Maternal-Fetal Medicine Center
The Maternal-Fetal Medicine Center at John R. Oishei Children's Hospital provides comprehensive, multidisciplinary care for women and families whose pregnancy is complicated by fetal abnormalities. As the regional quaternary referral center for complicated pregnancies in Western New York, we provide coordination of care within a single site to ease the burden to families.
- Maternal ultrasound
- Intrauterine Fetal Transfusions
- Fetal ECHO
- Genetic counseling
- Pediatric surgical specialist consultation
- Counseling regarding short and long term outcome expectations
- Psychological and social support
- Assistance to financial resources
Complications treated include, but are not limited to:
Abdominal wall defect
Abdominal wall defect occurs when the baby’s anterior abdominal wall has weaknesses from not properly forming during development, they are considered a congenital defect. These weaknesses allow abdominal organs to protrude through the abdominal wall and develop outside of the abdomen. Thus at delivery the organs that are usually within the abdomen are outside. There are many different types of abdominal wall defects the two most common are gastroschisis and omphaloceles.
- Airway obstruction
- Bladder exstrophy
- Bladder outlet obstruction (posterior urethral valve syndrome)
- Cardiac defects
- Cleft lip and/or palate
- Cloacal deformities
Clubfoot is an abnormal shortening of the muscles and tendons in your baby’s foot, causing the baby’s foot to be twisted at a sharp angle to the ankle, somewhat resembling a golf club. It can be mild or severe, and is usually not associated with any other problems in your newborn. Half of the babies who have clubfoot have it in both feet. If it is not corrected, it will make it hard for your baby to walk.
How is clubfoot diagnosed?
Clubfoot can be seen on ultrasound. Sometimes, due to the position of your baby in the ultrasounds, it is not seen before the baby is born. It is very easy to diagnose at birth, if it is not seen on an ultrasound before the baby is born. Occasionally, after the baby is born, he or she will need x-rays of his or her feet. If it is seen on an ultrasound, you will probably be put in contact with an orthopaedic surgeon (bone doctor), and maybe a genetic counselor, if your doctor thinks it might be part of a syndrome.
How do we fix club foot?
Because babies are so flexible, treatment for clubfoot usually starts within 1-2 weeks after the baby’s birth, so that the baby’s foot can be put back in the right position before he or she starts to walk. The most common treatment for clubfoot is stretching and casting, also known as the Ponseti Method. To do this, the baby will have his or her foot moved into the correct position, and then put into a cast. This cast will probably be replaced once or twice per week. After the casting, your baby may require surgery to lengthen his or her Achilles tendon. After the foot is back in position, you may need to do stretching exercises with your baby, and he or she may need to wear special braces or shoes. If this method does not work, there are other techniques that can be used, and if none of the traditional methods work, surgical correction is an option. In the majority of cases, your baby will most likely go on to walk and run normally, and wear normal shoes.
- Congenital diaphragmatic hernia
- Congenital Pulmonary Airway Malformations (CPAM)
- Dandy-Walker malformations
- Esophageal atresia/tracheoesophageal fistula
- Fetal Isoimmunization
Gastroschisis is the extrusion of bowel through a weakening in the abdominal wall usually to the right of the umbilical cord that is not covered unlike an omphalocele. With gastroschisis there is an increase risk of premature (before 37 weeks of gestation) delivery 60% of the time, intrauterine growth restriction in 50%, and stillbirth in 8-10%.
What causes Gastroschisis?
- Advanced maternal age (greater than or equal to 35 years of age)
- Pelvic infections, such as chlamydia, gonorrhea, trichimonas, urinary tract infection at time of conception
How is Gastroschisis diagnosed?
Seen during the first trimester screening ultrasound or during the anomaly ultrasound done in the second trimester of pregnancy
Management of Gastroschisis
- Serial ultrasounds to evaluate and detect any bowel problems.
- When the baby is delivered it needs to be done at a hospital that can offer a high level of care. The baby can be delivered vaginally. Cesarean sections are recommended only for breech presentation or complications in labor
- Once delivered emergent surgical care is necessary
- Lymphatic malformation (cervical, thoracic)
- Neural tube defects (not amenable to prenatal closure)
Omphalocele is a weakness in the central abdominal wall in which the abdominal organs protrude through into the base of the umbilical cord. The abdominal contents are bound within a thin membrane. The contents can consist of just small bowel to small and/or large bowel, liver, spleen, bladder, and stomach. With an omphalocele 50% also have cardiac defects, and40% of the time have gastrointestinal defects. There is increased rate of premature (before 37weeks) births, stillbirth, and neonatal death due to associated anomalies.
What causes an omphalocele?
In 30-40% of fetuses it is caused by genetic abnormalities. There are syndromes that have omphaloceles: Beckwith-Wiedemann syndrome, Pentalogy of Cantrell, OEIS complex.
How are Omphaloceles diagnosed?
Seen on the anomaly ultrasound conducted in the second trimester
Management of Omphaloceles
- Once seen on ultrasound other physiological defects are looked for. In order to diagnose if there are genetic abnormalities a test called an amniocentesis can be done.
- When the baby is delivered it needs to be done at a hospital that can offer a high level of care. The baby can be delivered vaginally. Cesarean sections are recommended if the omphaloceles are very large or they contain the liver.
- Following delivery surgical repair is dependent upon the size of the omphalocele.
Rh disease is a problem between your blood type and your baby’s blood type – when an Rh negative mother and a Rh positive father have an Rh positive baby. Sometimes, during pregnancy, your baby’s blood is able to enter your bloodstream. In an Rh negative mother, the baby’s blood causes a reaction where the mother makes antibodies against her baby’s blood. These antibodies are able to get through the placenta and can destroy the baby’s blood. This is bad for your baby, and in the worst cases, can lead to your baby’s death. This will not happen in your first pregnancy, but it can happen in your next pregnancies.
How do we test for Rh disease?
At the beginning of your pregnancy, your doctor will do a blood test on you to see your blood type and whether or not your blood has any antibodies to your baby’s blood. The antibody that your doctor is looking for is called anti-D antibody.
What do I do if I have Rh disease?
If you have Rh disease, your baby will have a special kind of ultrasound test called middle cerebral artery Doppler monitoring. It is a test where we measure the speed of the blood moving through an artery in your baby’s brain. It is not an invasive test, and it will probably be done every week. The antibody levels in your blood may also be tested. If we find that your baby does have severe anemia, he or she may need a blood transfusion before birth. After the baby is born, he or she may need to be monitored more closely for jaundice, and may require blood transfusions after he or she is born as well.
How do I prevent Rh disease?
If you have Rh negative blood, you will receive an injection of RhoGAM at 28 weeks of gestation. After you deliver, they will test your baby’s blood type, and if he or she is Rh positive, you will get another dose of RhoGAM. If he or she is Rh negative, you will not get anymore RhoGAM.
- Sacrococcygeal teratoma (cervical, sacral, or other location)