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Amniotic Band Syndrome

Amniotic Band Sequence

Amniotic band syndrome is still incompletely understood abnormality in which the fibrous bands entrap various parts of the fetal body, including the limbs, the trunk, the abdomen, as well as the cranium. The severity of injuries ranges from mild constrictive changes on the skin to mutilations and complete amputations. As the condition may be life-threatening, prenatal imaging studies (mainly ultrasonography) are necessary for making an early diagnosis.


Presentation

The clinical presentation of amniotic band syndrome stems from the interaction of the fetus with the abnormal fibrous tissue within amniotic cavity [1]. Virtually all cases display a unique collection of manifestations, ranging from only mild cutaneous signs to severe and life-threatening injuries [2] [3]. Almost any anatomical region in the body can be entrapped by amniotic bands, as studies have reported the head, the trunk, and both proximal and distal extremities as potential sites where defects have occurred [2] [4] [5] [6]. Limb abnormalities, however, seem to be the most common - focal constriction, brachydactyly, and peripheral nerve damage are typical cases, whereas injuries resulting in mutilations and total amputations are also possible [1] [2] [4] [5] [7]. In more recent studies, approximately 50% of patients showed isolated mutilation and/or constriction of distal parts of fingers and toes, without the presence of any additional abnormalities [7]. Typical defects of the trunk are related to the abdominal organs, where gastroschisis and gastropleuroschisis may be encountered, while spinal deformities, such as kyphosis, lordosis, and scoliosis are also frequently discovered in amniotic band syndrome [1]. In fact, the concomitant presence of spinal pathologies and defects of the abdominal wall are considered to be highly specific for amniotic band syndrome [1]. Newborns are could have different injuries of the cranium and face - acalvaria, acrania, encephalocele, microphthalmia, facial clefts, and nasal deformities [2] [3] [7].

Pleural Effusion
  • Fetal examination revealed a large cystic hydroma, generalized edema with pericardial and pleural effusions, and Turner's syndrome.[ncbi.nlm.nih.gov]
Brachydactyly
  • Limb abnormalities, however, seem to be the most common - focal constriction, brachydactyly, and peripheral nerve damage are typical cases, whereas injuries resulting in mutilations and total amputations are also possible.[symptoma.com]
  • The birth prevalence rate of ABS has been reported to be 0.89 per 10, 000 births.[ 2 ] The most common defects associated with ABS are limb defects such as focal constrictions, amputations, psuedosyndactyly, and brachydactyly.[ncbi.nlm.nih.gov]
Strabismus
  • In two cases, the strabismus was accompanied by other ocular manifestations, while in one patient strabismus and amblyopia were the sole ophthalmological findings.[ncbi.nlm.nih.gov]
  • J Pediatr Opthalmol Strabismus. 1991;28:238–239. Google Scholar 12. BenEzra D, Frucht Y, Paez JH, Zelikovitch A. Amniotic band syndrome and stabismus. J Pediatr Ophthalmol Strabusmus. 1982;19:33–38. Google Scholar 13.[link.springer.com]
Unilateral Epiphora
  • One of them presented with telecanthus, syndactyly, amputated toes, and unilateral epiphora diagnosed as congenital nasolacrimal duct obstruction.[ncbi.nlm.nih.gov]
Vaginal Bleeding
  • Interestingly, women living in high altitudes were shown to be at a much higher risk for carrying fetuses who develop this syndrome, whereas a history of febrile illnesses, use of pharmacological agents, and vaginal bleeding in the first trimester were[symptoma.com]
  • bleeding in the first trimester.[ 2 ] In our case series, all three women were primipara.[ncbi.nlm.nih.gov]
Cervical Incompetence
  • The pregnancy was complicated by cervical incompetence. Restriction of fetal head movement was important in sonographic diagnosis.[ncbi.nlm.nih.gov]
Profound Mental Retardation
  • We describe a 9-year-old girl with typical features of constriction band syndrome localized to the lower limbs who had also profound mental retardation and drug-resistant epilepsy associated with bilateral periventricular nodular heterotopia (a brain[ncbi.nlm.nih.gov]
Meningism
  • His postoperative course was complicated by meningitis with subsequent hydrocephalus necessitating ventriculoperitoneal shunt placement.[ncbi.nlm.nih.gov]
Foot Drop
  • Recently, he developed foot-drop due to pressure on the right common peroneal nerve by the constriction ring of the leg.[ncbi.nlm.nih.gov]
Paresis
  • All three cases demonstrate a paralytic strabismus due to a unilateral paresis-paralysis of the medial rectus in one case and of the superior rectus in another. The third case showed a bilateral lateral rectus paralysis.[ncbi.nlm.nih.gov]

Workup

Since it is necessary to evaluate the extent of limb and organ damage as soon as possible for the purposes of planning the optimal treatment, the diagnosis of amniotic band syndrome should be made prenatally. Fetal ultrasonography is regarded as the cornerstone for detecting edema of the limbs or fingers distally from where the amniotic band is located [4]. A prenatal diagnosis may be difficult, however, particularly in the earlier stages. More advanced imaging studies, including 4D ultrasonography and magnetic resonance imaging (MRI), have been also described as potentially useful methods [4] [5].

To raise a clinical suspicion, several risk factors have been proposed. Although no hereditary components of the disease are described, amniotic band syndrome is much more likely to develop in the setting of a positive family history [6]. As mentioned previously, several congenital abnormalities are associated with this condition such as cleft lip, cleft palate, and micrognathia, but also specific genetic syndromes such as Patau syndrome [6]. Interestingly, women living in high altitudes were shown to be at a much higher risk for carrying fetuses who develop this syndrome, whereas a history of febrile illnesses, use of pharmacological agents, and vaginal bleeding in the first trimester were observed as significant maternal factors [1] [6].

Pleural Effusion
  • Fetal examination revealed a large cystic hydroma, generalized edema with pericardial and pleural effusions, and Turner's syndrome.[ncbi.nlm.nih.gov]

Treatment

  • Given many morphological variations of the syndrome, a decision on the strategy of treatment should be made individually for each patient.[ncbi.nlm.nih.gov]
  • Michael Harrison, the Fetal Treatment Center was the first institution to develop fetal surgery techniques. The first open fetal surgery in the world was performed at UCSF over 2 decades ago.[web.archive.org]
  • Treatment for amniotic band syndrome Treatment for ABS depends on the severity of the symptoms. Most cases are relatively mild, have an excellent prognosis, and do not require treatment.[prairiepodiatry.com]

Prognosis

  • In the patient with distal edema and acrosyndactyly, early repair portends better prognosis. Improvements in prenatal diagnosis and fetoscopic surgical technique may eventually allow treatment of amniotic band syndrome in utero.[ncbi.nlm.nih.gov]
  • (Outcomes/Resolutions) The prognosis depends on the severity of the findings. In minor cases, the prognosis of Amniotic Band Syndrome is excellent.[dovemed.com]
  • Treatment and prognosis The prognosis is extremely variable, depending on the part that becomes entrapped.[radiopaedia.org]

Etiology

  • […] of early amniotic rupture in one sac of a dizygotic twin gestation without subsequent fetal abnormalities, and the paradoxical observations of discordance in monoamniotic and concordance in diamniotic twin gestations, fail to support an "exogenous" etiology[ncbi.nlm.nih.gov]
  • If the amniotic bands are swallowed while still partially attached to the placenta, the tether may lead to bizarre facial clefts and palate deficiencies. [10] Etiology Two main lines of thought exist regarding the etiology of ABS, attributing the condition[emedicine.com]

Epidemiology

  • Epidemiology and risk factors of amniotic band syndrome, or ADAM sequence. J Prenat Med. 2012; 6 :59–63. [ PMC free article ] [ PubMed ][ncbi.nlm.nih.gov]
  • Epidemiologic analysis of maternal factors and amniotic band defects. Birth Def Res (Part A). 2003;67:68-72 Hunter AG. A pilot study of the possible role of familial defects in anticoagulation as a cause for terminal limb reduction malformations.[rarediseases.org]
  • Paediatric and Perinatal Epidemiology 1993;7(4):395-403. Narang M, Nidhi, Danewa A. Streeter’s Dysplasia. Journal of Case Reports 2011;1(1):16-17.[casereports.in]
  • One of the few epidemiologic studies is a 1988 study from Atlanta that cites the incidence as 1.16 cases per 10,000 population. No sex predilection is recognized. The incidence overseas is similar to that in the United States.[emedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • It's presentation is variable in both type and severity, and pathophysiology and etiology still mystify those who have studied it in depth. Although better understood today, there remain many unanswered questions.[ncbi.nlm.nih.gov]
  • Pathophysiology The developing embryo sits within two cavities, the amnion and the chorion.[emedicine.com]

Prevention

  • A small, free-floating amniotic band may have lodged in the fetal uveal cleft at 5 to 6 weeks of gestation, preventing closure of the cleft and thus resulting in this first reported instance of an association between amniotic band syndrome and an isolated[ncbi.nlm.nih.gov]
  • Currently, there are no definitive methods available to prevent Amniotic Band Syndrome Prenatal monitoring using fetal ultrasound may help detect the condition early.[dovemed.com]

References

Article

  1. Burton DJ, Filly RA. Sonographic diagnosis of the amniotic band syndrome. Am J Roentgenol. 1991;156:555–558.
  2. Padmanabhan LD, Hamza ZV, Thampi MV, Nampoothiri S. Prenatal diagnosis of amniotic band syndrome. Indian J Radiol Imaging. 2016;26(1):63-66.
  3. Chandran S, Lim MK, Yu VY. Fetal acalvaria with amniotic band syndrome. Arch Dis Child Fetal Neonatal Ed. 2000;82:F11–13.
  4. Shetty P, Menezes LT, Tauro LF, Diddigi KA. Amniotic Band Syndrome. Indian J Surg. 2013;75(5):401-402.
  5. Paladini D, Foglia S, Sglavo G, Martinelli P. Congenital constriction band of the upper arm: the role of three-dimensional ultrasound in diagnosis, counseling and multidisciplinary consultation. Ultrasound Obstet Gynecol. 2004;23:520–522.
  6. 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(4):59-63.
  7. Orioli IM, Ribeiro MG, Castilla EE. Clinical and epidemiological studies of amniotic deformity, adhesion, and mutilation (ADAM) sequence in a South American (ECLAMC) population. Am J Med Genet A. 2003;118A:135–145.

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Last updated: 2019-06-28 10:35