combined early cleft lip and palate repair

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Journal of Plastic, Reconstructive & Aesthetic Surgery (2009) xx, 1e7

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Combined early cleft lip and palate repair inchildren under 10 months e a series of 106 patients

Andrew M. Hodges*

Department of Plastic Surgery, CoRSU Rehabilitation Hospital, PO Box 46, Kisubi, Kampala, Uganda

Received 11 June 2009; accepted 27 October 2009

KEYWORDSCleft lip;Cleft palate;Combined

* Tel.: þ256(0) 712563031.E-mail address: andrewhodges3001

1748-6815/$-seefrontmatterª2009Britdoi:10.1016/j.bjps.2009.10.033

Please cite this article in press as: Hpatients, J Plast Reconstr Aesthet Su

Summary This article reviews a series of 106 patients presenting with cleft lip and palatewho underwent a simultaneous combined cleft lip and palate repair under the age of 10months. The technique is described together with the early postoperative results. A singlesurgeon (the author) operated on the 106 patients. The youngest patient was 6 weeks old,and the smallest weighed 2.3 kg. All patients underwent palate repair, followed by liprepair, at a single sitting. The palate repair used the Sommerlad technique with radicalmuscle repositioning and bilateral lateral Langenbeck-type releasing incisions when indi-cated. Of the total, 71 patients (67%) had a unilateral lip and palate cleft and underwenta modified Millard repair; 34 (32%) had a bilateral lip defect and underwent a modified Mul-liken repair; and one (1%) had a midline cleft lip. Ten patients were excluded from the study,as their palate was deemed too wide to close. Instead, they underwent cleft lip repair andvomer flap to the anterior palate alone. The mean duration of the procedure was 97 min.There was neither mortality, nor significant anaesthetic complications. Two patients whohad low oxygen saturation postoperatively were taken back to the theatre. In both cases,the soft palate sutures were removed and the airway improved to a safe degree, permittingreturn to the ward for subsequent final repair. All patients were discharged home withoutany ongoing problems. One patient subsequently developed a unilateral dehiscence ofa bilateral lip, and seven patients underwent a second procedure to close a palatal fistula.Early follow-up results are encouraging, with only 8% of patients to date requiring a secondprocedure.Conclusion: Although technically challenging, cleft lip and palate repair in a single simulta-neous procedure is a successful and, most importantly, a safe procedure, which enables thecomplete clefting condition to be repaired early and in a single operation.ª 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published byElsevier Ltd. All rights reserved.

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odges AM, Combined early cleft lip and palate repair in children under 10 months e a series of 106rg (2009), doi:10.1016/j.bjps.2009.10.033

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Throughout the history of cleft surgery, there has beendebate concerning the optimal timing of surgical repair,and, in particular, the timing of cleft palate closure. Thethree main issues in this debate are the safety of thesurgery, the impact of the surgical process on midfacialgrowth and the impact of the timing on subsequent speechdevelopment. Probably the most common timing sequenceadopted the world over is to perform cleft lip repair at 3months, and cleft palate repair secondarily at around 9months. This protocol was adopted in our cleft service inUganda from inception until 2005. Reflection on our expe-rience and subsequent retrospective review of our data ledus to realise that children born with a cleft lip and palate ina resource-poor environment have a significant risk ofdeath from malnutrition because of the cleft-inducedinability to breastfeed. In addition, economic pressures onfamilies make it extremely difficult for them to travel(especially long distances) many times for surgery andfollow-up. In 2005, therefore, we changed our protocol forthe timing of cleft repair. All patients presenting witha complete cleft lip and palate underwent a one-stage cleftlip and palate repair if they were deemed fit for anaes-thesia. The purpose of this study was to assess the technicalaspect of performing such a one-stage operation and toassess the safety and early postoperative results in infantsless than 10 months of age.

Material and methods

All patients who presented to the cleft team of the Mengohospital with a complete cleft lip and palate were consid-ered for a one-stage cleft lip and palate repair. Patientsunder the age of 10 months were enrolled into the studyand a single surgeon (the author) operated on all thepatients in this study. Many were malnourished onpresentation; however, malnutrition was not in itselfa contraindication to operate as long as they were feeding,gaining weight and had no other concurrent illness. A totalof 106 patients aged 6 weeks to 9 months underwentcombined cleft lip and palate repair. Eleven were excludedfrom the study: one underwent only a palate repairbecause of a lack of cross-matched blood, and in 10patients the palate was deemed too wide for completepalate repair e these patients underwent cleft lip repairand anterior vomer flap. No presurgical or postsurgicalorthodontics was used.

A paediatric anaesthetist clinically evaluated patientspreoperatively, and weight and haemoglobin (Hb) levelswere recorded. All patients were intubated using a pre-formed oral endotracheal tube. Blood transfusions wereadministered as required, taking into account the preop-erative Hb level and the blood loss during the procedure.Postoperatively, patients were closely monitored for 24 hduring which time hourly oxygen saturation levels, heartrate and respiratory rate were recorded. At 48 h, a repeatHb level was recorded. Oral feeding was commenced in therecovery room initially using sugared water. Patients whowere observed to be feeding and breathing satisfactorilywere discharged, usually at day 5. Subsequently, thepatients were followed up at 3- and 6-month intervals andyearly thereafter.

Please cite this article in press as: Hodges AM, Combined early cleft lipatients, J Plast Reconstr Aesthet Surg (2009), doi:10.1016/j.bjps.200

Surgical technique

After satisfactory induction of anaesthesia, and placementof an endotracheal tube with a throat pack, the patient isplaced in the supine position at the end of the operatingtable, with the neck slightly extended and the operatingtable tilted in a slight reverse Trendelenburg position.Preoperative photographs are taken, and the lip ismeasured and key points on the Cupid’s bow are markedand tattooed. The palate and lip are infiltrated witha mixture of lignocaine and 1:100 000 adrenaline. There-after, the operative field is cleaned and draped. Thesurgeon sits at the end of the table throughout theprocedure.

Palate repair

The repair is essentially a Sommerlad palate repair1 withsome modifications. A Sommerlad palate gag is insertedand the dimensions of the cleft are measured. Measure-ments are taken at the junction of the hard and softpalates, as this is frequently the most challenging areato close in a wide palate. The width of cleft in comparisonto the width of each palatal shelf gives an indication as tohow challenging the repair will be, but no clear cut-offpoint has been identified beyond which palate repair is notpossible. An incision is made along the edge of the cleft,and the nasal mucosa is freed from the palatal shelf. Onthe non-cleft side, in a unilateral cleft palate, the vomerflap is developed and brought over to meet the freed nasalmucosa from under the palatal shelf on the cleft side. Thenasal mucosa is apposed with interrupted sutures. If thereis tension at the junction of hard and soft palates, an‘extended’ vomer flap is used. This frequently is requiredif the cleft is wider than 10-mm at the junction of thehard and soft palates. A triangular extension to the vomerflap extending posteriorly into the pharynx is raised,elevated and used to fill the gap at the junction of hardand soft palates. Once the nasal layer is completed, thepalatal muscle bundles on each side of the cleft aredissected free from nasal and oral mucosa to enable themto be retroposed posteriorly and sutured together in theform of a sling. Usually bilateral lateral Langenbeck-typereleasing incisions are required, and the lateral incisioncould be made all the way anteriorly on one or both sidesin a wide alveolar cleft to enable the oral mucosa to beapposed anteriorly. Then, oral mucosa is sutured together.Following repair, the pack around the endotracheal tube ismaintained and a further pack inserted to aid haemostasisin the mouth. The gag is removed and attention turned tothe lip repair. At the completion of the lip repair, hae-mostasis is confirmed and the packs are removed prior toextubation.

Unilateral Lip repair

A Millard repair2,3 is employed with a number of modifica-tions. Opposing points on the white roll are tattooedearlier. A curved incision along the mucosa on the cleft sideenables the lip to rotate down. Modest muscle dissectionseparates muscle from skin and mucosa. On the non-cleft

p and palate repair in children under 10 months e a series of 1069.10.033

Figure 1 Bilateral cleft lip and palate repaired at 4 months of age with a 6 month follow-up.

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Combined early cleft lip and palate repair in children under 10 months 3

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side, a small triangular Tennison flap4 and a Noordhoffmucosal flap5 are used to augment lip-length deficiency onthe cleft side e these triangles are fitted into back-cutsabove the white roll and on the dry mucosa, respectively.No back cut is required in the nasal floor on the non-cleftside. Modest muscle dissection is employed, and, inparticular, the proximal muscle bundles are not dissectedaway from the alar base and so muscle apposition aids innasal correction. Mucosal flaps are preserved and used asrequired to assist in closing any defect caused by a widealveolar cleft. If the anterior cleft is not wide, no buccalsulcus back cuts are required. The nasal correction includesmobilisation over the maxilla subperiosteally and excisionof the nasal web and freeing of mucosa from cartilage.Mucosa is sutured first, followed by muscle and then skin.Finally, a suture could be passed from the alar rim to theaccessory nasal cartilage to lift and slightly overcorrect thecleft nostril.

Bilateral lip repair

A modified Mulliken6 repair is performed. A narrow prola-bial skin flap is raised based proximally and prolabial fattytissue thinned. The prolabial mucosa is turned over to coverthe prolabium. Laterally, mucosa is paired from the cleftedges and the muscle is dissected back more radically thanfor a unilateral lip as it has to be mobilised sufficiently toenable it to reach across to the midline. Buccal sulcus backcuts are required to enable lateral mucosa also to reach themidline. Nasal dissection is as for unilateral lip with nasalweb excisions, but it is performed on both nostrils. Mucosais sutured followed by muscle and skin. Sutures to lift thealar rim and improve the columellar complete the repair.


Results

A total of 106 patients underwent combined lip and palaterepair of which 62 (58%) were male and 44 were female(42%). Of these, 71 patients (67%) had a unilateral lip, 34(32%) had a bilateral lip and one (1%) had a midline cleft lip.The youngest patient in the study was 6 weeks old; 71% ofthe study group was under 6 months of age and the averageage was 4.5 months. The smallest patient at the time ofoperation was 2.3 kg and the average weight of the patientswas 4.9 kg. The average operating time, measured from thecommencement of palate repair to the completion of liprepair, was 97 min (range 70e135 min). All patients hadsuccessful closure of the cleft palate, although 22 requiredthe ‘extended vomer flap’ to fill the gap at the level of hardand soft palates. The palates had an average width of11 mm (range 7e15 mm) and those patients who requiredan extended vomer flap had an average width of 12.3 mm(range 10e15 mm) (Figure 5). Thirty-two patients (28%)required a transfusion averaging 94 ml per patient (32e300 ml). The average preoperative Hb in all patients was9.7 g dl�1 (range 5.8e13.9 g dl�1) and the average Hb forpatients who were transfused was 8.9 g dl�1 (range 5.8e12.5 g dl�1). The average drop in Hb in patients who werenot transfused was 1 g/dl�1, whereas on average it rose by2.5 g dl�1 in patients who were transfused. There werethree perioperative complications. Two patients hadoxygen saturation levels persistently below 90% post-operatively when breathing air alone, and it was felt thata degree of pharyngeal obstruction might be a contributingfactor. One patient was immediately taken back to thetheatre and the soft palate sutures were removed. Theother patient, who had the same procedure following the


Figure 2 Unilateral cleft lip and palate repaired at 3 months of age with a 6 month follow-up.

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oxygen saturation, showed no improvement by day 3. Bothpatients demonstrated an immediate return to normaloxygen saturation. One patient with a wide bilateral lip hada unilateral dehiscence, which was repaired at a later date.

Figure 3 Unilateral cleft lip and palate showing palat


Oxygen was administered if the oxygen saturation droppedand stayed below 94%. Ten patients (10%) required someoxygen postoperatively, but only two patients had notnormalised by 24 h. One continued to improve without

e repair on table and early appearance of lip repair.


Figure 4 Bilateral cleft lip and palate repaired at 4 months of age in a 3.4 kg baby. Post-op photos at 1 year follow-up with nofistula.

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intervention and the other improved after the soft palatesutures were removed. There was neither perioperativemortality nor episodes of postoperative haemorrhage. Allpatients commenced feeding without requiring a naso-gastric tube and postoperative pain was not a significantissue; and they were discharged home feeding well andpain free.

On review after �3 months, seven patients have hada fistula requiring closure (6.5%), although a total of 26patients (25%) had some degree of nasal incontinence onfeeding with liquids e23 with anterior fistulae, one smallmid-palatal fistula and two partial soft-palate dehiscences.Following the initial repair, nine patients have hada subsequent procedure (seven fistula repairs, one re-repairof bilateral lip and one re-repair of palate). Therefore, atpresent, 92% of patients have only required one procedure.

Follow-up of patients has been incomplete with only 64patients returning to the clinic for review (60%). 6 Repre-sentative unilateral and bilateral cleft patients are illus-trated in Figures 1e6.

Discussion

To our knowledge, very few cleft centres performcombined cleft lip and palate repairs, and, more


specifically, few centres perform cleft palate repair oninfants under the age of 6 months. Desai7 reported a seriesof 100 children who underwent neonatal cleft lip repairfollowed by cleft palate repair at the age of 16 weeks. DeMey8 has reported a series of 18 infants with complete cleftlip and palate repaired at 3 months of age. The mostcommon protocol adopted in our experience is cleft liprepair at around 3 months of age, followed by palate repairat 9 months or even later. When there is an already-established and successful protocol in place, strong reasonsare required to change this, particularly if there could behigher risk or the procedure could adversely affect long-term results. In our resource-poor environment, we realisedthat the protocol of early lip repair, followed by laterpalate repair, has some significant disadvantages:

� Retrospective study of our cleft series has demon-strated a very different distribution of cleft lipscompared to cleft lip and palate and to isolatedpalates,9 wherein 74% of patients had an isolated cleftlip and 24% had a cleft lip and palate. In most inci-dence surveys, cleft lip and palates are significantlymore common than isolated cleft lips.10 By extrapo-lation using data from other surveys, we suggest that75% of cleft lip and palate patients do not attend for


Figure 5 Wide cleft palate (14 mm at junction of hard and soft palate) in a seven month old baby, with closure using the‘extended vomer flap’.

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surgery. This is supported by an ongoing incidencesurvey in Kampala where, at present, 71% of infantsborn with a cleft have a cleft lip and palate (ascompared to the 24% in our hospital series). Our cleftpalate patients are significantly more malnourishedthan those presenting with a cleft lip, and we believethat the reason for non-attendance of infants witha cleft palate is the high risk of mortality of up to 75%in this environment.� The driving factor for parents to present their infants

for surgery is the appearance of the cleft lip. There islittle understanding about the importance of cleftpalate repair, which is demonstrated starkly in thisstudy wherein 10 patients underwent only anteriorvomer flap and cleft lip repair due to the width of their

Figure 6 unilateral cleft lip and palate repai


cleft palate. Only one patient returned for palaterepair e a 90% default rate.

Therefore, it seems clear that the palate should berepaired prior to the lip to improve the chance ofcompletion of surgery; besides, they should be repairedearlier to improve feeding or should be monitored toimprove their nutrition prior to surgery. As we do not havethe resources to monitor infants who present from acrossthe country and from neighbouring countries, we chose tooperate earlier.

In choosing to operate earlier, our main concern wassafety of the surgery. We previously had no mortality per-forming palate repairs on infants over 10 months of age.We, therefore, closely monitored infants in recovery and on

red at 3 months with a 3 month follow-up.


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the ward and chose to take down soft palates if there wasany evidence of pharyngeal obstruction. In this series,there has been neither any mortality, nor significantcomplications. The two patients taken back to the theatrewith low oxygen saturations were not severely compro-mised, but we preferred to err on the side of caution ratherthan risk an impending obstruction. It should be emphasisedthat anaesthetic safety is dependent on a high degree ofexpertise and skill from the anaesthetic staff and vigilanceto enable early detection of problems.

Performing palate repair on small infants is challengingand there is a learning curve. Initially, patients wereexcluded from the study if the palate looked too chal-lenging; however, with increasing experience, fewerpatients were excluded. Nine of the ten patients whounderwent anterior vomer flap rather than palate repairwere performed in the first cohort of 35 patients. In thesubsequent 72 patients, palate closure was successfullyperformed regardless of palate width in all but one patient(who had a cleft width of 17 mm, which is 2 mm wider thanthe widest repair). Undoubtedly, the ‘extended vomer flap’procedure has been of assistance in achieving closure at thedifficult area of junction of hard and soft palates and thisnew use of the vomer flap could be added to the list ofexisting uses for this flap.11

This study primarily aimed at assessing the safety of theprocedure, and we have demonstrated that our patients donot have significant problems postoperatively with thiscombined procedure. In our environment, it is more diffi-cult for us to demonstrate the long-term effects because ofthe difficulty in performing follow-up studies. We areobviously concerned about the effect on midfacial growth;however, we hope that very early palate repair and musclerepositioning may benefit speech development. In thisstudy, we have only reviewed 60% of patients post-operatively as patients who travel long distances are lesslikely to return for follow-up review.

Conclusion

Combined cleft lip and palate repair can be performedsafely and offer significant advantages, including


a reduction in mortality and completion of surgery in oneevent. Patients recover very quickly from this procedureand 92% of the patients thus far have not required furtherprocedures.

Acknowledgements

This study and the treatment of these patients have beenpossible due to funding by Smile Train.

Conflict of interest statement

None.

References

1. Sommerlad BC. A technique for cleft palate repair. PlastReconstr Surg 2003;112:1542e8.

2. Millard DR. Complete unilateral clefts of the lip. Plast ReconstrSurg 1960;25:595.

3. Millard DR. Extensions of the rotation-advancement principle forwide unilateral clefts of the lip. Plast Reconstr Surg 1968;42:535.

4. Tennison CW. The repair of the unilateral cleft lip by thestencil method. Plast Reconstr Surg 1995;2:175.

5. Noordhoff MS. The surgical technique for the unilateral cleftlip-nasal deformity. Taipei: Noordhoff Craniofacial Founda-tion; 1997.

6. Mulliken JB. Primary repair of bilateral cleft lip and nasaldeformity. Plast Reconstr Surg; 108:181e194.

7. Desai SN. Early cleft palate repair completed before the age of16 weeks; observation on a personal series of 100 children. Br JPlast Surg 1983;36:300e4.

8. De Mey A, Malevez C, Mansbach AL, et al. Prise en charge desfentes labio-maxillo-palatines a l’hopital des enfants ReineFabiola de Bruxelles. Ann Chir Plast Esthet 2002;47:134e7.

9. Wilson J, Hodges AM. Cleft lip and palate surgery at MengoHospital, Uganda: Where have all the palates gone? 2008:submitted for publication.

10. Derijcke A, Eerens A, Carels C. The incidence of oral clefts:a review. Br J Oral Maxillofac Surg 1996;34:488e94.

11. Agrawal K, Panda KN. Use of vomer flap in palatoplasty:revisited. Cleft palate-Craniofacial Journal. 2006;43:30e7.


combined early cleft lip and palate repair

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