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The Epidemiology of Congenital Upper Limb Anomalies (CULA) In Population of D. I. Khan Division, Pakistan
Keywords:
Congenital upper limb anomalies; epidemiology; OMT classification; Polydactyly; Orthopedics; Syndactyly; Hand.Abstract
Objective: To find out the Prevalence of Congenital Upper Limb Anomalies by its distribution according to Type, Gender, Family history & Cousin marriages in one calendar year.
Study Design: cross sectional study.
Place and Duration of study: Department of Orthopaedics Medical Teaching Institute Dera Ismail Khan, Pakistan from January 1, 2022 to December 31, 2022.
Materials & Methods: Infants (from birth to one year age) of both genders having congenital anomalies of upper extremities were included in this study. Infants with cerebral palsy, birth palsy and birth trauma were excluded. Swanson’s classification was used for classification and types of congenital upper limb anomalies. All cases were analyzed for the type of CULA, gender, family history and parents as first-degree relatives i.e., cousin marriages. Categorical variables were measured as count and percentages while numeric variable was measured as mean and standard deviation, with 5% margin of error and 95% confidence interval, p-value < 0.05 was set as significant.
Results: The overall prevalence of congenital upper extremity anomalies was 142(0.26%), which is similar to International ratios. Polydactyly was most common (51 cases) followed by syndactyly (39 cases) and radial club hands (17 cases). The ratio among boys and girls was 52% and 48% respectively. Forty (28%) patients had a positive family history for CULA as compared to 102 (72%) patients, with p-value <0.05 which was significant. One hundred and twelve (79%) patients had their parents as first degree relative i.e., cousin marriages as compared to 30 (21%) patients with no history of cousin marriages, with p-value <0.05 which was significant.
Conclusions: In our study it was found that Polydactyly is the commonest congenital upper limb anomaly. There was increased incidence of CULA in Cousin marriages & patients with positive family history for CULA. Increased incidence of CULA was found in boys as compared to girls.
References
Goswami P, Kumar M, Effendi S. Frequency and variation of congenital anomalies of the upper limb at Liaquat University Hospital, Jamshoro. Int J Sci Basic Appl Res (IJSBAR) 2014;17(2):275–282.
Hoyert, D.L, Xu, J.Q. Deaths: Preliminary data for 2011. Natl Vital Stat Rep 2012; 61, 40–42.
World Health Organization. “Congenital Anomalies,” Fact Sheet 370; World Health Organization: Geneva, Switzerland, 2014; Available online: http://www.who.int/mediacentre/factsheets/fs370/en/ (accessed on 5 August 2022).
Gallant GG, Bora FW. Congenital deformities of the upper extremity. J Am Acad Orthop Surg 1996 May 1;4(3):162-71.
Bae DS, Canizares MF, Miller PE, Roberts S, Vuillermin C, Wall LB et al. Intraobserver and interobserver reliability of the Oberg-Manske-Tonkin (OMT) classification: establishing a registry on congenital upper limb differences. J Pediatr Orthop 2018 Jan1; 38:69–74.
Mburia-Mwalili A, Yang, W. Birth Defects Surveillance in the United States: Challenges and Implications of International Classification of Diseases, Tenth Revision, Clinical Modification Implementation. Int Sch Res Not 2014; 2014.
Chung MS. Congenital differences of the upper extremity: classification and treatment principles. Clin Orthop Surg 2011 Sep 1;3(3):172-7.
Kozin SH. Upper Extremity Congenital Anomalies. J Bone Joint Surg (Am) 2003;85: 1564–1576.
Farhud D, Walizadeh G, Farhud I. Oto palato-digital syndrome in an Iranian infant. MONATSSCHIR KINDERH. 1989; 137 (10): 681-3.
Shahnazi M, Azari S. Contributing factors in major malformations in neonatals born in alzahra medical-educational hospital, Tabriz. J Caring Sci 2010; 5 (18): 49-56.
Hematyar M, Khajouie P. Prevalence of congenital anomalies in 1000 live births in Javaheri Hospital, Tehran, 2004. Med Sci J Islamic Azad Univ 2005 Jun10;15(2):75-8.
Swanson AB, Swanson GD, Tada K. A classification for congenital limb malformation. J Hand Surg Am 1983 Sep 1;8(5):693-702.
Ekblom AG, Laurell T, Arner M. Epidemiology of congenital upper limb anomalies in Stockholm, Sweden, 1997 to 2007: application of the Oberg, Manske, and Tonkin classification. J Hand Surg Am 2014 Feb 1;39(2):237-48.
Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental biology and classification of congenital anomalies of the hand and upper extremity. J Hand Surg Am 2010 Dec; 35(12):2066–2076.
Dobyns JH. Congenital hand deformities. Hand surg 1988; 1:255-552.
Goldfarb CA, Shaw N, Steffen JA, Wall LB. The prevalence of congenital hand and upper extremity anomalies based upon the New York Congenital Malformations Registry. J Pediatr Orthop 2017 Mar; 37(2):144.
Shin YH, Baek GH, Kim YJ, Kim MJ, Kim JK. Epidemiology of congenital upper limb anomalies in Korea: A nationwide population-based study. PLoS One 2021 Mar 9;16(3): e0248105.
Ogino T, Minami A, Fukuda K, Kato H. Congenital anomalies of the upper limb among the Japanese in Sapporo. J Hand Surg Am 1986 Jun;11(3):364-71.
Al-Gazali LI, Dawodu AH, Sabarinathan K, Varghese M. The profile of major congenital abnormalities in the United Arab Emirates (UAE) population. J Med Genet 1995 Jan 1;32(1):7-13.
Alijahan R, Mirzarahimi M, Ahmadi-Hadi P, Hazrati S. Prevalence of Congenital Abnormalities and Its Related Risk Factors in Ardabil, Iran, 2011. Iran J Obstet Gynecol Infertil 2013 May; 16(54): 16-25.
Farhud D, Walizadeh GR, Kamali MS. Congenital malformations and genetic diseases in Iranian infants. Hum Genet 1986 Dec;74 (4): 382-5.
Golalipour MJ, Ahmadpour-Kacho M, Vakili MA. Congenital malformations at a referral hospital in Gorgan, Islamic Republic of Iran. East Mediterr Health J 2005;11(4): 707-15.
Senes FM, Calevo MG, Adani R, Baldrighi C, Bassetto F, Corain M et al. Hand and Upper Limb Malformations in Italy: A Multicentric Study. J Hand Surg Am (Asian-Pacific Volume) 2021 Sep 1;26(03):345-50.
2 Parikh YN, Kalathia MB, Soodhana D. Clinical profile of congenital limb anomalies in neonates. Int J Contemp Pediatr 2018 Feb 22;5(2):299.
Uzun H, Özdemir FD, Üstün GG, Sakarya AH, Bitik O, Aksu AE. Oberg-Manske-Tonkin classification of congenital upper extremity anomalies: the first report from Turkey. Ann Plast Surg 2020 Sep 1;85(3):245-50.
Barik S, Pandita N, Paul S, Kumari O, Singh V. Prevalence of congenital limb defects in Uttarakhand state in India–A hospital-based retrospective cross-sectional study. Clin Epidemiol Glob Health 2021 Jan 1; 9:99-103.
