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布基纳法索儿童先天性畸形及与口面部裂隙相关的病症

Congenital malformations and medical conditions associated with orofacial clefts in children in Burkina Faso.

作者信息

Nagalo Kisito, Ouédraogo Isso, Laberge Jean-Martin, Caouette-Laberge Louise, Turgeon Jean

机构信息

Service of Paediatrics, El Fateh-Suka Clinic, Ouagadougou, Burkina Faso.

UFR/SDS, University of Ouagadougou, Ouagadougou, Burkina Faso.

出版信息

BMC Pediatr. 2017 Mar 14;17(1):72. doi: 10.1186/s12887-017-0833-9.

Abstract

BACKGROUND

Orofacial clefts are usually isolated cases but can be associated with other congenital malformations that are either recognised or unrecognised syndromes. The reported prevalence and pattern of such associated malformations, however, vary among studies.

OBJECTIVES

To assess the frequencies and aetiologies of congenital malformations and associated medical conditions in children with orofacial clefts in Burkina Faso (Western Africa).

METHODS

A retrospective descriptive study was carried out at the El Fateh-Suka Clinic in Ouagadougou, Burkina Faso. All children who attended surgery for the repair of a cleft lip and/or palate were included in this study.

RESULTS

The frequency of congenital malformations associated with cleft lip and/or palate was 39/185 (21.1%). In the group with multiple congenital malformations of unknown origin (34 patients; 18.4%), 66.7% had cleft lip and palate, followed by isolated cleft lip (27.4%) and isolated cleft palate (5.9%). The digestive system (35.3%), the musculoskeletal system (19.6%), and eye, ear, face, and neck (15.7%) were the most affected systems. In the group of syndromic malformations (five patients; 2.7%), amniotic band syndrome (one patient), Van der Woode syndrome (one patient), Goltz syndrome (one patient), and holoprosencephaly (two patients) were identified. Medical conditions included anaemia (39.4%), infections (9.2%), malnutrition (7.5%), and haemoglobinopathies (4.3%).

CONCLUSIONS

Congenital malformations and medical co-morbidities were frequent in children with OFCs. Further studies and a National Malformations Registry are needed to improve the comprehension of OFCs in Burkina Faso.

摘要

背景

口面部裂隙通常为孤立病例,但可能与其他先天性畸形相关,这些畸形可能是已被认识的综合征,也可能是未被认识的综合征。然而,此类相关畸形的报告患病率和模式在不同研究中有所不同。

目的

评估布基纳法索(西非)口面部裂隙患儿先天性畸形及相关疾病的发生率和病因。

方法

在布基纳法索瓦加杜古的法塔赫 - 苏卡诊所进行了一项回顾性描述性研究。本研究纳入了所有因唇裂和/或腭裂修复手术前来就诊的儿童。

结果

与唇裂和/或腭裂相关的先天性畸形发生率为39/185(21.1%)。在多系统先天性畸形病因不明组(34例患者;18.4%)中,66.7%为唇腭裂,其次是单纯唇裂(27.4%)和单纯腭裂(5.9%)。受影响最严重的系统是消化系统(35.3%)、肌肉骨骼系统(19.6%)以及眼、耳、面和颈部(15.7%)。在综合征性畸形组(5例患者;2.7%)中,确诊了羊膜带综合征(1例患者)、范德伍德综合征(1例患者)、戈尔茨综合征(1例患者)和前脑无裂畸形(2例患者)。相关疾病包括贫血(39.4%)、感染(9.2%)、营养不良(7.5%)和血红蛋白病(4.3%)。

结论

口面部裂隙患儿中先天性畸形和合并症很常见。需要进一步研究并建立国家畸形登记处,以提高对布基纳法索口面部裂隙的认识。

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