain speech sounds. (28) (29) (30) When repair of the palate is performed between 9 and 18 months of age, theCleft Palate Repair incidence of associated growth restriction affectingThere are two main goals of cleft palate repair the maxillary development is approximatelyduring infancy: (1) the water-tight closure of the 25%. (31) (32) (33) Cleft palate reconstruction requiresentire oronasal communication involving the hard the mobilization of multilayered flaps to reconstructand soft palate. the defect due to the failure of fusion of
the palatal shelves. space between the nasopharynx preserves an anterior pedicle for increased bloodand oropharynx during certain speech sounds, the supply to the flaps. (34; 35; 36) This technique is alsosurgeon must also reconstruct the musculature of successful in achieving a layered closure but may bethe velopharyngeal mechanism. The musculature of more difficult when suturing the nasal mucosa nearthe levator palatini is abnormally inserted on the the anteriorly based pedicle attachments. Anotherposterior aspect of the hard palate and therefore common technique is the Furlow double-opposing Zmust be disinserted and reconstructed in the plasty, which attempts to lengthen the palate bymidline. Many techniques for repair like; The taking advantage of a Z-plasty technique on bothBardach two-flap palatoplasty uses two large nasal mucosa and oral mucosa (Fig.12). (36) (37)fullthickness layered dissection and brought to themidline for closure (Fig.11).Fig.11: A, A unilateral cleft of the primary and secondary Fig.12: A, A complete cleft of the secondary palate (bothpalates, typical involvement from the anterior vestibule to the hard and soft) is shown from the incisive foramen to the uvula.uvula. B, The Bardach palatoplasty technique requires two B, The Furlow double opposing Z-plasty technique requireslarge full-thickness mucoperiosteal flaps to be elevated from that separate Z-plasty flaps be developed on the oral and theneach palate shelf. The anterior of the cleft is not reconstructed nasal side. C, The flaps are then transposed to theoreticallyuntil the mixed dentition stage. C, A layered closure is lengthen the soft palate. A nasal side closure is completed inperformed palatoplasty by reapproximating the nasal the standard fashion anterior to the junction of the hard andmucosa. D, Once the nasal mucosa and musculature of the soft palate. D, The oral side flaps are then transposed andsoft palate are approximated, the oral mucosa is closed in the closed in a similar fashion completing the palate closure. (12)midline. (12)flaps that are mobilized with The von Langenbecktechnique is similar to the Bardach palatoplasty but
Bone Grafting References:At one time, bone grafting tended to be performed 1. NHS inform. www.nhsinform.co.uk. [Online] NHS, Octoberonly in bilateral alveolar clefts to fix a mobile 4, 2011. http://www.nhsinform.co.uk/health-premaxilla to the maxilla. This reason remains library/articles/c/cleft-lip-and-palate/introduction.valid.81 It has however now been extended to 2. cleft smile. www.cleftsmile.org. [Online] Cleft Lip and Palate Foundation of Smiles Inc., april 16, 2011.include the achievement of a continuous alveolus in http://www.cleftsmile.org/custom/introduction/.all cleft patients with an alveolar defect. For those 3. RIDEN, K. Key Topics in Oral and Maxillofacial Surgery.patients who will subsequently need a maxillary Plymouth, UK : BIOS Scientific PublisherLs td, 1998. p. 69. ISBN 1 85996 030 8.osteotomy, it creates the possibility of thatprocedure being carried out more safely, and 4. Wray, David, et al. Textbook for General and Oral Surgery. London : Elsevier Science, 2003. pp. 130 - 139. ISBNperhaps with more stability, in one piece. (13) 0 4430 7083 0. 5. Sadler, T.W. Langmans Medical embryology. s.l. : Lippincott Williams & Wilkins, 2003. ISBN: 0781743109.Conclusions 6. Classification and birth prevalence of orofacial clefts.The complete care of patients with clefts requires an Tolarova MM, Cervenka. 2, San Francisco : Am J Med Genet,interdisciplinary approach that demands precise 1998, Vol. 75, pp. 126–37. 1552-4833.surgical execution of the different procedures 7. Ellis, Edward. Management of Patients with Orofacial Clefts. [book auth.] Edward Ellis III, Myron R. Tucker Jamesessential to correct cleft deformities, as well as R. Hupp. Contemporary Oral and Maxillofacial Surgery, 4regular long-term follow-up. Clinicians experienced ED. US : Mosby Elsevier, 2002, p. 628.in the comprehensive interdisciplinary care of 8. Identification of susceptibility loci for nonsyndromic cleft lip with or without cleft palate in a two stage genome scan ofpatients with clefts are best equipped to deal with affected sib pairs. Prescott NJ, Lees MM,Winter RM,Malcolmthese concerns. The treatment of patients with cleft S. 3, London : Hum Genet, 2000, European Journal of Human Genetics, Vol. 106, pp. 345-350.and craniofacial deformities should be free of bias 9. Mutations of PVRL1 encoding a cell-cell adhesionand should demand team care that is patient, family, molecule/ herpesvirus receptor, in cleft lip/ palate-ectodermaland community oriented. Only in this fashion can dysplasia. Suzuki K, Hu D, Bustos T, et al. US : Nature Publishing Group, 2000, Nature Genetics journal, Vol. 25, pp.the overall treatment be optimally successful. This 427-430. 1061-4036type of care maximizes the patient’s ability to grow 10. MSX1 mutation is associated with orofacial clefting andinto adulthood and succeed in life without focusing tooth agenesis in humans. Van den Boogaard MJ, Dorland M, Beemer FA, van Amstel. US : l Nature Publishing Group,on their defect. 2000, Nature Genetics journa, Vol. 24, pp. 427- 430. 1061- 4036. 11. Eastern illinois university. [Online] http://www.ux1.eiu.edu/~cfmah/cleft/syndromes.html.
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