Management of an Infant with a Hernia and Palpable Thrill
Infants with a hernia and palpable thrill should undergo prompt surgical intervention with appropriate preoperative assessment, as this presentation likely indicates congenital diaphragmatic hernia (CDH) with pulmonary hypertension requiring specialized management. 1
Diagnostic Approach
- CT scan of the chest and abdomen is the gold standard for diagnosing diaphragmatic hernia in infants with a sensitivity and specificity of 14-82% and 87%, respectively 1
- Initial chest X-ray should be performed but may be normal in up to 62% of cases, particularly with right-sided hernias 1
- Echocardiogram is recommended to assess for pulmonary hypertension, which commonly accompanies congenital diaphragmatic hernia 1
- The palpable thrill suggests vascular involvement or pulmonary hypertension, requiring careful evaluation before surgical intervention 1
Preoperative Management
- Minimize peak inspiratory pressure and avoid large tidal volumes to reduce ventilator-associated acute lung injury in infants with CDH 1
- High-frequency oscillatory ventilation is a reasonable alternative when poor lung compliance, low volumes, and poor gas exchange complicate the clinical course 1
- For infants with severe pulmonary hypertension:
- Inhaled nitric oxide (iNO) therapy can improve oxygenation but should be used cautiously in subjects with suspected left ventricular dysfunction 1
- Sildenafil is reasonable as adjunctive therapy for infants refractory to iNO, especially with an oxygenation index exceeding 25 1
- Inhaled prostacyclin analogs may be considered as adjunctive therapy for infants refractory to iNO 1
Surgical Management
- Extracorporeal membrane oxygenation (ECMO) is recommended for patients with CDH with severe pulmonary hypertension who do not respond to medical therapy 1
- Laparoscopic repair is preferred in hemodynamically stable infants without significant comorbidities 1
- Open surgical approach should be reserved for:
- Hemodynamically unstable patients
- When laparoscopic skills or equipment are unavailable
- When exploratory laparotomy is needed for other reasons 1
- Damage Control Surgery is an option for critical and unstable patients 1
Special Considerations
- Prostaglandin E1 may be considered to maintain patency of the ductus arteriosus and improve cardiac output in infants with CDH and suprasystemic levels of pulmonary hypertension or right ventricular failure 1
- Supportive care with digitalis and diuretic therapy is reasonable with signs of right heart failure but should be initiated cautiously 1
- Evaluation for long-term pulmonary arterial hypertension-specific therapy should follow recommendations for all children with pulmonary hypertension 1
Post-operative Management
- Serial echocardiograms are recommended to monitor the response to pulmonary hypertension therapy 1
- Supplemental oxygen therapy should be maintained with the goal of keeping O2 saturations between 92% and 95% 1
- Close monitoring for recurrence of the hernia and complications related to pulmonary hypertension is essential 1