© Borgis - New Medicine 1/2001, s. 30-32
Zofia Dudkiewicz, Maria Hortis-Dzierzbicka
Early surgical management of unilateral and bilateral cleft lip and palate - standards of care
Department of Paediatric and Adolescent Surgery National Research Institute for Mother and Child, Warsaw, Poland Centre of Craniofacial Disorders
Head: Associate Professor Zofia Dudkiewicz, M.D.
Summary
This paper presents standards for early surgical treatment of unilateral and bilateral cleft lip and palate as well as isolated cleft palate, developed and in use at the Centre for Craniofacial Disorders National Research Institute for the Mother and Child, Warsaw, Poland. The authors discuss the arguments for such an approach, in the light of modern views on factors influencing maxilla growth and speech development in patients with orofacial clefts.
Clefts of the lip and palate result in a functional imabalance of structures forming the facial portion of the skull and in deformities affecting the external facial appearance, dental relationships, craniofacial growth, ENT status and speech. In view of the multi-disciplinary character of treatment and rehabilitation, combined with the need for long-term follow-up of surgical results, quality and effectiveness of multi-stage treatment, the establishment of specialist centres to treat these defects is warranted, as is the establishment of databanks that might facilitate the development of standards.
The management of children with cleft lip and/or palate begins within the first year of life, and may be continued until the patient is 19-20 years old. Surgical procedures and other forms of therapy undertaken in the early period affect the patient´s vital functions and appearance throughout life. This is why it is of paramount importance to document all forms of therapy and the outcome, starting in very early childhood and ending when the growth process is completed. The outcome varies depending on the type of the anomaly and - to some degree - on the extent of the deformity.
Early restoration of the continuity of the clefted craniofacial structures facilitates the development and rehabilitation of speech (2, 3, 5, 6). No firm data is available on any association between an improvement in conditions for occlusion rehabilitation and any particular surgical technique. The common opinion is that there are numerous routes to success (1), and this success mainly depends on appropriate orthodontic treatment, continued phoniatric diagnostic and therapeutic management, and regular speech therapy.
In classifying clefts, the most popular systems are based on embryological foundations, such as the classification developed by Kernahan and Stark (4). This defines the incising foramen as the embryological border between the clefts of the phylogenetically primary and secondary palates. Thus, the authors distinguish between two major types of cleft, which form a multitude of combinations. Clefted anatomical regions situated in the anterior position vis a vis the incisive foramen (the lip, alveolar process and a part of the hard palate) have become known as clefts of the primary palate, while clefted regions posterior to the incisive foramen (the major part of the hard palate and the soft palate) have been termed clefts of the secondary palate. Appropriate marking of the side of the cleft has helped to establish a classification system that is at present the most widely recognized in the world.
Therapeutic management
The management of a craniofacial cleft is multi-specialist, often multi-stage, and long-term. Since the defect itself is clearly visible on the face, can often be heard when the patient speaks, and the stigma of cleft lip remains even after the best surgical treatment, the effects of such a deformity on the personal and social life of the patient is enormous. Hence the selection of proper surgical, orthodontic, phoniatric and otolaryngologic management, as well as speech rehabilitation at each stage of treatment, are of paramount importance.
Our standards of surgical management in primary and secondary palate clefts
In common with more and more cleft surgeons (2, 5), we believe that elevating the mucoperiosteum at the time of palate repair might be detrimental to the development of the maxilla.
After many years of surgical experience in cleft care we fully agree with Ross (7) in his comment that scarring in the areas of denuded bone is the main cause of maxillary growth impairment in orofacial clefts.
In our Craniofacial Centre in the years 1981-2000 ca 300 children with unilateral or cleft lip and palate underwent early one - stage primary surgical repair of UCLP at a mean age of 6-8 mo. During this time three methods of palatal cleft repair were employed.
In the first method (56 children now being aged 15-20 yrs), cleft palates were closed by a modified von Langenbeck procedure. A two - layer closure of the hard palate was performed. Two to five mm of denuded bone was left by the alveolus. In order to keep the facial muscular balance a rib bone graft of about 1 cm length was inserted subperiosteally at the level of the base of the nasal ala. A triangular flap for lip repair was used.
In the second method (82 children being now aged 15-10 yrs) bone grafting was abandoned. To close the palatal cleft, which was also closed with two layers, only one mucoperiosteal flap was mobilised and sutured to the clefted side. A large denuded bone area was left at the unclefted side.
In the third method (about 160 children now aged from 8 mo to 10 yrs) a raised and prolonged vomer flap was used for closing the palatal cleft. All palatal wounds were tightly sutured. No area of denuded bone was left by the alveolus. Almost all children operated on before the end of the first year of life achieved normal conditions for speech development before starting school. Minor articulation errors were caused by dental problems.
The change in surgical techniques of palatal surgery to the least traumatic one in the third group resulted in a significantly better occlusion conditions in this group.
Bilateral cleft lip and palate
3-4 months of life - a complete reconstruction of the soft palate cleft, closing of the fissure in the hard palate, and reconstruction of the lip on the narrower side. A partial closing of the contralateral hard palate. All wounds must be tightly closed.
6 weeks later - a complete reconstruction of the lip, oral vestibule and palate. Make sure the continuity of the orbicular muscle of the mouth is completely restored and the hypoplastic cartilages of the medial crus are released. Major palate-supplying vessels should not be dissected. Strive for a tight closure of all wounds.
Early closures are medically warranted due to the uncontrolled growth of the intermaxillary bone and the resultant deformity of the maxilla, which exerts a marked effect on future problems in surgical, orthodontic, phoniatric and logopedic management.
4-5 years of life - the nasal septum is lengthened and minor lip corrective procedures are performed.
9-11 years of life - elimination of the residual alveolar cleft by secondary bone grafting. Possible reconstruction of the intermaxillary bone is performed if needed.
16-18 years of life - minor cosmetic corrections, scar dermabrasion.
Unilateral cleft lip and palate
6-8 months of life (the first period of maxilla growth stabilization) - a one-stage reconstruction of the nose, lip and palate. Currently, an earlier date for surgery is dictated by the mother´s desire to breast-feed (the child is either his or her fed mother´s his or her milk from a bottle, or is breast-fed under supervision). In the future, if we are sure that decreased surgical trauma is associated with a lower degree of maxilla deformity, the first operation will be scheduled when the child is 3-4 months old.
2-3 years of life - bone grafts are implanted into the alveolar process, as recommended in view of nasal obstruction and fistulas between the hard palate and the intermaxillary bone that hinder appropriate speech therapy. When the child is 5 years old, a pharynoplasty is performed in patients with velopharyngeal incompetence due to structural deficiences.
9-11 years of life - secondary bone grafting is the standard procedure in reconstructing the cleft maxilla. Minor cosmetic corrections are performed.
16-18 years of life - final bone and soft tissue repair procedures, and cosmetic procedures, e.g. scar dermabrasion.
Isolated cleft palate
6-8 months of life - a complete reconstruction of the palate.
2-5 years of life - closure of palatal fistulas or a pharyngoplasty when recommended by a phoniatrician or speech therapist. Such procedures should be treated as complications of the primary reconstruction or as the effect of primary soft tissue hypoplasia.
Our early observations over several years indicate good effects from surgical treatment performed in 3/4--month-old children, which may suggest that medically indicated procedures may contribute to advancing the date of surgery in all types of orofacial cleft defects, allowing a greater possibility of breast-feeding these patients.
Incomplete clefts and complicated cases are treated individually, following the principles described below:
Strive for a complete reconstruction of the peripheral speech apparatus.
Strive for minimum surgical trauma (elimination of healing by granulation).
Have respect for the existing, although deformed, structures. Strive for the restoration of well-organised tissues and systems. Limit excisions, and observe the principle that all elements are important.
Perform the reconstruction of the alveolar process using an iliac bone graft when a team decision is reached and the child is 8-11 years old. Usually, the decision is affected by the process of permanent teeth eruption and conditions for speech rehabilitation.
In patients with orofacial clefts, craniofacial reconstruction procedures are treated as final and performed after the permanent teeth have stabilised.
Medical documentation in patients with clefts should be consistent with the minimum documentation approved by Eurocleft in June 1999 (see Tables).
In our opinion, such documentation should include:
External facial appearance - photographs of the face at rest and in use motion.
Video recording of mimical facial movements.
Speech recording and an evaluation of speech disturbances characteristic of cleft defects.
Videonasofiberoscopy and functional cephalometry as quantitative methods of visualization and assessment of the palatopharyngeal closure.
Static cephalometry, teleroentgenograms and dental casts.
Evaluation of the patient´s psychosocial status using specially developed questionnaires.
Epidemiology/genetics.
Capitalizing on the experience gained from three national conferences and acquired from international publications and meetings, we at the Institute for Mother and Child hereby put forward a proposal to adopt a universally - accepted format for medical documentation that will constitute the foundation for a database. This will permit establishment of a greater number of patient groups treated by various methods and facilitate the elimination of poor results. We do not suggest a change in surgical management, but through our own exemple we ask that you do your best in order to render the comparison of surgical results possible. And thus:
Let us establish centres.
The two most active centres should be acknowledged as teaching facilities.
Working in specialist groups, representatives of all centres should approve documentation standards.
The same representatives should compare their sample medical records in the presence of all team members.
As a result we will have established standards of surgical management.
We believe that even if medical records pertaining to initial treatment are incomplete, but data are available on the character of the defect and the management is described, each centre where conceptual treatment is carried out may join our investigations, especially since they are based on principles of international studies.
Piśmiennictwo
1. Berkovitz A: A multicenter retrospective 30 study of serial complete unilateral cleft lip and palate and complete bilateral cleft lip and palate to evaluate treatment: Part 1 - The participating institutions and research aims. CP Craniofac J 1999, 36:413-424. 2. Desai SN: Early cleft palate repair completed before the age of 16 weeks: observations on a personal series of 100 children. Br J Plast Surg 1983, 36:300-304. 3. Dorf DS, Curtin JW: Early cleft palate pair and speech outcome. Plast Reconstr Surg 1982, 70:74-81. 4. Kernahan DA, Stark RB: A new classification for cleft lip and palate. Plast Reconstr Surg 1958, 22:435-441. 5. Malek R, Martinez H, Mousset MR, Trichet C: Multidisciplinary management of cleft lip and palate in Paris, France. In: Multidisciplinary management of cleft lip and palate. Saunders Comp, New York 1990. 6. Randall P, La Rossa D, et al.: Cleft palate closure at 3 to 7 months of age: a preliminary report. Plast Reconstr Surg 1983, 71:624-628. 7. Ross B: Treatment variables affecting growth in unilateral cleft lip and palate. Part 5: Timing of palate repair. CPJ 1987, 24:54-63.
