|Year : 2018 | Volume
| Issue : 1 | Page : 40-42
Amniotic band syndrome: A case report and review of literature
Karthik Srevatsa, Manasi Gosavi, Pratap Kangle Ranjit, Krutika Mihirkumar Joshi
Department of Pathology, JNMC, KLE Academy of Higher Education and Research, Belgaum, Karnataka, India
|Date of Web Publication||27-Jul-2018|
Department of Pathology, JNMC, KLE Academy of Higher Education and Research, Belgaum, Karnataka
Source of Support: None, Conflict of Interest: None
Amniotic band syndrome is a rare disorder with reported incidence of 1 in 15000. The spectrum of the presentation includes a simple Streeter band on one side to multiple anomalies including craniofacial malformation. In the present case, an 18-year-old primigravida with presented with 18 weeks 3 days’ period of gestation and complains of per vaginal leak. A male fetus weighing 520 g with attached umbilical cord measuring 4 cm was received. External examination of the fetus revealed cleft lip with cleft palate, low-set ears, clubfoot on the right side, amputation of left leg 2.3 cm away from the tibial tuberosity and left palm syndactyly. Microscopic examination of the organs was within normal limits. This case is presented with an intention to contribute to the existing literature and to present the range of anomalies that is seen with this condition, with an emphasis on early detection and diagnosis.
Keywords: Amniotic deformity, adhesions, mutilations complex, amniotic band syndrome, Streeter band
|How to cite this article:|
Srevatsa K, Gosavi M, Ranjit P, Joshi KM. Amniotic band syndrome: A case report and review of literature. J Sci Soc 2018;45:40-2
| Introduction|| |
Amniotic band syndrome is a disease of uncertain etiology with the reported incidence of 1 in 15,000. The early detection and timely intervention give rise to excellent outcome failing which the outcome varies from a mere cosmetic defect to even fatal death.
| Case Report|| |
An 18-year-old primigravida with 18 weeks 3 days’ period of gestation came with per vaginal leak and complained of watery discharge since one night which was foul smelling and nonsticky. The previous antenatal checkup was not done. The patient had no other significant medical history. Obstetric examination had revealed uterus of about 18 weeks in size with cephalic presentation. Ultrasonography at the time of examination had revealed multiple anomalies. A stillborn baby was delivered. After obtaining appropriate consent, the fetus was sent for autopsy. The autopsy examination revealed a male fetus weighing around 520 g along with attached umbilical cord measuring 4 cm. Placenta measured 13 cm × 9 cm × 2 cm along with attached umbilical cord measuring 18 cm. Grossly, the placenta and umbilical cord were unremarkable. External examination of the fetus revealed cleft lip with cleft palate, low-set ears, clubfoot on the right side, amputation of left leg 2.3 cm away from the tibial tuberosity and left palm syndactyly, as depicted in [Figure 1]a, [Figure 2]a and [Figure 2]b. No bands or membrane-like structures were adherent to the fetus or umbilical cord at the time of receiving the autopsy. The fetus was subjected to infantogram which revealed a soft tissue shadow at the left axillary region which is depicted in [Figure 1]b. Grossly, the organs were found in situ and normal. The microscopic examination of the soft-tissue shadow was suggestive of cystic hygroma, rest of the microscopic examination of organs were within normal limits. Gross and histological examination of the umbilical cord was within normal limits. Placenta was not received.
|Figure 1: (a) Black solid arrowhead depicting clubfoot, black arrow depicting autoamputation of the left lower limb, white solid arrowhead depicting autoamputation of the left upper limb. (b) Infantogram depicting soft-tissue shadow in the left axillary region|
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|Figure 2: (a) Depicting cleft palate. (b) White solid arrowhead depicting cleft lip, black arrow depicting autoamputation of the left upper limb|
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| Discussion|| |
Amniotic membrane is essentially a fetal epidermis such as layer. Amniotic band syndrome is also called as congenital construction band syndrome, amniotic deformity, adhesions, mutilations complex, and Streeter band. The incidence of amniotic band syndrome to be around 1 in 15,000 to around 1 in 10,000. The spectrum of the presentation includes a simple Streeter band on one side to multiple anomalies including craniofacial malformation. The two theories of amniotic band syndrome include intrinsic theory and extrinsic theory. The intrinsic model proposed by Streeter states that anomalies and fibrous band have common origin because of improper development of germinal disc in the early embryo. The bands represent either sheets of macerated epidermis or these are the result of circumscribed areas of imperfect histogenesis. The aberrant tissue represents merely residues of an abnormal developmental process and not a cause. This theory is often used to explain major craniofacial abnormalities, body-wall defects, and internal organ abnormalities. The extrinsic model is widely accepted with a sequence of events involving rupture of amnion followed by loss of amniotic fluid and extravasations of parts of the fetus into the chorionic cavity. The exact nature of anomalies depends on timing of rupture of band. The primary defect may be due to an early rupture of the amnion caused by an intrinsic weakness, inflammation, and trauma. This would permit fluid leakage, leading to the introduction of the fetus into the chorionic cavity. The chorion reabsorbs this fluid and stimulates the proliferation of mesenchymal bands. These entangle the fetus and limit its movements, with subsequent mechanical constrictions.
The various anomalies that are associated with amniotic band syndrome include clubfoot, choanal atresia, gastroschisis, omphalocele, bladder exstrophy, imperforate anus and anencephaly, facial clefting, asymptomatic microphthalmia, incomplete or absent cranial calcification, and anterior abdominal wall defects.
Other abnormalities include syndactyly, lymphedema, clubfoot, phalangeal hypoplasia, and limb-length discrepancy. Pseudoarthrosis, metatarsus adductus, peripheral nerve palsy, dystrophic nails, cleft lip, and palate skin-tube pedicles visceral body wall malformations and eccentric craniofacial synostosis defects are other associations. Congenital brain abnormalities, cardiac malformations, short stature, spina bifida, reported in the literature probably are most likely to represent incidental findings. Fetal death associated with amniotic band strangulation of the umbilical cord has also been reported. Family history is not contributory, and the syndrome occurs in no particular association with known genetic or chromosomal disorders, and the syndrome is almost always sporadic in nature. Risk factors for amniotic band syndrome include young maternal age, unplanned pregnancy, hyperthermia, nonsteroidal anti-inflammatory drug usage. First pregnancy is more likely to have these defects, and the exact reason for this is not clear; however it is thought that the vascularity of a multi gravid uterus is likely to be better than that of a Primi gravid uterus., Antenatal risk factors associated with amniotic band syndrome include prematurity of less than 37 weeks, low birth weight of less than 2,500 g, maternal illness during pregnancy, maternal drug exposure, and maternal hemorrhage/trauma.
It is difficult to visualize the amniotic bands in the first trimester also more difficult is to identify these bands if they are present in the extremities. The ultrasonographic analysis allows for the detection of amniotic band syndrome prenatally by visualization of amniotic sheets or bands attached to the fetus. It is difficult to visualize the amniotic bands in the first trimester, and it is more difficult to identify these bands if these are present in the extremities. However, in the second and third trimesters of pregnancy, it is relatively easy to detect the major anomalies of amniotic band syndrome by its characteristic features and restriction of motion. The sequential ultrasonographic examinations, along with restricted fetal movements, help in correct diagnosis of amniotic band syndrome in utero. Amniotic band syndrome should be considered when characteristic asymmetric fetal anomalies are visualized ultrasonographically irrespective of the presence or absence of fibrous membranes. [Table 1] describes the diagnostic criteria for amniotic band syndrome.
|Table 1: Patterson's diagnostic criteria for congenital ring constrictions|
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The rupture of amniotic band leads to entrapment of fetal parts by the sticky mesodermal brands which arise from the chronic side of amnion. Once the band is formed, there is decreased blood supply and subsequent amputation. [Table 2] describes the usual malformations that help in timing the rupture of amniotic band.
Shallow grooves or bands unless associated with neurovascular compromise require no operative treatment. Deep constriction bands must be surgically released immediately to prevent the risk of auto-amputation or gangrene.
Overall, the goal is to improve functionality and to minimize additional problems as the child grows. Parental counseling is recommended to convey that there is no known associated risk for subsequent pregnancies.
| Conclusion|| |
Amniotic band syndrome can result in various anomalies. This case report intends to add to the literature available regarding the same and highlights the role of fetal autopsy in documenting the anomalies present.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]