Archives of Medicine and Health Sciences

CASE REPORT
Year
: 2018  |  Volume : 6  |  Issue : 2  |  Page : 260--261

Amniotic band syndrome


Umma Abdullahi Idris 
 Department of Paediatrics, Federal Medical Centre, Birnin Kudu, Jigawa State, Nigeria

Correspondence Address:
Dr. Umma Abdullahi Idris
Department of Paediatrics, Federal Medical Centre, Birnin Kudu, Jigawa State
Nigeria

Abstract

Amniotic band syndrome is a rare and complex congenital disorder characterized by constricting rings, acrosyndactyly, or often amputations of extremities of neonates. It is a heterogeneous condition, with a broad spectrum of anomalies, where intrinsic alternate with extrinsic causes as amniotic band rupture. The condition can be diagnosed prenatally with an abdominal ultrasound. However, in most reported cases, the diagnosis was made at birth. Treatment includes surgery and rehabilitation. The author reports the first documented case in the north western Nigeria in a term male neonate. The malformation included a constriction band in the right upper arm and distal lower limb with ipsilateral congenital talipes equinovarus. He was stabilized and referred to orthopedic surgeon for expert management.



How to cite this article:
Idris UA. Amniotic band syndrome.Arch Med Health Sci 2018;6:260-261


How to cite this URL:
Idris UA. Amniotic band syndrome. Arch Med Health Sci [serial online] 2018 [cited 2019 Aug 23 ];6:260-261
Available from: http://www.amhsjournal.org/text.asp?2018/6/2/260/248705


Full Text



 Introduction



Amniotic band syndrome (ABS) recognized since 300 BC is a complex disorder characterized by constricting rings, acrosyndactyly, or often amputations of extremities of neonates. It is a rare condition and often is associated with multiple musculoskeletal abnormalities.[1] It is analogs to Streeter dysplasia and amniotic deformity, adhesion, and mutilation sequence.[1],[2] It is a heterogeneous condition, with a broad spectrum of anomalies, where intrinsic causes, as defect of germplasm, vascular disruption, and disturbance of threshold boundaries of morphogens during early gastrulation, alternate with extrinsic causes as amniotic band rupture to explain the condition.[2]

The incidence is estimated to be one case per 10,000–15,000 populations.[2],[3] It usually presents with a characteristics malformation of the limbs. This report describes the clinical presentation of an ABS in a term male neonate.

 Case Report



One-day-old term baby boy was referred to our facility from a peripheral hospital with abnormalities of the right upper and lower limbs noticed at birth. He was a product of full-term supervised pregnancy. The 20-year-old primiparous mother was said to be healthy throughout the pregnancy which was planned and denied any history of abdominal trauma or use of contraception. Mother had folic acid, proguanil, and tetanus prophylaxis during pregnancy. Antenatal ultrasonography was not done. Delivery was through spontaneous vertex delivery, and the baby cried immediately after birth. On examination, the baby was active pink at room temperature and weighed 3.2 kg. Examination of the extremities showed a constricting band in the right upper arm [Figure 1] and the distal portion of the right leg with ipsilateral congenital talipes equinovarus [Figure 2]. The left extremities are normal.{Figure 1}{Figure 2}

 Discussion



The pathogenesis of ABS is postulated based on two theories which are referred to as the “extrinsic model” and the “intrinsic model.[2]” The intrinsic model was proposed by Streeter in the 1930 and suggests that the anomalies and the fibrous bands have a common origin, caused by a perturbation of developing germinal disc of the early embryo.[4] Later, the Torpin's model of the 1965,[5] the “extrinsic theory,” suggested that the birth defects are caused by the action of the fibrous amniotic bands with the sequence rupture of the amnion, followed by loss of amniotic fluid and extrusion of all or parts of the fetus into the chorionic cavity. These encircling strands cause extrinsic compression on the head or limb, leading to the formation of bands, vascular occlusion, and eventually amputation.[1],[2]

There is no known specific mode of inheritance of ABS; however, there are some reported cases of ABS among families with collagen disorders, more specifically Ehlers–Danlos syndrome,[2] in other diseases that involve connective tissue, e.g., in osteogenesis imperfecta.[2] Risk factors implicated in the causation ABS include mother's age, especially primiparas under the age of 25,[2] abdominal trauma,[6] unsuccessful abortion, amniocentesis, and drugs such as ergotamine, acetaminophen, and misoprostol.[2],[3]

This condition is reported to have an incidence of 1 in 12,000 to[2],[3] 15,000 populations and no sex predilection.[7] However, many cases reported by previous authors were male babies.[8],[9]

Depending on the severity of the constriction, the defect may be as minimal as a merely cosmetic band. Deeper bands may cause lymphatic obstruction leading to edema and vascular compromise that necessitates immediate release. Pressure from the bands may potentially cause abnormalities distal to the constriction, such as hemihypertrophy, anterolateral bowing, pseudoarthrosis, leg-length discrepancy, and resistant teratologic clubfeet. These conditions may lead to limited function and difficulty with ambulation.[3],[4]

Constriction bands across the head and face may lead to facial clefts. If the cleft extends into the cranium, encephaloceles may result. Bands that cross the body may compromise the chest (thoracoschisis or extrathoracic heart) or abdomen (gastroschisis).[2],[5]

Diagnosis is made usually at birth, especially in the less developed countries. However, prenatal diagnosis using abdominal ultrasound has been made as early as 14 weeks of gestation, and later, in advanced pregnancies may detect common defects associated with ABS such as amniotic fluid bands, constriction rings, and syndactyly.[10]

Treatment of ABS is multidisciplinary involving the pediatricians and various surgical subspecialties and physiotherapist.

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.

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