Congenital diaphragmatic hernia (CDH) refers to a hole in the diaphragm which occurred prior to birth. The problems, however, are far more complicated than simply repairing the hole.
CDH occurs in 1 in 4000 live births and survival is about 70%. The problem with CDH is that the abdominal contents within the chest prevent normal lung development. This may result in small lungs and/or lung(s) which have hypertension. Pulmonary hypertension means the blood vessels to the lungs are so tightly constricted that blood from the heart can’t flow into the lungs to pick up oxygen. While medications are available to improve pulmonary hypertension, some newborn lungs simply don’t respond. Extracorporeal Cardiac Life Support (ECLS) can be used to keep the child alive until the hypertension resolves; however, some lungs can’t recover.
Although surgery can repair the hole in the diaphragm, the abnormal lung development is the major problem. Attempts at fetal repair have been abandoned at all U.S. centers due to poor outcomes. The few fetal interventions, such as plugging the trachea to make the lung(s) grow have marginal results. This procedure is now reserved for the worst cases, generally with a LHR (lung head ratio) <0.8 (or <1.0 if the liver is in the chest). Patients with low LHRs are offered a referral to The University of California – San Francisco (UCSF) for evaluation, but many choose to deliver at an ECLS center in Phoenix for a variety of reasons.
Thoracoscopic Repair of Congenital Diaphragmatic Hernia
Using 3 small incisions, digital camera technology, and delicate suturing instruments, the first thoracoscopic repair of a congenital diaphragmatic hernia (CDH) in the state of Arizona was done at Phoenix Children’s Hospital. Thoracoscopic (minimally invasive) repair of diaphragmatic hernia has become routine for the subset of patients with adequate lung function.
The technique builds upon previous types of minimally invasive procedures which have been skillfully adapted by the pediatric surgeons at Pediatric Surgeons of Phoenix. Since 2007, many additional children have benefited from minimally invasive repairs of CDH by our surgeons. Information on current research, awareness, and support for CDH can be found at CHERUBS.
Pediatric Surgeons of Phoenix will only care for patients with prenatally diagnosed congenital diaphragmatic hernias at our ECMO (ECLS) capable hospitals — Phoenix Children’s Hospital (PCH) and St Joseph’s Hospital and Medical Center (SJHMC). Patients with prenatally diagnosed congenital diaphragmatic hernia are strongly encouraged to deliver at a center capable of offering ECMO (ECLS).
We work with both major neonatology groups in Greater Phoenix – Neonatal Associates, Ltd. (NAL) and Phoenix Perinatal Associates (PPA or Pediatrix) and coordinate with all high risk obstetricians at ECMO (ECLS) centers in Phoenix.
ECLS – Extracorporeal cardiac life support – the current terminology for ECMO. ECLS involves placing one or more large tubes in the jugular or femoral veins to remove de-oxygenated blood, oxygenate it, and then return it to the patient in a constant circuit. This type of heart/lung bypass may allow us to keep someone alive until their own lungs can support them. An alternative version of ECLS (venoarterial, or ‘VA’) places the oxygenated blood back in a major artery such as the femoral or carotid artery. This is used in patients whose heart cannot circulate the oxygenated blood adequately.
ECMO – Extracorporeal membrane oxygenation (an older, but very commonly used term) is heart/lung bypass which can keep someone alive several weeks or longer until their own lungs can support them. The name was changed because a membrane oxygenator is no longer used to oxygenate the blood. ECMO was developed at the University of Michigan by Dr Robert Bartlett.