Singhania N*, Joshi A**, Gupta AS***.
(* Junior Resident, ** Senior Resident *** Professor. Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)
A multigravida woman presented to us with a fetal MRI showing amniotic band towards the left lateral uterine wall with no gross anomaly. There was a progressive reduction in the thickness of the amniotic band over the antenatal period. She went into spontaneous labor at term and delivered, a healthy male child of 3.436 kg, with no limb or any other gross anomalies. Pregnancy with amniotic band is a rare occurrence; one such case has been reported here.
Amniotic band is a rare clinical entity which can present with a wide range of features in the fetus from multi organ involvement ranging from complete absence of an extremity to rarely no abnormality at all. Here, we present a case of a multigravida with confirmed amniotic band on fetal MRI with no specific fetal defects. The incidence of amniotic bands, associated dilemmas and the role of fetal MRI will be discussed.
A 29 year old woman, married since 3 years, Gravida 3, MTP 1, Abortion 1 registered in our hospital at 8.3 weeks of gestation. She had regular antenatal follow up. At 16 weeks of gestation routine obstetric imaging was done suggestive of a small synechiae on left side of the uterine wall in the antero-posterior and cephalo-caudal direction measuring 2.5 cm in length, partially dividing the amniotic cavity. Fetal parts and placenta were seen on either side of it. She was advised a fetal MRI, which was suggestive of a linear T2 hypointense band towards left lateral wall at mid-corpus extending between anterior and posterior myometrium. A 1-2 mm thick amniotic band was going from placenta to anterior wall of gestational sac, one fetal foot was seen lateral to it (Figure 1). However, limb movements were normal. She was followed up regularly in the antenatal period. A repeat ultrasonography done at a later date showed no obvious fetal limb mal-positioning or gross anomaly. All other routine investigations were within normal limits. Her antenatal course remained uneventful. She went into spontaneous labor at term and delivered at 39.5 weeks. An outlet forceps was applied in view of prolonged second stage of labor. She delivered a male child of 3.436 kg with an APGAR score of 9/10 at 1 minute and 5 minute. Baby had good cry with no limb or any other gross abnormalities detected. She was discharged 48 hours post-delivery.
Amniotic band syndrome (ABS) is a collection of congenital anomalies which can range from minor constriction bands to major deformities which are incompatible with life. This entity has remained a dilemma and has been extensively studied for years. The incidence ranges from 1:1200 to 1:15000 live births and 178:10000 spontaneous abortions. The organs affected depend upon the location of the amniotic band. There are several different terminologies used to describe this entity, the commonly used terminologies include amnion rupture sequence, aberrant tissue band syndrome, ADAM (amniotic deformity, adhesions, mutilations) complex, constriction band syndrome and Streeter's dysplasia. The exact etiopathogenesis of ABS is still unknown. ABS is not of a genetic origin since it has not been found to occur in siblings. Maternal trauma, oophorectomy during pregnancy, amniocentesis, intrauterine devices and connective tissue disorder (Ehler Danlos Syndrome) are found to play some role in its etiopathogenesis.[5,6,7,8]
There have been numerous theories proposed for the etiopathogenesis of this condition. Two of the most widely acknowledged theories are the‘Extrinsic’ and ‘Intrinsic’ Theory. Amongst the two, the most widely accepted theory is the extrinsic theory (amniotic disruption), proposed by Torpin in 1965. According to this theory, the occurrence of an amniotic rupture leads to loss of amniotic fluid with extrusion of all or some parts of the fetus into the chorionic cavity. This subjects the fetus to compression leading to entanglement of fetal limbs or other body parts in the remnants of the amnion. The type of anomalies that occur depend on the timing of amniotic rupture during pregnancy as well as the site and the degree of compression of the underlying structures. According to Higginbottom et al, the damage that takes place before 45 days of gestation leads to severe facial clefts and severe defects of the brain and calvarium, while damage after 45 days of gestation resulted in limb involvement without facial and central nervous involvement. Whereas Streeter's intrinsic (embryonic dysplasia) theory suggests that fetal disruptions result from imperfect histogenesis.
The most common fetal part affected are the limbs, these present as digital amputations, constriction rings or acrosyndactyly. Other uncommon defects noted include talipes equinovarus, scoliosis, craniofacial abnormalities, such as cleft lip or cleft palate, orbital defects, corneal abnormalities, body wall defects, and internal organ abnormalities.[3,12] It has been noted that even a single constriction band can lead to digital or extremities amputation or may affect the growth of a particular body part. An amniotic band can be diagnosed on ultrasonography (USG) as early as 12 weeks of gestation. The USG diagnostic criteria for amniotic bands are appearance of linear echoes floating in amniotic fluid, constriction rings on extremities and irregular limb amputations. A fetal MRI is more sensitive and helpful in diagnosing difficult cases. The treatment of amniotic bands is surgical which should have a multidisciplinary approach and should be individualized for each case. Prenatal treatment of ABS has also been attempted in the form of fetoscopic laser cutting of amniotic bands, before their compression causes malformation.
Amniotic band is a rare occurrence in pregnancy. The exact mechanism of its occurrence still remains unanswered. Owing to the wide range of clinical presentations, this condition warrants need for proper patient education and routine antenatal follow-up to ensure a timely detection.
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Singhania N, Joshi A, Gupta AS. Pregnancy outcome with an amniotic band. JPGO 2018. Volume 5 No.10. Available from: http://www.jpgo.org/2018/10/pregnancy-outcome-with-amniotic-band.html