Congenital Diaphragmatic Hernia / CDH is a defect in the diaphragm of the fetus. Most often (88 percent) Congenital Diaphragmatic Hernia / CDH is a posterolateral defect in the left hemidiaphragm on one side. This leads to herniation of the viscera into the thorax, resulting in pulmonary hypoplasia and respiratory embarrassment.
Associated anomalies are seen in 25 to 57 percent of all cases of Congenital Diaphragmatic Hernia / CDH and 95 percent of stillborns with Congenital Diaphragmatic Hernia / CDH.
These anomalies include congenital heart defects, hydronephrosis, renal agenesis, intestinal atresia, extralobar sequestrations and neurologic defects, including hydrocephalus, anencephaly, and spina bifida.
Chromosomal anomalies, including trisomy 21, 18, and 13, occur in association with Congenital Diaphragmatic Hernia / CDH in 10 to 20 percent of cases that are diagnosed prenatally.
The Cincinnati Children's Fetal Care Center CDH Team offers a unique management strategy that begins with the prenatal Congenital Diaphragmatic Hernia / CDH prognostic profile and continues through delivery, care in the NICU and post-discharge and long-term follow- up with continuity of care provided by the same team of physicians. Principals that characterize the CDH Team management strategy include:
The Congenital Diaphragmatic Hernia / CDH Team includes an experienced multi-disciplinary team of:
Our family-centered approach encourages parents to attend and participate in every aspect of their child’s care, including family-centered rounds, which are conducted daily at the patient’s bedside.
Our CDH Team works closely with referring obstetricians and maternal-fetal medicine specialists to facilitate prenatal management. We also work closely with pediatricians who will be assuming primary care once the baby has been discharged home.
This integrated approach has achieved survival rates for isolated CDH of 92 percent, and 100 percent for fetuses with LHR ≥ 1.0.
The long-term outcome of infants with Congenital Diaphragmatic Hernia / CDH who survive the neonatal period depends on the severity of the underlying pulmonary hypoplasia, the severity of pulmonary hypertension and the degree of chronic lung disease resulting from long-term breathing support.
Also affecting the long-term outcome are other health conditions seen in up to 20-30 percent of infants with Congenital Diaphragmatic Hernia / CDH. Such conditions can include neurological problems, reactive airway disease, hearing loss, seizures and developmental delay.
Gastroesophageal reflux may affect as many as 50-62 percent of Congenital Diaphragmatic Hernia / CDH survivors. Musculoskeletal problems affecting the stability of the trunk can also develop. Some infants who survive Congenital Diaphragmatic Hernia / CDH fail to thrive, suffering feeding difficulties that can result in 30 percent remaining below the fifth percentile in weight despite optimal caloric intake.
Although not all infant survivors of Congenital Diaphragmatic Hernia / CDH develop these problems, our follow-up programs are focused on early intervention when problems are recognized to optimize patient outcomes.
For more information or to request an appointment, contact the Cincinnati Children's Fetal Care Center at 1-888-338-2559 or use our online form.