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Infantile hepatic hemangioendothelioma: the role of surgery
Insights
Infantile hepatic hemangioendothelioma (IHH) often presents in early infancy with cardiac failure and coagulopathy. Conservative management is recommended initially, reserving surgery for severe, intractable cases.
Area of Science:
- Pediatric Gastroenterology
- Neonatal Cardiology
- Vascular Anomalies
Background:
- Infantile hepatic hemangioendothelioma (IHH) is a rare benign liver tumor in infants.
- Early diagnosis and management are crucial due to potential life-threatening complications.
Observation:
- 16 infants with IHH were analyzed; 88% presented before 3 months.
- Common symptoms included high-output cardiac failure (69%), consumptive coagulopathy (75%), and anemia (75%).
- Antenatal ultrasound suspected diagnosis in 44% of cases.
Findings:
- Medical management stabilized 44% of infants with cardiac failure or coagulopathy.
- Surgical resection (partial hepatectomy) was successful in 4 patients.
- Embolization and spontaneous involution were observed in some cases.
Implications:
- Conservative treatment is advised for IHH, especially for high-output cardiac failure and consumptive coagulopathy.
- Surgery should be reserved for refractory cases.
- Understanding IHH presentation aids in timely diagnosis and intervention.
Abstract:
Of 16 infants with infantile hepatic hemangioendothelioma, 14 (88%) presented before age 3 months. For seven cases (44%), the diagnosis was suspected from antenatal ultrasonographic findings. Two (13%) presented with asymptomatic hepatomegaly. The most common presenting features were high-output cardiac failure in 11 (69%), consumptive coagulopathy in 12 (75%), and anemia in 12(75%). Sixty-three percent of the children had associated cutaneous hemangiomata, and disseminated hemangiomatosis was noted in two (13%). Medical measures were effective in stabilizing seven (44%) cases with high-output congestive cardiac failure and/or consumptive coagulopathy. Partial right hepatectomy was successful for four patients; the only death occurred in a newborn, after intraperitoneal rupture of the hepatic hemangioma. Embolization was used in two children to induce involution. Spontaneous involution occurred in two patients. Initially, hepatic hemangiomas should be treated conservatively, with surgery reserved for intractable cardiac failure and/or refractory consumptive coagulopathy.
