Congenital Nonsyndromic Anonychia of Toes Due to Amniotic Bands

IF 0.2 Q4 DERMATOLOGY
Abanti Bagchi, Asok Kumar Gangopadhyay, Nikhileswar Khawash, Sujata Sengupta, Swikruti Mohapatra
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引用次数: 0

Abstract

Madam, Congenital anonychia is a rare disorder which might be either a part of a syndrome or an isolated occurrence.[1] Non-syndromic anonychia can be partial or total. Amniotic band syndrome (ABS) may present with a variety of congenital anomalies or as a very subtle, localized form.[2] ABS is due to rupture of the amnion early in pregnancy resulting in multiple loose strands called amniotic bands, that adhere to and may entangle the fetus as well.[3] Invasive procedures like chorionic villus sampling may lead this condition. We report a 3-day-old male neonate who presented with absence of all 10 toenails with normal fingernails, since birth. Constriction bands were present circumferentially in all toes, proximal to the areas of absent nails, and also over the ball of great toe on the left foot [Figures 1-3]. The fingernails were normal and there were no band-like depressions over them [Figure 4]. The baby was born by normal vaginal delivery as the first child of non-consanguineous parents and there was no history of any maternal drug intake, hypertension, or diabetes mellitus during pregnancy. The baby was otherwise normal with no other skin lesions or congenital anomalies. Radiology of feet showed no abnormality. Based on the clinical findings, a diagnosis of congenital anonychia due to amniotic constriction bands was made.Figure 1: Whitish areas representative of absent nails with ring-like constrictions proximallyFigure 2: Constriction rings on the plantar surfaceFigure 3: Bilaterally symmetrical involvement of both feetFigure 4: Normal fingernailsWhile the formation of amniotic bands in early gestation may lead to spontaneous abortions, in more advanced adanced gestation, classical rings, fissures, acro-syndactylization, or amputation of limbs may occur.[4] Clinically, constrictive rings, limb defects, neural or spine defects, and craniofacial defects are the four categories of presentation. Recently developed advanced three-dimensional ultrasound can diagnose these defects easily. Craniofacial abnormalities, such as encephalocele, cleft lip and palate, and spinal defects are rarely reported anomalies due to ABS. Most ABS cases are sporadic with no known recurrence risk unless in cases of familial ABS.[3] Our patient fortunately was mildly affected with only the absence of nails distal to the ring-like constrictions. This type of congenital nonsyndromic anonychia due to amniotic bands is a rare occurrence. 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. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
羊膜带所致先天性无综合征性足趾缺失
夫人,先天性匿名症是一种罕见的疾病,可能是综合征的一部分,也可能是孤立的。[1]非综合征性匿名可以是部分匿名或全部匿名。羊膜带综合征(ABS)可能表现为各种先天性异常,也可能表现为非常微妙的局部形式。[2]ABS是由于妊娠早期羊膜破裂导致多股松散的羊膜带,这些羊膜带粘附并可能缠绕胎儿。[3]侵入性手术如绒毛膜绒毛取样可能导致这种情况。我们报告了一个3天大的男婴谁提出了所有10个脚趾甲与正常的指甲,因为出生。所有脚趾,近端缺失趾甲区域,以及左脚大脚趾球周围都有环状缩窄带[图1-3]。指甲正常,指甲上方未见带状凹陷[图4]。该婴儿为非近亲父母正常阴道分娩的第一个孩子,怀孕期间母亲无任何药物摄入、高血压或糖尿病史。婴儿在其他方面都很正常,没有其他皮肤损伤或先天性异常。足部放射学未见异常。根据临床表现,诊断为先天性羊膜收缩带所致的先天性匿名症。图2:足底表面的环状缩窄图3:双侧对称受累图4:正常指甲在妊娠早期羊膜带的形成可能导致自然流产,在妊娠晚期,可能发生典型的环状、裂隙、趾间跨化或截肢。[4]临床上,收缩环、肢体缺损、神经或脊柱缺损和颅面缺损是四种表现。近年来发展的先进的三维超声可以很容易地诊断这些缺陷。颅面异常,如脑膨出、唇腭裂和脊柱缺损,很少报道ABS引起的异常。大多数ABS病例是散发性的,没有已知的复发风险,除非是家族性ABS。[3]我们的患者幸运的是轻微的影响,只有环状缩窄远端没有指甲。这种由羊膜带引起的先天性无综合征性失语症是很少见的。患者同意声明作者证明他们已经获得了所有适当的患者同意表格。以患者同意在期刊上报道其图像和其他临床信息的形式。患者明白他们的姓名和首字母不会被公布,并将尽力隐藏他们的身份,但不能保证匿名。财政支持及赞助无。利益冲突没有利益冲突。
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