Amniotic Band Syndrome

ABS is a group of rare congenital abnormalities that is caused by strands of the amniotic membrane attaching and tangling up with the fetus. Depending on the location and severity, amniotic bands can constrict limbs causing amputations, fuse hands and feet causing webbing of fingers and toes, and constrict other portions of the body causing severe defects. The cause of ABS is unknown. During pregnancy, there are two linings to the pregnancy sac that holds the fetus and fluid. The outer layer is called the chorion and the inner layer is called the amnion. Amniotic bands are thought to occur when the inner amniotic membrane ruptures or tears without injury to the outer layer. The strands of torn amniotic membrane float in the fluid and become tangled up with the fetus. The severity and complications of ABS present in a wide range. Bands wrapped around fingers or toes of the fetus can result in amputations or fusions (syndactyly) of those digits. Clubbed feet can result from bands on the foot. Severe constriction around arms and legs can cause major amputations. Bands that wrap around the face can cause deformities such as cleft lip or palate. These conditions are treated after the baby is born. In more severe cases, bands can cause defects in the abdominal or chest wall or cause constriction of the blood vessels for vital organs. ABS can be life-threatening if the bands wrap around the umbilical cord or the neck. It is unclear how often ABS occurs as it is difficult to diagnose. Best estimates suggest that ABS occurs in approximately 1 in 1,200 to 15,000 live births.

How is ABS diagnosed?

ABS is very difficult to diagnose. Prenatal ultrasound may not be able to identify the bands but only see the results of the fusion such as missing or deformed limbs.

What will happen during pregnancy?

If ABS is suspected, a detailed ultrasound will be performed to detect the presence of anomalies. In some cases a fetal echocardiogram to examine your baby's heart may be recommended.

Will a fetal treatment be required?

Fetal treatment for ABS is rarely necessary. In certain conditions, amniotic bands may need to be cut during pregnancy if they are wrapped around the

umbilical cord or threaten to amputate a limb. If it is determined that fetal intervention is the best option for your baby, your team at the Center will develop a comprehensive plan for you and your baby. Cutting the amniotic bands is performed utilizing a fetoscopic technique. A small camera is inserted through the mother's abdomen and uterus into the amniotic sac in order to see and cut the bands. The procedure is typically performed under regional anesthesia. Afterwards, you will be admitted to the Women's Center at Children's Memorial Hermann Hospital to monitor for complications. After discharge, you will continue to be followed by the Center and your obstetrician for ongoing care.

What special considerations should be made for delivery?

Type of delivery - Typically, pregnancies with ABS do not require cesarean delivery. The need for fetal intervention should not impact your mode of delivery. The delivery plan will be carefully discussed with you and your obstetrician. Place of delivery - If all the prenatal monitoring suggests that your baby is doing well, the baby can be delivered at the hospital of your choice. However, the hospital should be prepared to handle any immediate needs of your newborn and have a neonatal intensive care unit with the capability to provide specialized care. Time of delivery - Unless there are signs of serious complications of ABS, there is no reason to intentionally induce an early delivery. The team at the Center may recommend early delivery for pregnancies that appear to be in danger.

What will happen at birth?

Immediately following delivery, doctors will carefully evaluate and begin treatment if necessary. You and your loved ones should be able to immediately hold and bond with your newborn. If needed, the baby will be stabilized in an intensive care unit and treated by the neonatologists, specialists in high-risk newborns.

What are the long-term outcomes and considerations?

A baby with ABS may need treatment after birth. Most of the complications of ABS are not life-threatening and may be treated on an elective basis. Depending on the complications, some babies may need corrective surgery in order to improve cosmetic features or functionality of hands and feet. These conditions are typically treated by pediatric orthopedic and plastic surgeons.

Your team at the Center will develop a comprehensive treatment plan and introduce you to the specialists that treat these conditions.

Amniotic Band Syndrome is a group of congenital birth defects believed to be caused by entrapment of fetal parts (usually a limb or digits) in fibrous amniotic bands while in utero. In other words: Before the baby was born the body parts that shows signs of ABS (arm, fingers, toes, etc.,) were caught up and entangled in string-like bands. This caused abnormalities that were present at birth.

What Causes Amniotic Band Syndrome?

Amniotic Band Syndrome is not genetic (i.e. not inherited). It is extremely unlikely that ABS will affect a future pregnancy. To date, no prenatal factors have been associated with ABS. The commonly accepted view is that ABS occurs when the inner membrane (amnion) ruptures without injury to the outer membrane (chorion), this exposes the baby to fibrous sticky tissue (bands) from the ruptured amnion which can float in the waters of the uterus, these fibrous tissues can entangle the baby reducing blood supply and causing congenital abnormalities. In some cases a complete natural amputation of a digit(s) or limb may occur before birth or the digit(s) or limbs may be necrotic (dead) and require surgical amputation following birth. A strong relationship between ABS and clubfoot exists. A 31.5% of associated clubfoot deformity and ABS can be correlated with 20% occurring bilaterally. Other abnormalities found with ABS include: clubhands, cleft lip, and/or cleft palate, and hemangioma.

Effects of Amniotic Band Syndrome

With Amniotic Banding no two cases are exactly alike. There are several features that are relatively consistent: syndactyly (webbed digits), distal ring constrictions, deformity of the nails, stunted growth of the small bones in the digits, limb length discrepancy, distal lymphedema (swelling), and congenital band indentations. If a band wraps tightly around a limb, the limb can actually be completely amputated. If the band is across the babys face it can cause cleft palate. In a large number of cases the baby is also born with clubfeet. ABS is also the cause of numerous miscarriages, such as when a band becomes wrapped around the umbilical cord. In this photo of Evans hand you can see the banding line from his wrist to his pinky finger, he also has a banding line around the wrist. This photo is after two operations that separated his webbed fingers (syndactyly). His three fingers are small, with abnormal finger nails, and missing some joints, there is also some lymphedema (swelling at the joint) of the index finger middle joint. There is poor circulation in the ring finger as there were no major arteries in it at the time of surgery. Even on a hot July day, if you hold Evans hand you can feel how much colder this finger is then the rest of his hand. Read some of the ABS stories to see how each case is different.

Amniotic Band Syndrome Alternative Names

Some of these are different names for ABS and some are other syndromes that are often confused with ABS. Misdiagnosis is apparently common. Amniotic Deformity, Adhesions and Mutilations Amniotic band disruption complex or sequence Amniotic bands and sheets Annular constriction bands Congenital ring constriction Constriction Band Syndrome and Amniotic Constriction Band Syndrome Intrauterine amputation Streeters dysplasia

TEARS (The Early Amnion Rupture Spectrum) Congenital Transverse Defects or Deficiency Limb-body Wall Complex

Treatment Options
Treatment depends on which body part the band was wrapped around and the severity of the abnormality it caused. In Utero Surgery - has been performed to free limbs from amniotic bands that threaten to amputate limbs. Results of the first surgeries of this type were published in the Journal of Ultrasound in Obstetrics & Gynecology. It is the first minimally invasive treatment to correct a non-lethal fetal condition. Check out these links: Read Thomass Story The Center for Fetal Diagnosis and Treatment Treating Syndactyly - Evan had two plastic surgeries to separate his webbed fingers. A great description of syndactyly and what surgery does can be found at HandWorld Treatment of Congenital Amputation - War Amps Site Growing Hands -Distraction Augmentation Manoplasty The technique pioneered by Dr. Cowen grows up to one inch of bone per month to create palms and lengthen fingers. A strange looking contraption of steel rods, pins, and screws is inserted through the existing bone. Screws are turned at an even rate several times a day to achieve growth of up to one millimeter a day. Turning the screws takes up slack and the body responds to this stress by growing bone. Botei. com One of Dr. Cowens patients Josefas history; a girl that was born with missing fingers and toes. Explains the long treatment to reconstruct the fingers of her hands. Treating Cleft Lip and Palate National Cleft & Craniofacial Awareness Treating Stabismus - Strabismus.org Treating Clubfoot- The Clubfoot Club (our other site)A strong relationship between ABS and clubfoot exists. A 31.5% of

associated clubfoot deformity and ABS can be correlated with 20% occurring bilaterally. Clubfeet are now treated nonsurgically using the Ponseti Method