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Year : 2015  |  Volume : 58  |  Issue : 4  |  Page : 528-530
Significance of fibrotic bands in utero - Amniotic band sequence with limb body wall complex: A rare case of fetal autopsy

Department of Pathology, Government Medical College, Kottayam, Kerala, India

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Date of Web Publication4-Nov-2015


Amniotic band sequence (ABS) includes a wide spectrum of abnormalities resulting from entrapment of various fetal parts from a disrupted amnion, ranging from a mere constriction ring affecting a finger to a fatal form called limb body wall complex (LBWC). Reported cases of ABS with LBWC are very few. The spectrum of anomalies depends on which part gets entrapped and at what point of gestation. Hence, the clinical presentation can be extremely variable. Early detection of such cases using sonology is really challenging due to the small size of the fibrotic bands. Here, we present a case of amniotic band syndrome with LBWC in a fetus at 24 weeks of gestation, which was referred for an autopsy. The fetus also showed scoliosis, gastroschisis, lumbosacral meningocele, congenital talipes equinovarus, and cleft palate, thus having features of placenta cranial and placenta abdominal phenotype which is very rare.

Keywords: Amniotic band sequence, congenital anomalies, fetal autopsy, limb body wall complex

How to cite this article:
Devi P L, Cicy P J, Thambi R, Poothiode U. Significance of fibrotic bands in utero - Amniotic band sequence with limb body wall complex: A rare case of fetal autopsy. Indian J Pathol Microbiol 2015;58:528-30

How to cite this URL:
Devi P L, Cicy P J, Thambi R, Poothiode U. Significance of fibrotic bands in utero - Amniotic band sequence with limb body wall complex: A rare case of fetal autopsy. Indian J Pathol Microbiol [serial online] 2015 [cited 2021 Jul 25];58:528-30. Available from: https://www.ijpmonline.org/text.asp?2015/58/4/528/168876

   Introduction Top

Amniotic band sequence (ABS) refers to a wide range of congenital anomalies, most typically limb and digital amputations or rarely as life-threatening anomalies such as body wall defects and neural tube defects.[1],[2] Multiple factors are implied in the pathogenesis of limb body wall complex (LBWC)/ABS. Intrinsic causes, as a 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.[1],[3] LBWC is a rare type of, fetal malformation syndrome in ABS, appearing in 0.21–0.31/10000 deliveries.[4] The diagnosis of this entity is based on two of the three given features: (1) Exencephaly/encephalocele and facial clefts (2) thoraco- and/or abdominoschisis and (3) limb defects.[4],[5],[6]

   Case Report Top

The case we are presenting here is a fetus of 24 weeks gestation with facial clefts, abdominal wall defect with gastroschisis and meningomyelocele. This we received as products of conception of a 19-year-old primigravida who presented at 21 ± 4 weeks gestation with anomaly scan report for a fetal autopsy. The antenatal period was uneventful. X-ray examination showed scoliosis of the lumbosacral spine and congenital talipes equinovarus [Figure 1]a. The fetus weighed 580 g. including the placenta which was seen adherent to the anterior body wall defect and partly covering it. All the abdominal organs including the intestine were protruding out through this defect without a membranous covering. The umbilical cord was extremely short, and placenta was attached to the forehead by a fibrous band, adjacent to the facial cleft [Figure 1]b and [Figure 1]c. Lumbosacral meningomyelocele was present as a cystic mass at the region of sacrum and coccyx and slightly to the left side [Figure 1]d. All external orifices were patent. Examination of internal organs revealed a short esophagus, cysts in the liver and retroperitoneum. Histology showed fibrocollagenous tissue in the connecting band, and meningocele showed lining epithelium on both aspects [Figure 2]a and [Figure 2]b. Higher magnification of the same showing squamous lining on one aspect and neuroepithelium on the other [Figure 2]c. Liver parenchyma showed cysts lined by mesothelial cells [Figure 2]d. Thus, a final diagnosis of ABS with LBWC was given. Because of the fatal nature of the disease and unwillingness of the parents, further investigations were not performed in this case.
Figure 1: (a) X-ray showing scoliosis and congenital talipes equinovarus. (b) Gross showing abdominal wall defects and an amniotic band in the forehead region. (c) Gross picture showing facial cleft. (d) Fetus showing lumbosacral meningomyelocele

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Figure 2: (a) Microscopy demonstrating amniotic band with a lining epithelium (H and E, ×200). (b) Photomicrograph of meningiomyelocele (H and E, ×100). (c) Higher magnification of the same showing squamous lining on one aspect and neuroepithelium on the other (H and E, ×200). (d) Hepatic cyst, inset showing higher magnification of hepatic cyst ×200 (H and E, ×10)

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   Discussion Top

ABS is a destructive fetal complex caused by the disruption of the amnion followed by entanglement of the fetal parts in the amniotic bands resulting in bizarre and asymmetrical defects that can involve several organs, especially limbs, cranium or spine.[1],[3],[4] This congenital disorder is known by multiple names and confusion regarding nomenclature is mainly attributed to the heterogeneity in clinical manifestations, etiopathogenesis, and recurrence risk of amniotic band-associated anomalies. Some authors consider ABS the most appropriate term since the pattern of anomalies is secondary to disruption of heterogeneous etiology.

The estimated incidence of ABS ranges from 1:1200 to 1:15,000 in live births.[1] It affects both sexes equally. Incidence of LBWC is still rarer.[4]

Though different theories have been proposed for ABS the exact pathogenesis is not well known. The exogenous theory suggests disruption of amnion and the endogenous theory suggests vascular compromise as the etiological factor.[1] Some authors suggest a genetic predisposition also but without any conclusive evidence.[1],[7] Acquired factors such as diabetes, use of drugs, tobacco and iatrogenic factors like the sting from amniocentesis may also play an active role.[1]

The diagnostic criteria for LBWC are still being discussed, but the most commonly quoted are those originally set forth by Van Allen et al. in 1987 which implies that an infant with encephalocele, facial clefts and limb defects can be considered as having LBWC.[4],[5] This is being disputed because it would be inappropriate to make the diagnosis of LBWC in the absence of a body wall defect, which is considered as the primary anomaly.[8] Some classify LBWC into two groups: Gastroschisis, for cases with an isolated and usually small body wall defect; and BWC, for those cases with body wall defect associated with other malformations, deformations, or disruptions. Russo et al. identified two distinct phenotypes of LBWC, one with craniofacial defects, facial clefts, amniotic adhesions and ABSs called placento-cranial adhesion phenotype and the other without craniofacial defects but with imperforate anus, urogenital abnormalities, lumbosacral meningomyelocele, and kyphoscoliosis called the placento-abdominal adhesion phenotype.[8],[9] The underlying mechanism in the former is thought to be early amnion rupture and vascular disruption while, in the latter it is early embryonic dysplasia.[6] The chance of recurrence in the next pregnancy is very low. However, the association of other malformations along with body wall defect makes the prognosis very poor.[8] Associated congenital anomalies are club foot, club hand, hemangiomas, micrognathia, or omphalocele and more complex syndromes like  Patau syndrome More Details or septal optic dysplasia.[1]

Importance of our case is that features of both the phenotypes described were present, so not fitting into any one distinct category. This is very rare and extensive literature search yielded only one other case with similar morphology and ours is the second case from India. Moreover in our case, internal organ anomalies such as hepatic and retroperitoneal cysts were present, which we have not come across in any of the articles or studies on LBWC.

Due to the small size it is extremely difficult to identify these bands during routine USS. A three-dimensional ultrasound scan or magnetic resonance imaging is thought to be more useful. In some cases, bands are seen attached to the face, and this can cause cleft lip and cleft palate. Anomalies of the abdominal wall may occur as exomphalos to gastroschisis to more complex forms such as pentalogy of Cantrell.[6] Structural components of the syndrome, usually exencephaly, can be identified on second-trimester ultrasound examination; however, definitive diagnosis of LBWC is usually made during postmortem examination.[8],[10]

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Conflicts of interest

There are no conflicts of interest.

   References Top

Cignini P, Giorlandino C, Padula F, Dugo N, Cafà EV, Spata A. Epidemiology and risk factors of amniotic band syndrome, or ADAM sequence. J Prenat Med 2012;6:59-63.  Back to cited text no. 1
Torpin R. Amniochorionic mesoblastic fibrous strings and amnionic bands: Associated constricting fetal malformations or fetal death. Am J Obstet Gynecol 1965;91:65-75.  Back to cited text no. 2
Das SK, Maharana SS, Subudhi M, Rao PV. The limb-abdominal wall complex defects, a form of amniotic band sydrome: A rare case report. J Clin Diagn Res 2013;7:543-4.  Back to cited text no. 3
Baruah P, Ray Choudhury P. Limb body wall complex with sacrococcygeal mass and agenesis of external genitalia. Case Rep Med 2013;2013:218626.  Back to cited text no. 4
Van Allen MI, Curry C, Gallagher L. Limb body wall complex: I. Pathogenesis. Am J Med Genet 1987;28:529-48.  Back to cited text no. 5
Prasun P, Behera BK, Pradhan M. Limb body wall complex. Indian J Pathol Microbiol 2008;51:255-6.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
Luehr B, Lipsett J, Quinlivan JA. Limb-body wall complex: A case series. J Matern Fetal Neonatal Med 2002;12:132-7.  Back to cited text no. 7
Plakkal N, John J, Jacob SE, Chithira J, Sampath S. Limb body wall complex in a still born fetus: A case report. Cases J 2008;1:86.  Back to cited text no. 8
Russo R, D'Armiento M, Angrisani P, Vecchione R. Limb body wall complex: A critical review and a nosological proposal. Am J Med Genet 1993;47:893-900.  Back to cited text no. 9
Chikkannaiah P, Dhumale H, Kangle R, Shekar R. Limb body wall complex: A rare anomaly. J Lab Physicians 2013;5:65-7.  Back to cited text no. 10
[PUBMED]  Medknow Journal  

Correspondence Address:
Dr. P Lekshmi Devi
Palapuzha Bhavanam, Nechipuzhoor, Pala, Kottayam, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.168876

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