|Year : 2019 | Volume
| Issue : 1 | Page : 82-86
Clinical and epidemiological profile of patients with cleft lip and palate anomaly: 10-year experience from a tertiary care center in the sub-himalayan state of Himachal Pradesh in Northern India
Vijay Kumar Diwana1, Gunjan Gupta1, Rajesh Chauhan1, Kunal Mahajan2, Aditi Mahajan1, Ranjan Gupta3, Kanika Mahajan3
1 Department of Plastic Surgery, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
2 Department of Cardiology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
3 Department of Prosthodontics, HP Government Dental College, Shimla, Himachal Pradesh, India
|Date of Web Publication||4-Feb-2019|
Department of Plastic Surgery, Indira Gandhi Medical College, Shimla - 171 001, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Background: There are no descriptive epidemiological data available from the state of Himachal Pradesh, India quantifying the problem of orofacial clefts. The present study aims at providing the baseline data from a tertiary care center in the state, on which future population studies can be conducted. Methods: We retrospectively analyzed the clinical and epidemiological data of 926 consecutive patients of oral clefts who underwent surgical repair at our institute over a 10-year period (2007–2016). Results: Out of a total of 926 patients, 507 (54.8%) were males and 419 (45.2%) were females with a male to female ratio of 1.2:1. Cleft lip with palate (CLP) was the most common type of orofacial cleft (486; 52.5%). It was more common among males (56.1% vs. 48.2%, P = 0.01) compared to cleft palate which was more common among females (21.5% vs. 32%, P < 0.002). Overall, unilateral clefts were more common than bilateral clefts (504 vs. 179, P < 0.0002), with left side being affected more commonly (324 vs. 180, P < 0.0002). Craniofacial anomalies were the most commonly reported associated defects. Only 231 (24.9%) patients were operated within the infancy period. The average age at the time of lip repair was 2.8 years, while for palatoplasty, it was 6 years. Conclusions: Orofacial clefts showed a male predominance, high unilateral:bilateral and left:right ratios, and CLP as the most common variety. Very few patients with cleft lip and palate underwent surgery at the recommended age. There is a need to strengthen the existing health-care infrastructure and knowledge to improve the scenario.
Keywords: Cleft lip, cleft palate, epidemiology, Himachal Pradesh
|How to cite this article:|
Diwana VK, Gupta G, Chauhan R, Mahajan K, Mahajan A, Gupta R, Mahajan K. Clinical and epidemiological profile of patients with cleft lip and palate anomaly: 10-year experience from a tertiary care center in the sub-himalayan state of Himachal Pradesh in Northern India. J Nat Sc Biol Med 2019;10:82-6
|How to cite this URL:|
Diwana VK, Gupta G, Chauhan R, Mahajan K, Mahajan A, Gupta R, Mahajan K. Clinical and epidemiological profile of patients with cleft lip and palate anomaly: 10-year experience from a tertiary care center in the sub-himalayan state of Himachal Pradesh in Northern India. J Nat Sc Biol Med [serial online] 2019 [cited 2019 Dec 10];10:82-6. Available from: http://www.jnsbm.org/text.asp?2019/10/1/82/251503
| Introduction|| |
Orofacial clefting (OFC) is the most common congenital anomaly of head and neck region. It is characterized by incomplete formation of structures separating oral and nasal cavity. The main categories are cleft lip (CL), cleft palate (CP), and cleft lip with palate (CLP). It is associated with a range of functional and esthetic problems which include impairment of speech, hearing, and dental development. These problems compound to social stigma and negatively affect the psychosocial life of affected individuals. OFC has a major impact on public health due to its complex etiology, lifelong morbidity, and multidisciplinary commitment required for treatment. Early contact of cleft team with affected individual ensures good functional and esthetic outcome. The incidence and geographic distribution of oral clefts vary widely across the world. This can be attributed to the difference in birth prevalence and underreporting of births as well as birth defects in the developing countries. The birth prevalence rate of infants born with craniofacial anomalies is 1.10/1000 births, which means 3 children with cleft are born every hour. A total of 26,950 new cases are reported from India every year., Inequalities exist both in the access and the quality of cleft care with distinct differences in urban versus rural access. Over the years, a backlog of untreated patients has accumulated making it a significant health-care problem in India. Further, in rural areas, such congenital malformations are being associated with number of myths such as “will of God” and “staring at solar eclipse during pregnancy,” leading to purposeful neglect and delay in treatment. The condition is expected to be even worse in mountainous region of North India. This is not only due to lack of health-care centers but also due to superstitions, unawareness, and hilly terrains which make traveling tough.
There are no descriptive epidemiological studies from the state of Himachal Pradesh that have addressed the problem of CL and palate. With the aim of bridging this knowledge gap, we retrospectively analyzed the clinical and epidemiological profile of CL and palate patients operated in a tertiary care center in the rural state of Himachal Pradesh in northern India. This is the only tertiary care center proficient in managing cleft patients in the whole state. Our study results are likely to reflect the status of the cleft care in the state.
| Methods|| |
This was a single-center retrospective observational study, conducted in the Department of Plastic Surgery, in a tertiary care hospital in the sub-Himalayan state of Himachal Pradesh in Northern India.
In the present study, we have retrospectively analyzed the data of all the patients with OFC who underwent surgery in our institute over a 10-year period (2007–2016), under Smile Train program.
The data related to sociodemographic profile and clinical features were collected from the electronic questionnaires completed by surgeons and health-care providers using Smile Train Express, an online medical record database. Parameters such as the age of presentation, sex, awareness about surgery, residence, diagnosis, type of surgery, and associated anomalies were studied. These data sets were then entered into Excel (Microsoft, Raymond, WA, USA).
Classification of orofacial clefts
Cleft types were classified according to the International Classification of Diseases-9 diagnosis codes, which utilize three general groups of CL, CLP, and CP. These were further categorized by laterality.
The descriptive statistics of the study population was reported as counts for categorical variables. The gender-based distribution of clinical characteristics was compared using Chi-square test. The data analysis was done using STATA 13 statistical software (StataCorp, College Station, TX, USA). Statistical significance was defined as two-sided P < 0.05.
| Results|| |
A total of 926 patients underwent cleft surgery over a 10-year period (2007–2016), of which 507 (54.8%) were male and 419 (45.2%) were female with a male to female ratio of 1.2:1. There were two plastic surgeons for the initial 3 years, but only one for the remaining 7 years. The most common type of OFC observed was CLP (486; 52.5%) followed by CP (243; 26.2%) and CL (197; 21.3%). CLP was more common among males (56.1% vs. 48.2%, P = 0.01) while CP was more common among females (21.5% vs. 32%, P < 0.002) [Table 1]. CL was also more common in males, but this result was not statistically significant [Table 1]. On further breakdown for the types of clefts, we observed that the most common type was isolated CP (24.4%) followed in frequency by left-sided CL with palate (22.5%), bilateral CL with palate (16.9%), right-sided CL with palate (12.9%), left-sided CL only (12.4%), right-sided CL only (6.5%), bilateral CL only (2.4%), and submucous CP (1.8%) [Table 2]. Overall, unilateral clefts were more common than bilateral clefts (504 vs. 179, P < 0.0002), with left side being affected more commonly (324 vs. 180, P < 0.0002).
|Table 2: Subdivisions of orofacial clefts according to the site and side involved|
Click here to view
It was observed that only 49 (5.3%) patients had family history of OFC, of which 23 (2.5%) had history among immediate relatives, while 26 (2.8%) had history among distant relatives. Abnormal speech was observed in 666 (72%) of patients. Patients were assessed for abnormalities in the other organ systems, and it was found that craniofacial anomalies were the most common followed by abnormalities of extremities and cardiovascular system [Table 3].
Overall, 231 (24.9%) were operated within the infancy period. Out of these 231 patients, only 8 patients underwent primary palatoplasty, while the remaining 223 patients underwent primary lip repair [Table 4]. Most of these infants (184, 19.9%) were operated within the age group of 5–8 months.
There was a wide range of variation in the age at the time of surgery. This ranged from a 26-day-old neonate to a 40-year-old middle-aged adult. However, the average age for lip repair was 2.8 years, while for the palatoplasty, it was 6 years. The year-wise trend in the average age at the time of each surgery was also recorded [Table 5].
Overall, 166 (18%) patients underwent secondary surgeries in the form of lip nose revision (71), fistula repair (84), and alveolar bone graft (11).
| Discussion|| |
The prevalence and clinical-epidemiological profile of patients with OFC vary in different populations around the world, as a result of substantial variability related to geographic origin, ethnicity, and socioeconomic conditions. The present study highlights the clinical and epidemiological profile of OFC patients attending a tertiary care institute in the state of Himachal Pradesh, India. As already stated, this is the only center in the state which is proficient in cleft care. Furthermore, since 90% of state population lives in rural areas, the present data are likely to represent trends of OFC in a predominantly rural population. Knowledge of such trends is important since two-thirds of Indian population live in rural areas, and taboos associated with these disorders are also more likely among populations with rural background. However, larger prospective studies are needed from other parts of country to make any generalization.
In our study, male to female ratio among oral cleft patients was 1.2:1. This was lesser when compared to the studies conducted in other parts of India., Dvivedi and Dvivedi observed a male to female ratio of 2:1 in a large prospective study among 4657 cleft patients in the state of Uttarakhand. Similarly, in a study of 411 cleft patients from Punjab, this ratio was 1.5:1. India is a country where female feticide is still prevalent. Moreover, many female cleft patients might not be brought to the hospital. For these reasons, difference in sex ratio is expected among various Indian states depending on their social, educational, and developmental indices. However, further research is warranted to identify the possible mechanisms behind this sex disparity.
The breakdown for the types of clefts varies between studies and regions but is approximately 45% CLP, 35% CL, and 20% CP. Our study also demonstrated CLP to be the predominant type of OFC and a high male to female sex ratio among CPL (1.4:1) and CL patients (1.3:1). In contrast, CP was more frequent in females with a high female: male ratio (1.2:1). The male predilection for CL and/or palate and female tendency toward CP is well known, indicating a different etiology. This gender difference is likely attributed to longer period of time for palatal closure in female embryos that essentially increases the time during which they may remain exposed to the palatogenic teratogens.
It was found that unilateral clefts were more common than bilateral [Table 2]. Furthermore, bilateral clefts were more prevalent among male patients almost twice to that in females. Both these findings were homogeneous with a descriptive study of clefts in a Nigerian institute by Ibrahim et al. Consistent with the results of another study by Blanco Davila, our study also demonstrated left-sided clefts to be more common than right-sided clefts. Prior research has not yielded any convincing explanation for these differences in the sidedness. It has been hypothesized that the blood vessels, supplying the right side of the fetal head, leave the aortic arch closer to the heart and perhaps provide better blood perfusion in comparison to those supplying the left side.
CL and palate patients often have associated congenital defects. The prevalence of associated anomalies and the organ systems most commonly affected vary among different studies. This is because of the differences in the methodology and the investigations performed to identify associated disorders. In the present study, craniofacial malformations were most commonly observed followed by limbs, cardiovascular, and urinary system. It is still not clear whether the association between clefts and other anomalies is genetically determined or coincidental. Larger context-specific studies would be required to identify such associations.
There was a wide range of variation in the age at the time of surgery with youngest case operated in the neonatal period and oldest at the age of 40 years. Majority of Indian surgeons prefer to repair lip before 6 months and palate by 6–18 months of age. However, maximum patients in our study were operated far past the optimal age of surgery with only one-fourth of the patients operated before 1 year of age. The average age at surgery for lip and palate in our study was 2.8 and 6 years, respectively. The delay for surgery could be due to many potential reasons. First, there could be inability to take timely medical consultation probably because of hilly terrain and inappropriate weather conditions in our state. Second, there is no other health-care center (government or private) in the state which deals with cleft surgeries, other than our institute. Furthermore, there is only one plastic surgeon proficient to operate cleft patients in our institute for the last 7 years, resulting in longer waiting lists and larger backlog. Third, the role of poverty, superstitions, and ignorance cannot be underestimated as our findings are consistent with those in the other developing countries, with lack of socioeconomic growth.
In a status report on the management of CL and palate patients in India, published in 2010, there was a consensus regarding the age of unilateral CL repair before 6 months. A significant number of cleft surgeons however believed in operating children with bilateral cleft after 6 months of age since they are more likely to have poor nutritional status, low body weight, and nutritional anemia. Majority of the surgeons agreed at performing palatoplasty between 6 and 18 months. However, as stated previously, the average age at surgery for all types of cleft was unacceptably high in our study [Table 5]. Furthermore, there has not been much decline in the average age at the time of surgery in past one decade for CP and unilateral CL [Table 5]. In contrast, average age at bilateral primary lip repair has shown a decline, and since 2013, average age of repair has been <0.7 years (8 months). The explanation for this difference is not clear. One hypothesis is that bilateral cleft is functionally and esthetically worse for the patients and their parents, which causes the parents to seek medical attention early.
Limitations of the study
No study is without limitations and so is ours. Being a single-center observational study, it is inherent to have referral and selection bias. The true demographic picture of the region cannot be represented by a hospital-based study like this. Although there is no other center in the state where cleft patients are referred/treated, the possibility of a significant number of patients undergoing treatment in nearby states cannot be underestimated. Second, the sample size of the study was small despite being a 10-year study. This is presumably because of the meager population of Himachal Pradesh. Further larger and longer studies are therefore warranted. The other limitations include retrospective nature of the study, lack of integrated management program, and loss to follow-up. Unlike other surgical conditions, management goal of oral cleft extends far beyond the surgical repair. This includes functional gain, esthetic outcome, and health-related quality of life. These outcomes could not be measured in the present study. The patients' and parents' perspective is a key component in deciding the treatment outcome and should ideally be included in such studies.
| Conclusions|| |
The present study provides the clinical and epidemiological data of patients presenting with orofacial clefts to a tertiary care center in the small Indian state of Himachal Pradesh. This is the first such study from this region. It was observed that very few patients undergo cleft surgery at recommended ages. This is possibly attributed to lack of awareness, workforce, and health-care initiative. Government needs to increase the availability of health-care centers and surgeons capable of performing cleft surgeries in the state. Nevertheless, such studies are bound to provide the baseline data on which the future research can be conducted, and cleft-care improvement outcomes can be measured.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
The surgeries were done with funding from Smile Train, an international children's charity organization. The organization had no role in analysis, interpretation and publication of this data. This retrospective study did not receive any specific grant from any other funding agency in the public, commercial, or not-for-profit sectors.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Eshete M, Butali A, Deressa W, Pagan-Rivera K, Hailu T, Abate F, et al.
Descriptive epidemiology of orofacial clefts in Ethiopia. J Craniofac Surg 2017;28:334-7.
Mossey PA, Modell B. Epidemiology of oral clefts 2012: An international perspective. Front Oral Biol 2012;16:1-8.
Mossey P, Little J. Addressing the challenges of cleft lip and palate research in India. Indian J Plast Surg 2009;42Suppl:S9-18.
Van Lierde KM, Dhaeseleer E, Luyten A, Van De Woestijne K, Vermeersch H, Roche N, et al.
Parent and child ratings of satisfaction with speech and facial appearance in flemish pre-pubescent boys and girls with unilateral cleft lip and palate. Int J Oral Maxillofac Surg 2012;41:192-9.
Mossey PA, Little J, Munger RG, Dixon MJ, Shaw WC. Cleft lip and palate. Lancet 2009;374:1773-85.
Dvivedi J, Dvivedi S. A clinical and demographic profile of the cleft lip and palate in Sub-Himalayan India: A hospital-based study. Indian J Plast Surg 2012;45:115-20.
] [Full text]
Moghe G, Mauli S, Thomas A, Obed VA. Bridging the gap: Addressing challenges toward improvement of cleft teamwork in a tertiary care center in North India: A pilot study. Cleft Palate Craniofac J 2013;50:473-80.
Conway JC, Taub PJ, Kling R, Oberoi K, Doucette J, Jabs EW, et al.
Ten-year experience of more than 35,000 orofacial clefts in Africa. BMC Pediatr 2015;15:8.
Mahajan K, Negi PC, Merwaha R, Mahajan N, Chauhan V, Asotra S, et al.
Gender differences in the management of acute coronary syndrome patients: One year results from HPIAR (HP-India ACS registry). Int J Cardiol 2017;248:1-6.
Uppal SK, Shah S, Mittal RK, Garg R, Gupta A. Epidemiology and clinical profile of cleft lip and palate patients, in a tertiary institute in Punjab, India: A preliminary study. J Cleft Lip Palate Craniofac Anom 2016;3:32-5.
Tabaie S. Stopping female feticide in India: The failure and unintended consequence of ultrasound restriction. J Glob Health 2017;7:010304.
Ibrahim A, Mshelbwala PM, Obiadazie AC, Ononiwu CN, Asuku ME, Ajike SO, et al
. A descriptive study of clefts of the primary and secondary palate seen in a tertiary institution in Nigeria. Niger J Surg Res 2013;15:7-12. [Full text]
Blanco-Davila F. Incidence of cleft lip and palate in the Northeast of Mexico: A 10-year study. J Craniofac Surg 2003;14:533-7.
Gopalakrishna A, Agrawal K. A status report on management of cleft lip and palate in India. Indian J Plast Surg 2010;43:66-75.
] [Full text]
Roldán JC, Pape HD, Koch H, Koller M. Ten-year cleft surgery in Nepal: Achievements and lessons learned for better cleft care abroad. Plast Reconstr Surg Glob Open 2016;4:e711.
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]