|Year : 2020 | Volume
| Issue : 3 | Page : 237-241
Pattern of congenital hand anomalies at a tertiary plastic surgery service in South-Western Nigeria: A 10-year, cross-sectional retrospective review
Afieharo Igbibia Michael, Samuel Adesina Ademola, Olayinka Adebanji Olawoye, Ayodele Olukayode Iyun, Odunayo Moronfoluwa Oluwatosin
Department of Surgery, Faculty of Clinical Sciences, College of Medicine, University of Ibadan; Department of Plastic, Reconstructive and Aesthetic Surgery, University College Hospital, Ibadan, Nigeria
|Date of Submission||15-Apr-2020|
|Date of Decision||05-Jun-2020|
|Date of Acceptance||09-Jun-2020|
|Date of Web Publication||17-Jul-2020|
Dr. Afieharo Igbibia Michael
Department of Surgery, Faculty of Clinical Sciences, College of Medicine, University of Ibadan, Ibadan
Source of Support: None, Conflict of Interest: None
Context: Although congenital hand anomalies are among the more common musculoskeletal anomalies worldwide, we do not know its prevalence in our practice. Aims: The aim of the study was to determine the pattern of congenital hand anomalies presenting to our tertiary plastic surgery outpatient service in South-Western Nigeria. Materials and Methods: This is a cross-sectional retrospective analysis of outpatient cases of congenital hand anomalies presenting over a 10-year period. Descriptive and inferential statistics were performed using frequencies, Student's t-test and Chi-square as appropriate. The data were analysed using IBM SPSS Statistics 23.0. The statistical significance value was set at P < 0.05. Results: One hundred and twenty-two cases were identified. The highest number of cases was seen in the year 2012, 23 patients (19.3%). There was a male preponderance of 66 patients (55.1%). Thirty-two patients (26.2%) presented as neonates and 36 (29.5%) as infants. Bilateral anomalies were seen in 67 patients (54.9%). The most common anomaly was failure of differentiation, 88 patients (72.1%) followed by duplication, 26 patients (21.3%). Syndactyly with 43 patients (35.2%) was the most common anomaly under failure of differentiation. No significant associations were found between the type of anomaly and gender or laterality. Conclusions: Syndactyly was the most common congenital hand anomaly in this study. There was a preponderance of bilateral involvement in both syndactyly and polydactyly.
Keywords: Africa, anomalies, congenital, hand
|How to cite this article:|
Michael AI, Ademola SA, Olawoye OA, Iyun AO, Oluwatosin OM. Pattern of congenital hand anomalies at a tertiary plastic surgery service in South-Western Nigeria: A 10-year, cross-sectional retrospective review. Niger Postgrad Med J 2020;27:237-41
|How to cite this URL:|
Michael AI, Ademola SA, Olawoye OA, Iyun AO, Oluwatosin OM. Pattern of congenital hand anomalies at a tertiary plastic surgery service in South-Western Nigeria: A 10-year, cross-sectional retrospective review. Niger Postgrad Med J [serial online] 2020 [cited 2020 Oct 28];27:237-41. Available from: https://www.npmj.org/text.asp?2020/27/3/237/289920
| Introduction|| |
Incidence and prevalence studies on congenital anomalies provide valuable data for monitoring the occurrence of these anomalies, planning of health services and, additionally, fosters research., While some hospital-based studies emanating from Nigeria have identified polydactyly as the most common congenital hand anomaly, others have reported a higher prevalence of syndactyly., Polydactyly are, however, more commonly reported in hospital-based surveillance of birth defects.,
Due to the absence of a national birth defect registry, the country's incidence of congenital hand anomaly is unknown. In Finland, Koskimies et al. reported a national birth incidence of congenital upper limb deficiencies of 5.25/10,000 live births. The incidence of congenital upper limb anomalies was reported to be 1 in 626 live births from a clinic-based study by Bowe and Conway in New York. Based on clinic surveys obtained from Japan, the USA and the United Kingdom, Lamb et al. analysed the population frequency of congenital malformations of the upper limb to be 10.9/10,000 live births. The prevalence of congenital upper limb anomalies was 1 in 506 live births in a study from Australia. These data were obtained from the Western Australian Birth Defects Registry. Birth defect registries in Nigeria are overdue.
The aetiological factors of congenital anomalies of the hand could be genetic, environmental or a combination of both factors. Some of these environmental factors include maternal exposure to thalidomide, alcohol, cyclophosphamide and rubella, particularly in the first trimester of pregnancy.,
Congenital hand anomalies could occur as isolated defects, in combination with anomalies in other regions of the body or as a component of syndromes, for example, Poland syndrome and Holt–Oram syndrome.,, Swanson classified congenital hand anomalies into seven broad types as failure of formation of parts (arrest of development), failure of differentiation (separation) of parts, duplication, overgrowth, undergrowth, constriction band syndrome and generalised skeletal abnormalities. Based on this classification as adopted by the International Federation of Surgical Societies of the Hand, we report on the pattern of congenital hand anomalies in our plastic surgery tertiary care facility. It is our aim to contribute to the body of knowledge available in this area.
| Materials and Methods|| |
Study setting and design
The study was performed at the University College Hospital. It is a tertiary health facility in Southwest Nigeria. The plastic surgery department is responsible for managing deformities of the hand (excluding the wrist) in adults and children. We performed a cross-sectional retrospective review of all paediatric patients who presented to the plastic surgery outpatient department between August 2007 and July 2017 with congenital hand anomalies. This study was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendment.
Data and statistical analysis
Records of all congenital hand anomalies were obtained from the departmental registry. Additional missing information such as gender, laterality, age at presentation, maternal age and presence of other congenital anomalies were sought from the case notes. All obtained data were entered into IBM SPSS Software (Armonk, NY, USA; 2015; version 23). Relative frequencies of the anomalies, gender, laterality and year of presentation were analysed. Associations between the anomaly and demographic characteristics were also analysed with Chi-square and Fisher's exact test as appropriate. P < 0.05 was termed statistically significant.
| Results|| |
Nine hundred and twenty-five paediatric patients presented at the outpatient clinic within the study period. Of these, 122 records of congenital hand anomalies were obtained. The prevalence of congenital hand anomalies among paediatric outpatient presentations was 13.2%. Most of the patients presented as infants, 56 (45.9%). Next to this were those who presented in the neonatal period. Only three adults were seen [Table 1]. Most of these congenital anomalies were seen in the year 2012 as illustrated in [Figure 1].
|Table 1: Demographic characteristics of patients with congenital hand anomalies|
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The most common anomaly was failure of differentiation, 88 (72.2%). Next to this was duplication, 26 (21.3%). No patients with complex skeletal hand anomaly was seen [Figure 2]. Within the subgroup of failure of differentiation, patients who had syndactyly (43 [35.2%]) were the most common followed by camptodactyly (29 [23.8%]) [Figure 3]. Examples of the congenital hand anomalies seen are shown in [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d, [Figure 4]e, [Figure 4]f.
|Figure 4: (a) Failure of formation of parts (phocomelia). (b) Failure of differentiation (syndactyly). (c) Duplication (bilateral post-axial polydactyly). (d) Overgrowth. (e) Undergrowth. (f) Constriction band syndrome|
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Sex distribution and laterality of the anomalies
Overall, there was a slight male preponderance, 66 (54.1), of anomalies with a male-to-female ratio of 1.1:1. As illustrated in [Table 2], this relationship was more marked among patients who had failure of differentiation. However, in duplication deformities, there was a female preponderance. In addition the three patients who had constriction bands were females. The majority of these deformities were bilateral, 67 (54.9%). The subgroup analysis maintained this trend. All the three cases of congenital constriction band syndrome had bilateral involvement [Table 3].
Occurrence of other anomalies and association between variables
The occurrence of anomalies in other regions of the body was seen in 27 (22.5%) patients and the most common region involved was the lower limb. This presence of other anomalies bore no relationship with the nature of the anomaly (P = 0.66). No syndromes were identified. When the deformities were grouped as either unilateral or bilateral deformities, the odds of a child developing these deformities on either side was not associated with the gender (odds ratio = 1.03, 95% confidence interval = 0.51–2.11). There was also no significant association between the gender and the nature of anomaly (P = 0.05). The laterality of the anomaly bore no significant relationship with the nature of the anomaly (P = 0.06).
| Discussion|| |
We sought to determine the pattern of congenital hand anomalies in our tertiary specialist practice, and we found that the prevalence of congenital hand anomalies was approximately 13% of our paediatric plastic surgery outpatient population. There were slightly more males; syndactyly was the most common anomaly and bilateral deformities were more prevalent than unilateral deformities. Although the prevalence of congenital anomalies is better obtained from birth registries and population studies, hospital prevalence can provide useful information on the occurrence of these deformities and foster more surveillance of these deformities.
Our finding of a higher number of syndactyly is similar to a study by Edomwonyi et al. from South-South Nigeria. Their hospital-based 10-year retrospective review of congenital musculoskeletal anomalies included 75 patients with hand-and-foot anomalies. Interestingly, they had no record of polydactyly. A similar hospital-based study from South-East Nigeria found a higher number of syndactyly followed by polydactyly, while Abulezz et al. in Egypt found more polydactyly than syndactyly in their plastic surgery outpatient service. This difference was however slight. A more recent prospective study in Nigeria however reported polydactyly as the most common digital anomaly seen. They recruited patients from paediatric, burns and plastic and postnatal clinics of two hospitals over a 5-year period. It is possible that inclusion of the postnatal clinic as a recruitment site contributed to these findings. Patients with polydactyly amenable to ligation do not present for specialist care., Authors from Kenya have also reported polydactyly as the most common anomaly from their hospital-based surveys, which included the maternity unit. Population-based studies from Australia and Sweden have reported failure of differentiation as the most common anomaly.,
The slight predominance of males in our study of 54.1% is similar to the study by Ekblom et al. They recorded a male predominance of 54%. Edomwonyi et al. and Ekemenye et al. both from southern Nigeria reported a higher male preponderance of 2:1 and 1.5:1, respectively., Giele et al. reported a male-to-female ratio of 3:2. This male predominance in these studies bore no relationship with the classification of the deformity as also seen in our study. There was however a female preponderance of 59.3% in the study by Abulezz et al. from Egypt.
Most of the deformities in this study occurred bilaterally, similar to the previous findings.,,, Bilateral involvement was reported to be seen in more generalised anomalies. However, in a study by Dao et al., there was an equal distribution of both unilateral and bilateral congenital hand anomalies. A quarter of our patients had other associated congenital anomalies, with the feet being more commonly affected, though no syndromes were identified. Goldfarb et al. found approximately a fifth of the patients in their population having congenital hand anomalies in association with syndromes. In Giele et al.'s study in West Australia, nearly half of the patients with congenital hand anomalies had anomalies in other parts of the body. They have a robust birth defects registry in their country that records all major and minor birth defects.
Country wise incidence of congenital upper limb malformations in Africa is unreported. Although a National Congenital Heart Defects Registry exists in Nigeria, no robust national or state birth defect registry exists. Robust birth defect registries such as the European Surveillance of Congenital Anomalies document birth defect from 39 registries in 21 countries in Europe. Their objectives include to provide essential epidemiologic information on congenital anomalies, to facilitate early warning of new teratogenic exposures and to facilitate the effectiveness of primary prevention. Another robust birth defect registry is the International Clearinghouse for Birth Defect Surveillance and Research (ICBDSR), a voluntary non-profit organisation affiliated with the World Health Organization, which has 42 member programs worldwide. It is noteworthy that these ICBDSR programmes have contributions from all the continents of the world except Africa.
With the absence of such registries in our setting, these anomalies are only recorded at presentation for specialist consultation. Over a quarter of the patients in this study presented within the neonatal age group. The majority of our patients however presented within 1 year of age. Mbaet al. also reported late presentation of these anomalies. Only 19% were seen in the neonatal period. They also had a much higher number of adult patients than that in this study. The mean age of presentation in the study from Egypt was 6 years. We found the highest number of patients presented in the year 2012. This retrospective finding is unable to determine if this increase was due to increased exposure to teratogens or a chance finding. This underscores the need for defect surveillance to determine any possible cause of increased birth defects within a population.
Due to the retrospective nature of the study, missing information in the data such as maternal age and exposure to teratogens in the antenatal period could not be obtained. The regional distribution of these patients would also have been important in determining the presence of clusters of congenital hand anomalies.
| Conclusions|| |
We have been able to report that syndactyly was the most common congenital hand anomaly presenting to our plastic surgery outpatient service. Bilateral involvement was more common, and the slight male preponderance bore no relationship with the nature of the deformity. Development of a standard pro forma for adequate documentation of congenital hand anomalies and subsequent prospective surveys would be helpful going forward.
We thank Dr Chidinma Akpa and Mrs Omolara Owujuyigbe for their assistance with data retrieval.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Koskimies E, Lindfors N, Gissler M, Peltonen J, Nietosvaara Y. Congenital upper limb deficiencies and associated malformations in Finland: A population-based study. J Hand Surg Am 2011;36:1058-65.
Chung KY, Hanemaayer A, Poenaru D. Pediatric hand surgery in global health: The Role for International Outreach. Ann Plast Surg 2017;78:162-70.
Ekemenye OP, Ikechukwu OC. Profile of congenital digital anomalies in children seen in two tertiary health facilities in Southern Nigeria. Int Surg J 2020;7:966-9.
Mba UC, Ogbonnaya IS, Onah II. Analysis of congenital hand anomalies at a Specialist Hospital in a developing Country. Internet J Hand Surg 2018;8:1-10.
Edomwonyi EO, Enemudo R, Morgan E, Omokehinde TJ. Pattern of congenital musculoskeletal anomalies in a suburban Nigerian tertiary hospital. Niger J Surg Sci 2018;28:7. [Full text]
Takai IU, Gaya SA, Sheu MT, Abdulsalam M. Pattern of birth defects at a University Teaching Hospital in Northern Nigeria: Retrospective review over a decade. Tropi J Obstet Gynaecol 2019;36:287-92.
Ajao AE, Adeoye IA. Prevalence, risk factors and outcome of congenital anomalies among neonatal admissions in Ogbomosho, Nigeria. BMC Pediatr 2019;19:88.
Bowe J, Conway H. Congenital deformities of the hands. Plast Reconstr Surg (1946) 1956;18:286-90.
Lamb DW, Wynne-Davies R, Soto L. An estimate of the population frequency of congenital malformations of the upper limb. J Hand Surg Am 1982;7:557-62.
Giele H, Giele C, Bower C, Allison M. The incidence and epidemiology of congenital upper limb anomalies: A total population study. J Hand Surg Am 2001;26:628-34.
Watt AJ, Chung KC. Duplication. Hand Clin 2009;25:215-27.
Rengasamy P. Congenital malformations attributed to prenatal exposure to cyclophosphamide. Anticancer Agents Med Chem 2017;17:1211-27.
Al-Qattan MM. Classification of hand anomalies in Poland's syndrome. Br J Plast Surg 2001;54:132-6.
Ibrahim A. Heart-hand syndrome in an African child. Nig J Cardiol 2013;10:81. [Full text]
Swanson AB. A classification for congenital limb malformations. J Hand Surg Am 1976;1:8-22.
Abulezz T, Talaat M, Elsani A, Allam K. Congenital hand anomalies in Upper Egypt. Indian J Plast Surg 2016;49:206-13.
] [Full text]
Muga R, Mumah SC, Juma PA. Congenital malformations among newborns in Kenya. Afr J Food Agric Nutr Dev 2009;9:814-29.
Ekblom AG, Laurell T, Arner M. Epidemiology of congenital upper limb anomalies in 562 children born in 1997 to 2007: A total population study from Stockholm, Sweden. J Hand Surg Am 2010;35:1742-54.
Dao KD, Shin AY, Billings A, Oberg KC, Wood VE. Surgical treatment of congenital syndactyly of the hand. J Am Acad Orthop Surg 2004;12:39-48.
Goldfarb CA, Wall LB, Bohn DC, Moen P, van Heest AE. Epidemiology of congenital upper limb anomalies in a Midwest United States population: An assessment using the Oberg, Manske and Tonkin Classification. J Hand Surg Am 2015;40:127-32.
Ekure EN, Bode-Thomas F, Sadoh WE, Orogade AA, Otaigbe BE, Ujunwa F, et al
. Congenital heart defects in Nigerian Children: Preliminary data from the National Pediatric Cardiac Registry. World J Pediatr Congenit Heart Surg 2017;8:699-706.
International Clearing House for Birth Defects Surveillance and Research. Available from: http://www.icbdsr.org/aboutus/
. [Last accessed on 2020 Feb 11].
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2], [Table 3]