|LETTER TO THE EDITOR
|Year : 2016 | Volume
| Issue : 5 | Page : 444-446
Amniotic band constriction leading to facial asymmetry
Rajesh Kumar Mandal1, Anupam Das2
1 Department of Dermatology, North Bengal Medical College, Sushrutanagar, Darjeeling, India
2 Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal, India
|Date of Web Publication||14-Sep-2016|
Building - “Prerana” 19, Phoolbagan, Kolkata - 700 086, West Bengal
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mandal RK, Das A. Amniotic band constriction leading to facial asymmetry. Indian Dermatol Online J 2016;7:444-6
Amniotic band syndrome is a constellation of congenital anomalies, which may be subtle or extensive., We hereby, report a case of amniotic band constriction over face and scalp in an infant leading to facial asymmetry. The rare occurrence of amniotic band constriction prompted us to report the case.
A 10-month-old female infant, with a congenital depressed scar over her face and scalp was brought to our clinic by her anxious parents. She was the second child born out of non-consanguinous marriage. Maternal age at conception was 22 years. There was no history of any significant illness, illicit drug intake, hypertension, and diabetes mellitus during pregnancy. Family history was noncontributory. The baby was delivered at home and antenatal ultrasonography documents were not available. Cutaneous examination revealed a linear depressed scar oriented transversely across the face and extending to the occipital area of scalp. There was a slight depression of the nasal bridge along with facial asymmetry [Figure 1] and [Figure 2]. There were no similar lesions elsewhere in the body. Besides, there were no other congenital anomalies detected on systemic survey. Hairs, nails, and mucosae were within normal limits. Based on the history and clinical examination, a diagnosis of amniotic band constriction was made.
|Figure 1: Linear depressed constriction band with depressed nasal bridge and facial asymmetry|
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Amniotic band syndrome (ABS) occurs in 1:1200 to 1:15,000 live births without any gender predilection. Family history is usually inconclusive, although there are some reports of ABS among families with Ehler–Danlos syndrome, osteogenesis imperfecta, epidermolysis bullosa, and others. Risk factors associated with the development of ABS include premature birth; low birth weight; maternal illness; trauma; drug exposure such as methadone, cocaine, mifepristone; oophorectomy during pregnancy; intrauterine contraceptive device; and amniocentesis. None of the risk factors were present in our case.
There are multiple hypotheses regarding the pathogenesis of ABS. However, according to the most accepted one, during the 12th week of gestation, amnion presses against the extracelomic space, resulting in obliteration of the space. Incomplete obliteration of this cavity leads to fragility of the amnion, thus making it susceptible to rupture. Following the rupture, the floating amniotic bands easily encircle a developing body part. When this process occurs in early gestation, it may lead to spontaneous abortions. However, if the constriction occurs during the later stages of embryonic development, classical manifestations include rings, fissures, acro-syndactylization, and/or intrauterine amputation of the extremities. If the fetus swallows the bands while they are still partially attached to the placenta, bizarre facial clefts and palatal deficiencies may result. The clefting in our patient could be attributed to this mechanism. Similarly, a severe case of median facial cleft in a case of ABS has been reported.
Clinically, ABS can manifest in numerous forms, the commonest ones being distal ring constrictions, deformities in the limbs, and amputations. Rare findings include syndactyly, lymphedema, clubfoot, cleft lip and palate, pseudo-arthrosis, peripheral nerve palsy, dislocation of hip, and craniofacial synostosis. Rarely, internal organs may be affected., Fetal death resulting from strangulation of the umbilical cord by strands of amniotic membrane has also been reported. During the end of first trimester or the beginning of second trimester, two-dimensional ultrasound can easily diagnose these defects and clinicians can counsel the parents regarding the pathology and the outcome. Recently, three-dimensional ultrasound has been shown to be of benefit in diagnosing this condition, even in the third trimester. These check-ups will ensure a timely diagnosis of defects associated with ABS and prompt intervention to minimize the untoward consequences after birth. Besides, all patients should be advised periodic follow-up until skeletal maturity because of the potential for recurrence of the constriction rings and for secondary contractures that may develop in due course.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]