The Full Story on CPAMs

CPAMs are abnormally developed lung tissue that doesn't participate in gas exchange, coming off normal bronchial airways but lacking normal alveoli.

Prenatal steroids (betamethasone 12mg given twice, 24 hours apart) have been shown to resolve hydrops and reduce the size of CPAM lesions such that the CVR can decrease.

A thoracoamniotic shunt is placed so that half is inside the fetal chest and half is in the amniotic space, allowing egress of fluid from the fetal chest into the amniotic space for continued decompression throughout pregnancy.

Fetal resection involves partially delivering the fetus, performing a thoracotomy, removing part of the lung, and placing the fetus back in the uterus to continue the pregnancy.

Mirror syndrome means the mother is showing evidence of what the baby has going on, with the mother developing significant edema, pleural effusion, and other symptoms very quickly.

During an EXIT procedure, the baby remains attached to mom through the umbilical cord while a thoracotomy and resection are performed, with the time available depending on how well mom tolerates it and uterine relaxation, typically ranging from a few minutes to rarely longer than an hour.

Air trapping can occur where air enters the CPAM area as the infant breathes in but doesn't come out as easily, causing progressive hyperinflation of the non-functioning lobe and rapid progressive respiratory distress.

Features suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation.

Features suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis.

Dr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that.

If a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications.

Single lung ventilation for thoracoscopic lobectomy is obtained by mainstem intubation of the contralateral bronchus, preventing the lung from overinflating during dissection of key vessels or structures.

For thoracoscopic lobectomy, the scope port should be placed over the major fissure in the mid-axillary line, anterior to the tip of the scapula, to allow working from front to back and avoid working in paradox.

When completing an incomplete fissure during lobectomy, work through it layer at a time like finger fracturing during liver lobectomy, starting at the front and working posteriorly until exposing the pulmonary artery.

Vascular control in thoracoscopic lobectomy is everything; dissect out vessels to get enough length to make a seal proximally and distally, then cut partway between seals to check for bleeding before completing division.

The bronchus sits right underneath the pulmonary artery and can be felt to help dissect behind the artery during lobectomy.

When dissecting behind the bronchus during lobectomy, stay hard on the backside of the bronchus and be aware that the pulmonary vein is right behind it.

Do not take the pulmonary vein trunk near the pericardium during lobectomy, because if the device fails, the vessel will retract into the pericardium and the child will bleed to death before you can do anything; ensure enough length away from pericardium for proximal control.