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Table of Contents
ORIGINAL ARTICLE
Year : 2021  |  Volume : 29  |  Issue : 5  |  Page : 14-16

Complicated patients with cleft lip and palate: Our experience with multidisciplinary approach


Department of Plastic, Reconstructive and Aesthetic Surgery, Cerrahpasa Medical Faculty, Istanbul University–Cerrahpasa, Fatih, Istanbul, Turkey

Date of Submission10-Jul-2020
Date of Acceptance27-Oct-2020
Date of Web Publication17-Mar-2021

Correspondence Address:
Dr. Ugur Celik
Cerrahpasa Medical Faculty, Istanbul University, Fatih, Istanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjps.tjps_77_20

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  Abstract 


Background: Cleft lip and palate is one of the most commonly seen congenital deformities. The aim of treatment should be providing normal speech, normal nutrition, normal appearance, and adaptation of the child in society. Thus, the importance of multidisciplinary approach in this patient group is high. In this study, we aim to present the patient results obtained from cleft lip and palate meeting records, with the attendance of plastic surgeon, ear, nose, throat surgeon, orthodontist, speech therapist, pedodontist, nutrition nurse and pediatrician, and genetics specialist when needed. Materials and Methods: Patient records of cleft lip and palate meeting held between September 1998 and September 2018 were evaluated retrospectively. The patients regarded as complex cases were examined in these meetings. Results: Seven hundred and five patients were regarded as complex cases to be examined at the cleft lip and palate meetings in this 20-year period. One hundred and five of these patients (15%) had the history of being inbred. Ninety-eight patients (14%) had additional anomaly of other systems. Thirty-six patients (5%) suffered from the cleft of lip and primary palate only. Cleft palate patient numbers for Veau Class 1, 2, 3, and 4 was 12 (2%), 99 (14%), 297 (42%), and 246 (35%), respectively. Conclusions: Creating multidisciplinary teams consisted of specialists from various fields is an accepted method for handling of this patient group. These multidisciplinary teams not only provide the patients to have care by professionals from different specialties at once but also have the benefits of data unification.

Keywords: Cleft lip, cleft palate, congenital anomalies, congenital clefts, multidisciplinary teams


How to cite this article:
Demiroz A, Celik U, Yalcin CE, Aydin Y. Complicated patients with cleft lip and palate: Our experience with multidisciplinary approach. Turk J Plast Surg 2021;29, Suppl S1:14-6

How to cite this URL:
Demiroz A, Celik U, Yalcin CE, Aydin Y. Complicated patients with cleft lip and palate: Our experience with multidisciplinary approach. Turk J Plast Surg [serial online] 2021 [cited 2022 Dec 8];29, Suppl S1:14-6. Available from: http://www.turkjplastsurg.org/text.asp?2021/29/5/14/311436




  Introduction Top


Cleft lip and palate is one of the most common congenital conditions.[1],[2] Patients with cleft lip and palate face many psychological, social, and medical problems, with the latter including difficulty feeding and swallowing, hearing, chronic ear infections, and speech problems. Evaluation starts during the prenatal period and continues until the definitive surgical procedures are complete. Moreover, not only because of the sequelae of the primary condition, it is important to keep in mind that other anomalies of different organ systems, such as musculoskeletal and cardiovascular, may accompany the clefts of the lip and palate. Therefore, it is important for every clinic to have an environment that can provide the multidisciplinary approach that can respond to these patients with various possible needs. The teams involved in the care of the patients with cleft lip and/or palate typically include plastic surgeons and otolaryngologists, orthodontists, speech therapists, pedondontists, nutrition nurses, pediatricians, and genetics specialists.[3]

In this study, we aimed to present our data of a large cohort collected between September 1998 and 2018 in order to demonstrate important and challenging aspects of management of patients with cleft lip and/or palate and provide our single-center experience.


  Materials and Methods Top


Data were extracted retrospectively from the records that were taken during the monthly cleft lip and palate meetings between September 1998 and September 2018. At our clinic, these monthly meetings for patients with cleft lip and palate are organized in order to achieve a multidisciplinary approach for the complex cases. Complex cases are the ones who are referred to our clinic from their physicians involved in their primary care because of challenges in treatment options. Demographical and medical information of these patients including age, sex, family history, consanguinity between the parents, education level of the parents, sibling count, maternal birth history, smoking history, maternal medication-drug use during pregnancy, timing and method of delivery, pre- and perinatal history of the child, presence of accompanying anomalies, previous cleft lip and palate related operation and interventions, history of orthodontics, plastic surgery, pedondontics, ear nose throat, speech therapy, and genetics interventions and visits if applicable are recorded separately on an individual software created just for cleft lip and palate patients.


  Results Top


Seven hundred and five cases were considered complex and were included in the study [Table 1]. Of these cases, 48% were male. Family history was positive for consanguinity in 15% of patients. There were other accompanying anomalies for 14% of the patients, with most of them having cardiac anomalies [Table 2]. Other common anomalies were extremity related, including mostly pes equinovarus and cranial, such as trigonocephaly. Fifteen patients (2%) presented with several types of face clefts. About 36 patients (5%) suffered from the cleft of lip and primary palate only. Of the 669 cases with cleft palate, the percentage of Veau Class I, II, III, and IV were 2, 14, 42, and 35%, respectively. Hypernasality was observed in 381 of 669 patients with cleft palate and physical examination revealed fistulas for 231 of these patients. The nasal deformity was present in 480 of 594 (80%) patients with cleft lip.
Table 1: Demographical information of the patients included in our study in addition to the frequencies of accompanying findings

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Table 2: Distribution of accompanying anomalies in our series of patients with cleft lip and/or palate

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  Discussion Top


The management of patients with cleft lip and palate requires a team approach which has to be initiated even from the prenatal period[4] and has to continue until early adulthood.[5] Multidisciplinary approach and the teams that should be included during assessment and treatment of patients with cleft lip and palate were previously described by the American Cleft Palate-Craniofacial Association and ideally, the teams should be composed of geneticists, speech therapists, audiologists, dentists, nurses, nutritionists, psychologists, orthodontists, pediatricians, otolaryngologists, psychologists, social workers, and plastic surgeons.[3] Not only after birth, Han et al. reported the benefits of a team approach to the parents of prenatally diagnosed patients with cleft lip and palate. In their research, they concluded that a team of plastic and reconstructive surgeons, psychiatrists, obstetricians, and pediatricians can provide psychological and medical support to the parents so that they can feel prepared for the management of this condition after birth.[4] The principle of multidisciplinary approach to these patients has been spreading around the world and with the developments in technology and the Internet, patients with cleft lip and palate born in underserved areas of the world can also receive the care they need.[6] Our meetings have been regularly performed for the past 20 years and the patients are evaluated simultaneously by plastic surgeons, otolaryngologists, speech therapists, feeding nurses, and orthodontists. As a reference center, the patients who were evaluated during our meetings consisted of adults, children, and newborns as well.

In a study from Brazil by Aquino et al., the rate of consanguinity among parents of patients with cleft lip and/or palate was reported to be approximately 6%. In our study, 15% of the patients had consanguineous parents, which is higher.[7] Consanguinity is not only a risk factor for cleft lip and palate development but also a risk factor for genetic malformations. The higher prevalence of consanguinity among our patients may be one of the reasons behind the complexity of their condition.

Cleft lip and palate may be accompanied by other congenital anomalies. Cardiac and skeletal anomalies were reported to be one of the most common types, which is supported by our study as well.[8] Another study by Altunhan et al. also reviewed the congenital anomalies associated with cleft lip and palate and concluded that cardiac anomalies are the most prevalent.[9]

Veau classification is a commonly used classification currently being used by many centers to stratify the severity and extent of the clefting among these patients. In a retrospective study, reviewing the rate of fistula and velopharyngeal insufficiency after primary cleft palate repair, distribution between Veau Classes I, II, III, and IV were 21, 30, 32, and 18% respectively.[10] In our study, Class III is the most common as well, however the number of patients with Class IV cleft lip and palate is significantly higher, making it the second most common. The difference between the distribution of Veau classification of our patients is expected since our cases include a subgroup of the general population with complex features that required referral to our center.

Hypernasality and velopharyngeal insufficiency after repair of the cleft lip may occur in 5%–38% of the patients.[11],[12] One of the other concerning complications following cleft palate repair is fistula development, which occurs in approximately 4.5%–4.9% of the patients.[5],[10],[13] More than half of our patients demonstrate features of velopharyngeal insufficiency and hypernasality. In addition, the number of our patients with oronasal fistula is nine times higher, and comparing to the previous evidence, our patients are a rather complex subset of the general population with a higher prevalence of more severe disease classification and higher rates of complications. Since hypernasality and other speech problems are relatively common among our patients, in order to determine whether they are caused either by velopharyngeal insufficiency or inadequate learning speech therapists are an indispensable part of our team.

Limitations of our study include its retrospective nature and relatively subjective criteria of inclusion or exclusion. Exclusion of relatively simple cases and predominant inclusion of complex cases may over – or underestimate some parameters regarding patients with cleft lip and palate.


  Conclusions Top


Children with cleft lip and palate have many problems to be solved in adulthood, adolescence, childhood, and even during the prenatal period. Precise planning of treatment and follow-up is extremely important. Therefore, creating multidisciplinary teams consisted of specialists from various fields is an accepted method for handling of this patient group. This team should include (1) dental specialists (orthodontist, pedodontist, etc.), (2) medical specialists (plastic surgeon, ear, nose, throat surgeon, pediatrician, etc.), and (3) allied health-care workers such as speech therapists and nutrition nurses. These multidisciplinary teams not only provide the patients a qualified care from different specialties at once but also have the benefits of data unification.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mossey PA, Little J, Munger RG, Dixon MJ, Shaw WC. Cleft lip and palate. Lancet 2009;374:1773-85.  Back to cited text no. 1
    
2.
Tanaka SA, Mahabir RC, Jupiter DC, Menezes JM. Updating the epidemiology of cleft lip with or without cleft palate. Plast Reconstr Surg 2012;129:511e-8.  Back to cited text no. 2
    
3.
Tollefson T, Sykes J. Unilateral cleft lip. Complete Cleft Care. New York, NY: Thieme; 2015. p. 37-62.  Back to cited text no. 3
    
4.
Han HH, Choi EJ, Kim JM, Shin JC, Rhie JW. The importance of multidisciplinary management during prenatal care for cleft lip and palate. Arch Plast Surg 2016;43:153-9.  Back to cited text no. 4
    
5.
Shaye D, Liu CC, Tollefson TT. Cleft lip and palate: An evidence-based review. Facial Plast Surg Clin North Am 2015;23:357-72.  Back to cited text no. 5
    
6.
Furr MC, Larkin E, Blakeley R, Albert TW, Tsugawa L, Weber SM. Extending multidisciplinary management of cleft palate to the developing world. J Oral Maxillofac Surg 2011;69:237-41.  Back to cited text no. 6
    
7.
Aquino SN, Paranaíba LM, Martelli DR, Swerts MS, Barros LM, Bonan PR, et al. Study of patients with cleft lip and palate with consanguineous parents. Braz J Otorhinolaryngol 2011;77:19-23.  Back to cited text no. 7
    
8.
Shafi T, Khan MR, Atiq M. Congenital heart disease and associated malformations in children with cleft lip and palate in Pakistan. Br J Plast Surg 2003;56:106-9.  Back to cited text no. 8
    
9.
Altunhan H, Annagür A, Konak M, Ertuğrul S, Ors R, Koç H. The incidence of congenital anomalies associated with cleft palate/cleft lip and palate in neonates in the Konya region, Turkey. Br J Oral Maxillofac Surg 2012;50:541-4.  Back to cited text no. 9
    
10.
Yuan N, Dorafshar AH, Follmar KE, Pendleton C, Ferguson K, Redett RJ 3rd. Effects of cleft width and veau type on incidence of palatal fistula and velopharyngeal insufficiency after cleft palate repair. Ann Plast Surg 2016;76:406-10.  Back to cited text no. 10
    
11.
Witt PD, D'Antonio LL. Velopharyngeal insufficiency and secondary palatal management. A new look at an old problem. Clin Plast Surg 1993;20:707-21.  Back to cited text no. 11
    
12.
Fisher DM, Sommerlad BC. Cleft lip, cleft palate, and velopharyngeal insufficiency. Plast Reconstr Surg 2011;128:342e-60.  Back to cited text no. 12
    
13.
Bykowski MR, Naran S, Winger DG, Losee JE. The rate of oronasal fistula following primary cleft palate surgery: A meta-analysis. Cleft Palate Craniofac J 2015;52:e81-7.  Back to cited text no. 13
    



 
 
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