Answer C001

BRTS                                           
 Birmingham Radiology Training Scheme

Home
Up

Answer C001
Answer C002
Answer C003
Answer C004
Answer C005

Answer: Congenital amputation secondary to amniotic bands

 

 

The image demonstrates an example of Amniotic Constriction Band syndrome, also known as Streeter's syndrome.
The annular constriction bands can involve any part of the body and vary from minor digital deformities to reports of nerve palsies and pseudoarthroses of long bones to congenital amputations, including a case report of a congenital decapitation! The band depth varies from a superficial skin constriction to strangulation of the distal limb.
Most cases are sporadic. Streeter proposed a genetic predisposition. Recent reports suggest that interference with embryonic circulation in the first month of gestation can derange the graded expression of organizational genes resulting in local defects in the organization of the embryo. Others suggest that amniotic bands alone cause the constrictions.
Up to 50% have other congenital anomalies including cleft lip and palate and club foot deformity. Hand and finger anomalies occur in up to 80%.
There is a familial variant which is transmitted as an autosomal dominant trait and carries the eponym " Michelin tire baby syndrome" or multiple benign circumferential skin creases.
Band excision and Z or V-Y plasty repair may suffice for soft tissue lesions. Club feet in association with constriction bands are relatively rigid and difficult to treat.
A fetal lamb model of this disorder has been developed by simple banding of extremities in utero and is claimed to replicate the clinical features of the disease.

Amniotic Bands

Synonyms
Disorder Subdivisions
General Discussion
Resources

National Organization for Rare Disorders

Important
It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

bullet

Early Constraint Defects

bullet

Oligohydramnios Sequence

bullet

Streeter Anomaly

bullet

Amniotic Band Sequence

bullet

ABS

bullet

Constricting Bands, Congenital

bullet

Amniotic Band Syndrome

bullet

Amniotic Band Disruption Complex

bullet

Amniotic Rupture Sequence

Disorder Subdivisions

bullet

None

General Discussion

Amniotic bands is an abnormal condition of fetal development in which fibrous bands of tissue that originate from the amniotic sac encircle and constrict certain fetal areas, disrupting fetal growth. The amniotic sac is the thin, membranous sac that contains amniotic fluid and the developing fetus during pregnancy. Amniotic band formation may be associated with early rupture of the amniotic membrane, chronic leakage and abnormally decreased levels of the fluid surrounding the developing fetus (oligohydramnios), and abnormally decreased fetal movements. Associated structural malformations are extremely variable and may depend upon the time of amniotic band formation during fetal development. Such deformities may include abnormalities of the arms and legs (limbs), fingers and toes (digits), spine, lungs, facial area, skull, and/or abdominal and chest region.

[*] [P] Congenital Constricting Bands
from OMIM
A considerable body of observations indicated that rupture of the amnion and constriction of members which are displaced through holes in the amnion are involved. Amputated parts have been recovered in some instances ...

Congenital Constriction Band Syndrome
Southern Orthopaedic Association.
"... Congenital annular constricting bands ( CACBs ) ... incidence estimated to be between one in 5,000 to 10,000 live births ... presents itself with constriction bands, auto amputations, syndactyly, acrosyndactyly, and visceral anomalies ..." [P] Gastroschisis in association with a limb - body - wall ( LBW ) complex

[P] Presumptive limb body wall complex detected by Ultrasound - ( limb body wall )
" ... Limb body wall complex refers to combination of disruptive and lethal abnormalities which start early in the gestational process ... include cranio-facial abnormalities, scoliosis, ventral body wall defect ( thoraco - abdominoschisis ), limb deformations, short umbilical cord, and others ... include short umbilical cord syndrome, body-stalk anomaly and amniotic band syndrome ..."
 
Amniotic Band Syndrome: a population-based study in two Australian states.
by C. Bower, King Edward Memorial Hospital, Western Australia.
"A search for cases of amniotic band syndrome was made in two population-based Australian birth defects registries ... 2.03 per10,000 births ... more common in mothers younger than 25 years of age, and in first birth ..."
 
Intra-amniotic Band-like Structures
Martin Necas, RDMS, RVT
"... Band-like structures within the gestational sac are frequently noted on obstetric sonograms during the first and second trimester of pregnancy ... confusion still exists in the medical community about the precise diagnosis in these cases ... raises undue concern about the proper development ..." 

An example of Amniotic Constriction Band Syndrome
An article by Damian McCormack.
Annular constriction bands can involve anypart of the body and vary from minor digital deformities to reports of nerve palsies and pseudoartroses of long bones to congenital amputations, including acase report of a congenital decapitation!

Illustration Limb Body Wall ( LBW ) complex with constricting amniotic bands

Significance of fetography in prenatal diagnosis of limb body wall complex ( LBWC )
by H. Fukumasu, Department of Obstetrics and Gynecology, Osaka City University Medical School, Japan.
"... The major difficulty we found was severe oligohydramnios or a very complex relationship between the herniated viscera and the deformities." 

[Spanish] Caudal appendix in sequential defects of amniotic rupture
Pathology Unit of the Acapulco General Hospital.
" ... amniotic adhesion, limb body wall complex, and the presence of a pseudotail ... "  

Illustration- Abdominal Wall Defect, Stomach
Body-Stalk Anomaly.

Illustration - Lateral Abdominal Wall Complex, ( LBWC )

Illustration - Disrupted lower extremities and left hand ( LBWC )

Amniotic Bands and Sheets
" ... Abnormalities of the membrane include amniotic sheets and amniotic bands ... overall they affect around 0.8% of pregnancies ...amniotic sheets are the most common ..."

Adam Complex ( amniotic deformity, adhesion, mutilation )
"... Eight patients with the craniofacial defects and limb anomalies ofthe ADAM complex ... facial abnormalities comprise clefts and distortion and dislocation of craniofacial structures ... ADAM complex varies enormously..." 

Limb deformations in oligohydramnios sequence: effects of gestational age and duration of oligohydramnios
An article by C. Christianson, D. Huff and E.McPherson. "... The causes of oligohydramnios included premature rupture ofmembranes ( 44 cases ), fetal renal insufficiency ( 25 cases ), idiopathic ( 15cases ), and twin-twin transfusion ( 6 cases ) ..."

Lower-Limb Pediatric Prosthetics: General Considerations and Philosophy

Preventing Deformities
"... Amniotic band syndrome is a rare condition ... field of operative fetoscopy, or operations on the baby while it is still in the womb... they found that if the constriction was released, the limb could be rescued... in January 1997, Dr. Quintero and his colleagues tried the same concept on a21-week old human fetus ... the procedure was performed through an endoscope, afiber optic camera inserted into the uterus ..."

In utero Lysis of Amniotic Bands
Quintero RA; et al ... Ultrasound Obstet Gynecol 1997 Nov;10(5):316-20 ... Fetuses with amniotic band syndrome with associated limb constriction in which the amniotic band was surgically interrupted to avoid spontaneous amputation of the extremity ...

Amniotic Band Syndrome in Fetal Lambs. I: Fetoscopic Release & Morphometric Outcome
Cromblehome TM; J Pediatr Surg 1995 Jul;30(7):974-8.
Eight fetal lambs underwent banding of their extremities with umbilical tape at 100 days' gestation ... There were no differences between fetoscopically released limbs and control limbs ...

Limb Amputation in Amniotic Band Syndrome: Serial Ultrasonographic and Doppler Observations
Ultrasound Obstet Gynecol 1997 Nov;10(5):312-5 ...
Bilateral fetal lower limb edema diagnosed previously. Ultrasonography showed a constriction ring around both legs, and ... This was followed by the gradual bending, breaking and resorption of the tibia and fibula of the right leg. Between weeks 30 and 34, gradual shrinkage of the remains of the right leg beneath the knee was recorded by serial ultrasonic observations. At the 38th week of gestation, a male infant was born by normal vaginal delivery. Examination at birth revealed amputation of the right leg below the knee, with a denuded end of the stump ... This case afforded us the opportunity of inutero following of natural limb amputation in the amniotic band syndrome ...

International Child Amputee Network

Amniotic Bands
Disease Information by National Organization for Rare Disorders

Amniotic Band Sequence ( ABS )
Malcolm Cheng, MD, FAAP, FRCPC ...
B. Historical Perspective ... Hippocrates: First suggested amniotic bands as a cause of limb deformities or amputations. 1812: Chaussier described 3 cases of upper limb separation at birth ... 1975: Kino, et. al. showed haemorrhagic disruption of tissue in rats preceded limb deformation ...

Congenital Deformities

Teaching Files: The Placenta
A partial list of fetal and neonatal conditions which should lead to placental examination include congenital malformation, stillbirth, neonatal death, fetal growth retardation of any type, hydrops, meconium in amniotic fluid ...

Amniotic Band Syndrome
Luís Flávio Gonçalves, MD, Philippe Jeanty, MD, PhD ... Vanderbilt University ...
Synonyms: ADAM complex ( amniotic deformities, adhesion, mutilation ), amniotic band sequence, amniotic disruption complex, annular grooves, congenital amputation, congenital Constricting bands, streeter bands, transverse terminal ...
Definition: Amniotic band syndrome is a set of congenital malformations ranging from minor constriction rings and lymph-edema of the digits to complex, bizzarre multiple congenital anomalies that are attributed to amniotic bands that stick, entangle and disrupt fetal parts ...
Prevalence: The prevalence for live births is 7.7 :10,000; for spontaneous abortions it can be as high as 178:10.000 ...

[Ukrainian] Amniotic Constricting Band Syndrome Fact Sheet for Specialists
Pathogenesis ... Clinical picture ... Associated anomalies ... Prenatal diagnosis ... Prognosis ... Delivery management ... OMIM number

 

                    
                                       Copyright © Birmingham Radiology Training Scheme 2003 . All  rights reserved.  
                                Last modified: Wednesday December 24, 2003.