After experiencing an uneventful pregnancy to date, Charissa Howe was shocked when her obstetrician informed her that her 21-week ultrasound had revealed something was wrong.
Diagnosis of Amniotic Band Syndrome
Charissa was told that the ultrasound had revealed amniotic band syndrome (ABS), a group of congenital abnormalities caused by bands of amnion that attach to the fetus. These bands may cause anatomical deformities, by attaching to or constricting parts of the fetus, or death, by constricting the umbilical cord. This constriction can lead to webbing of fingers and toes, amputation of limbs and severe defects of the head and face, spine, umbilical cord and body wall.
There are several theories on the cause of ABS. The most widely accepted cause is a rupture of the amnion occurring early in gestation. The fibrous bands of amnion that occur from the amniotic rupture may encircle the limbs, fingers or toes, resulting in tourniquet-like defects which, over time and fetal growth, progress to amputations.
A subsequent 3D ultrasound on Charissa revealed constriction of the left wrist by a band causing swelling in the baby’s left hand, and three fingers on the right hand appeared fused by a band. Additionally, there was concern that the umbilical cord was being constricted by an encircling band. It was recommended that Charissa continue to be monitored by ultrasound and wait for the child to be born.
Charissa and her husband instead sought a second opinion at another institution in their home state of Pennsylvania. The MFM physicians there concluded that the almost certain loss of the baby’s left hand along with the risk posed by the possible constriction of the umbilical cord warranted immediate action. They advised immediate treatment and recommended the Howes travel to Cincinnati for evaluation at the Cincinnati Children's Fetal Care Center.
Evaluation at the Fetal Care Center
The Howes made the trip the following day, and Charissa underwent a fetal MRI and a level II ultrasound at 22 weeks’ gestation. These diagnostic imaging studies confirmed ABS, resulting in swelling of loops of abnormally clustered umbilical cord and a threatened amputation of the left hand.
The indications for fetal surgery in ABS may be either a life-threatening condition if it involves constriction of the umbilical cord or, more commonly, threatened limb amputation. Since both of these conditions were present and both the mother and baby were in excellent health, doctors at the Fetal Care Center decided they were good candidates for fetoscopic surgery to release the bands.
Fetoscopic Surgery for Release of Bands
Two days after the evaluation and consultation, the fetoscopic surgery was performed by fetal surgeons Timothy Crombleholme, MD, and Foong-Yen Lim, MD. The constricting amniotic bands were released by severing the band with a laser. The surgery successfully relieved the ongoing constriction around the left wrist and umbilical cord.
Charissa was able to return home to the care of her referring OB physician in Pennsylvania within a few days of the surgery. Six weeks after fetoscopic surgery, her son, Levi, was born at 28 weeks gestation by C-section due to premature rupture of membranes. After seven weeks in the NICU, Levi was discharged on Oct. 5, 2008. He has been quickly reaching developmental milestones ever since. Charissa reports that Levi, 7 months old, has no major deficits in hand function. He will need a series of cosmetic surgeries in the future to free up the fused fingers on his right hand and may need removal of scar tissue on his left wrist caused by the initial presence of the bands.
Contact the Fetal Care Center
If you would like to request an appointment or get more information about the Cincinnati Children's Fetal Care Center, call us at 1-888-338-2559 (1-888-FETAL59).