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Year : 2014  |  Volume : 10  |  Issue : 2  |  Page : 15-17

Amniotic band syndrome in a Nigerian cleft patient: A case report

1 Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, University of Port Harcourt, Port Harcourt, Nigeria
2 Department of Child Dental Health, Faculty of Dentistry, University of Port Harcourt, Port Harcourt, Nigeria
3 Department of Paediatrics and Child Health, Faculty of Clinical Sciences, College of Health Sciences, University of Port Harcourt, Port Harcourt, Nigeria

Date of Web Publication15-Apr-2015

Correspondence Address:
Oladimeji Adeniyi Akadiri
PO Box 212, Choba, Port Harcourt, Rivers State
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0794-9316.155175

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Amniotic band syndrome is a set of congenital malformations ranging from minor constriction rings and lymphedema of the digits to complex, bizarre multiple congenital anomalies that are attributed to amniotic bands that stick, entangle, and disrupt fetal parts. The most commonly associated anomalies include amputations, constriction bands, syndactyly, craniofacial defects, and club feet. A case of amniotic band syndrome involving limb anomalies and orofacial cleft deformity in a Nigerian child is reported.

Keywords: Amniotic band syndrome, limb anomalies, Nigerian child, orofacial cleft

How to cite this article:
Akadiri OA, Eigbobo JO, Otaigbe BE. Amniotic band syndrome in a Nigerian cleft patient: A case report. Nigerian J Plast Surg 2014;10:15-7

How to cite this URL:
Akadiri OA, Eigbobo JO, Otaigbe BE. Amniotic band syndrome in a Nigerian cleft patient: A case report. Nigerian J Plast Surg [serial online] 2014 [cited 2024 Mar 2];10:15-7. Available from:

  Introduction Top

Amniotic band syndrome (ABS) is a congenital disorder caused by entrapment of fetal parts in fibrous amniotic bands while in utero. Various descriptive synonyms have been adopted for the condition among which are "ADAM complex," "amniotic band sequence," "congenital constriction bands," and "pseudoainhum," [1] It occurs in 1:1200 to 1:15000 live births. [1] It accounts for 178 in 10,000 miscarriages [1] and 1 in 70 still births. [2] The most common anomalies are found in the limbs, with hand and finger anomalies occurring in up to 80% of cases, being about twice as common as accompanying foot deformities. [3] Unilateral club foot can be seen in 30% of foot anomalies, whereas bilateral involvement occurs in up to 20%. [3] Up to 50% of cases may have other congenital anomalies including orofacial clefts, talipes, and spinal deformities. [4]

More severe cases can involve the abdomen (limb-body wall complex) and calvarium (encephalocele). [2] Involvement of internal viscus is said to be very rare except in case of abdominal-wall or abdominothoracic-wall defects. [5] Prognosis and treatment depend on the location and severity of the constricting bands [1],[6] but early detection using three-dimensional (3D) ultrasonography and/or magnetic resonance imaging (MRI) could provide opportunity for prenatal correction and improved outcome.

A case is here reported to emphasize the importance of comprehensive antenatal evaluation and the ignorance and superstition expressed by the mother.

  Case Report Top

A 6-year-old boy with congenital orofacial cleft was brought to our clinic. He was the fourth in a family of five children; all other children were said to be normal. The father and mother were non-consanguineous artisans of low socioeconomic class. Maternal age at conception was 24 years.

The mother gave a history of febrile illness during the first trimester of pregnancy. She denied using any illicit drug but only medications prescribed at the antenatal clinic. She claimed that the gestational age was protracted until 52 weeks but delivery was per vaginal and uneventful. The mother believed that her protracted pregnancy was because the child was tied in her womb by her enemies and that this was responsible for the deformities.

The patient was examined by a team of specialists: Maxillofacial surgeon, pedodontist, and pediatric cardiologist. He was found with left unilateral incomplete cleft of the primary palate [Figure 1] and bifid uvula. Other congenital anomalies [Figure 2] detected were constriction rings on the ring finger and missing proximal phalange of the middle finger on the right hand, complex, incomplete syndactyly of the right foot, a clubbed left foot, and constriction ring around the lower third of the left leg. Clinical and echocardiographic examinations confirmed functionally and structurally normal heart; the atrial and ventricular dimensions were within normal limits and the interatrial and interventricular septa were intact. Review and examination of other systems was unremarkable. A clinical diagnosis of ABS was made.
Figure 1: Patient with left unilateral incomplete cleft of the primary palate

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Figure 2: Other associated deformities: (a) Syndactyl, (b) Constriction ring on left leg and club foot, (c) Proximal constriction of right fourth finger

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  Discussion Top

ABS comprises a set of congenital malformations ranging from minor constriction rings and lymphedema of the digits to complex bizarre multiple congenital anomalies that are attributed to amniotic bands that stick, entangle, and disrupt fetal parts. [7],[8] It occurs in 1:1200 to 1:15000 live births with no sex predilection. Family history seldom reveals any direct inheritance pattern since the syndrome occurs in no particular association with known genetic or chromosomal disorders and there is no recurrence in siblings or children of affected adults. However, there are some reports of ABS among families with collagen disorders and some other connective tissue diseases such as Ehler-Danlos syndrome, osteogenesis imperfecta, and epidermolysis bullosa congenital. [9] The patient here presented is the fourth among five siblings and there is no occurrence in other siblings, the parents also denied any known history of connective tissue diseases in their families.

The exact etiology of the ABS is not known; early rupture of the amniotic membrane is the most accepted view. [5],[8] However, genetic, vascular, familial, and mesodermal disruptions have been proposed as etiological factors. [10] Prenatal risk factors associated include prematurity (<37 weeks), low birth weight (<2500 g), maternal illness (during pregnancy), maternal hemorrhage/trauma, maternal drug exposure such as methadone. [1] Other factors are oophorectomy during pregnancy, [11] intrauterine contraceptive device, [12] and amniocentesis. [13] Most of these were excluded in the present case. Although there was a positive history of maternal illness during pregnancy, the nature and severity of illness could not be elicited but might probably be a mild illness as the mother denied taking any other drug besides those prescribed at the antenatal clinic. Prematurity of less than 37 weeks rather than protracted gestation was associated with ABS. In the present case, there was a history of prolonged gestation of up to 52 weeks which the mother superstitiously believed was masterminded by her enemies through diabolical means and was responsible for the birth defects in her child. Though, there was no objective evidence to confirm the history of prolonged gestation.

The clinical features of ABS is multiple and of variable severity. Distal ring constrictions, limb deformities, and intrauterine amputations are the most common findings. [7] Other abnormalities found are syndactyly of the fingers and toes, progressive lymphedema, clubfoot, clubhands, stunted growth of fingers and toes and limb length discrepancy, [7] cleft lip and palate. Less common are pseudarthrosis, metatarsus adductus, peripheral nerve palsy, dystrophic nails, postnatal gangrene, dislocated hip, visceral body wall malformations, [8] eccentric craniofacial synostosis defects, and skin-tubed pedicles. [2] The internal organs are rarely affected, therefore it was no surprise that the heart of the patient was normal in structure and function. On the contrary, Karaman et al. [5] reported internal organ deformity in a 10-day-old subject who had a severe form of ABS with brain anomalies. Similarly, Halder [14] reported abnormal anatomy of the heart and multiple internal malformations in stillborn fetuses.

Fetal death associated with amniotic band strangulation of the umbilical cord has also been reported. [14] Considering the risk therefore, it is judicious that every pregnant woman, irrespective of the socieconomic status, undergo serial ultrasonographic examinations especially with 3D ultrasound scanner so as to detect and possibly minimize the extent and severity of the defect that may accompany the syndrome through prenatal or early postnatal surgical intervention. In this case, the mother presented no report of any ultrasound done during her antenatal care.

  Conclusion Top

A case of amniotic band syndrome involving orofacial cleft deformity has been reported in a child from an indigent background. The combination of ignorance, superstitious belief, and poverty had kept the child from proper medical evaluation and prompt corrective intervention. This case thus underscores the importance of community health education and properly supervised antenatal care and referral systems for pregnant women. The authors are of the opinion that such undertakings could debrief the locales of traditional superstitions and encourage utility of the facilities and expertise that are now available to mitigate the severity of congenital birth defects.

  References Top

Ross MG. Pathogenesis of amniotic band syndrome. Am J Obstet Gynecol 2007;197:219-20.  Back to cited text no. 1
Glass JM. Fetal decapitation associated with amniotic bands. J Diagn Med Sonography 2007;26:32-4.  Back to cited text no. 2
Allen LM, Silverman RK, Nosovitch JT, Lohnes TM, Williams KD. Constriction rings and congenital amputations of the fingers and toes in a mild case of amniotic band syndrome. J Diagn Med Sonography 2007;23:280-5.  Back to cited text no. 3
Hill DS. Extensive Nonunion of the Amnion: An unusual presentation of Amniotic band syndrome? J Diagn Med Sonogr 2010;26:263-5.  Back to cited text no. 4
Karaman A, Kahveci H, Öztürk C. Amniotic band sequence associated with Fronto-Ethmoidal Meningo-Encephalocele. New J Med 2012;29:187-8.  Back to cited text no. 5
Rapini RP, Bolognia JL, Jorizzo JL. Dermatology. Vol. 2. St. Louis: Mosby; 2007.  Back to cited text no. 6
Adeosun OO, James O, Akinmoladun VI, Owobu T. Amniotic band syndrome associated with orofacial clefts: A report of two cases. Oral Surg 2012;5:185-9.  Back to cited text no. 7
Mahmood NA. Amniotic bands: Are they all threat for the fetus? Bahrain Med Bull 2008;30:1-6.  Back to cited text no. 8
Kroes HY, Pals G, van Essen AJ. Ehlers-Danlos syndrome type IV: Unusual congenital anomalies in a mother and son with a COL3A1 mutation and a normal collagen III protein profile. Clin Genet 2003;63:224-7.  Back to cited text no. 9
Nyberg DA, Mahony BS, Pretorius DH. Diagnostic Ultrasound of Fetal Anomalies. Littleton, MA: Year Book Medical Publishers 1990;146:42.  Back to cited text no. 10
Tanaka O, Koh T, Otani H. Amniogenic band anomalies in a fifth-month fetus and in a newborn from maternal oophorectomy during early pregnancy. Teratology 1986;33:187-93.  Back to cited text no. 11
Csécsei K, Szeifert GT, Papp Z. Amniotic bands associated with early rupture of amnion due to an intrauterine device. Zentralbl Gynakol 1987;109:378-41.  Back to cited text no. 12
Kohn G. The amniotic band syndrome: A possible complication of amniocentesis. Prenat Diagn 1987;8:303-5.  Back to cited text no. 13
Halder A. Amniotic band syndrome and/or limb body wall complex: Split or lump. Appl Clin Genet 2010;3:7-15.  Back to cited text no. 14


  [Figure 1], [Figure 2]


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