Diaphragmatic Defects in Infants: Acute Management and Repair

Vocabulary flashcards covering classifications, measurements, surgical techniques, and medical terms related to congenital diaphragmatic hernia (CDH) management.

Bochdalek Hernia

A posterolateral diaphragmatic defect present at birth that accounts for greater than $95\%$ of congenital diaphragmatic hernias.

Morgagni Hernia

An anteromedial diaphragmatic defect present at birth.

Isolated CDH

A congenital diaphragmatic hernia that is not associated with a known genetic mutation or other congenital defects, accounting for approximately $60\%$ of cases.

Non-Isolated CDH

A congenital diaphragmatic hernia associated with genetic mutations or other congenital anomalies, with concomitant congenital heart disease being the most common form occurring in $50\%$ of cases.

Observed-to-Expected Lung-to-Head Ratio (O/E LHR)

The most commonly used prenatal measurement for assessing the severity of a congenital diaphragmatic hernia.

Mild CDH

A classification of severity characterized by an O/E LHR of $36\%$ to $45\%$ with liver down, or greater than $45\%$ regardless of liver position, associated with greater than $75\%$ survival.

Moderate CDH

A classification of severity characterized by an O/E LHR of $26\%$ to $35\%$ with liver down, or $36\%$ to $45\%$ with liver up, associated with $40\%$ to $60\%$ survival.

Severe CDH

A classification of severity characterized by an O/E LHR of $15\%$ to $25\%$, associated with $20\%$ to $30\%$ survival.

Extremely Severe CDH

A classification of severity characterized by an O/E LHR of less than $15\%$, associated with less than $5\%$ survival.

Fetal Endoscopic Tracheal Occlusion (FETO)

A surgical procedure performed between $27$ and $30$ weeks gestation for severe CDH ($\text{O/E LHR} < 25\%$) involving the placement of an occlusive balloon in the fetal airway to promote hypoplastic lung growth.

Gentle Ventilation

A lung-protective ventilatory strategy in CDH management targeting peak inspiratory pressures $\le 25$ to $28\,\text{cm H}_2\text{O}$ and initial tidal volumes of approximately $4\,\text{mL/kg}$ while permitting hypercapnia.

Reversible Components of CDH-PH

Aspects of congenital diaphragmatic hernia-associated pulmonary hypertension related to hyperreactivity of the pulmonary vascular bed that serve as targets for medical intervention.

Irreversible Components of CDH-PH

Structural defects in CDH-associated pulmonary hypertension characterized by a paucity of vascular bed branching combined with hyperplasia of the muscular media layer.

Inhaled Nitric Oxide (iNO)

The most common medical agent used for treating CDH-associated pulmonary hypertension, though it may worsen heart failure in patients with impaired left ventricular function.

Bivalirudin

A direct thrombin inhibitor used for anticoagulation during ECMO in CDH patients to reduce bleeding risks compared to heparin.

Type A Defect

A small diaphragmatic hernia defect entirely surrounded by muscle, based on Congenital Diaphragmatic Hernia Study Group (CDHSG) standardized reporting.

Type B Defect

A diaphragmatic hernia defect lacking muscle on the chest wall that involves less than $50\%$ of the hemidiaphragm.

Type C Defect

A diaphragmatic hernia defect lacking muscle on the chest wall that involves greater than $50\%$ of the hemidiaphragm.

Type D Defect

A diaphragmatic hernia defect characterized by complete or near-complete agenesis of the hemidiaphragm.

Polytetrafluoroethylene (PTFE)

A synthetic polymer material commonly used for mesh-based patch closure of diaphragmatic defects.

Small Intestinal Submucosa (SIS)

The most common biologic mesh used in congenital diaphragmatic hernia repairs.

Myofascial Flap Repair

A surgical technique using a portion of the patient's own abdominal wall musculature, such as the transversalis muscle and fascia, to repair large diaphragmatic defects without synthetic mesh.