Summary
Moderator Dr. Taizo Nakano introduced the session and reviewed a classification system for vascular anomalies, focusing on benign vascular tumors such as hepatic hemangiomas. They also discussed the challenges in diagnosing and treating congenital and infantile hemangiomas, particularly in the liver, and the potential use of pharmacotherapy, laser therapy, and ligating the hepatic artery for treatment.
Moderator Dr. Kulunkowski introduced the panelists, including Christy Rylan, Leah Warley, Elizabeth Tang, and Zavaletta Vaz Zavaletta, all of whom have expertise in vascular anomalies. The discussion highlighted the similarities between skin and liver hemangiomas, and the two forms of liver hemangiomas: multifocal and diffuse. Elizabeth Tang was invited to discuss the imaging characteristics of congenital and infantile hemangiomas.
Distinguishing Hemangiomas in Pediatric Patients
The challenges in distinguishing between congenital and infantile hemangiomas in pediatric patients were discussed, particularly in terms of ultrasound ecogenicity. While infantile hemangiomas are typically hypoechoic, congenital hemangiomas can be heterogeneous. The importance of considering the enhancement pattern on MRI or contrast liver ultrasound, as well as diffusion restriction, is necessary to differentiate between hemangiomas and other potential diagnoses such as hepatoblastoma or neuroblastoma. The panelists advised using extracellular gadolinium agents for liver MRI and cautioned against using hepatobiliary contrast agents because of their less distinct enhancement patterns. They noted that hypervascularity on Doppler ultrasound can be indicative of infantile or cutaneous hemangiomas, but not all have this feature.
Infantile Hemangiomas Classification and Clinical Utility
The classification and clinical utility of infantile hemangiomas were discussed. The term "hemangiomatosis" is no longer clinically useful, so the focus should be on the number and confluence of tumors. The genetic classification of vascular anomalies was discussed, with particular focus on the role of type 3 iodothyronine deiodinase in infantile hemangiomas. A panelist highlighted the importance of screening for hepatic hemangiomas in patients with multiple cutaneous lesions, as it can lead to earlier identification and less complications. The team also reviewed ultrasound images of congenital and infantile hemangiomas, noting the hypervascularity and expected evolution of these lesions.
Infantile Hemangiomas and Associated Risks
Dr. Kulunkowski discussed a patient's chest X-ray and the subsequent diagnosis of infantile hemangiomas of the liver. A panelist shared her findings from the registry, highlighting the risk factors for mortality, including diffuse lesions and congestive heart failure. She also noted that many patients with abdominal compartment syndrome had a high mortality rate. Dr. Leah Wehrli then presented a case of a premature baby born with a large hepatic mass, which was later diagnosed as a hepatic hemangioma. The baby's clinical course was monitored, and her liver enzymes, bilirubin, and alpha-fetoprotein (AFP) levels were closely observed. Dr. Wehrli shared the clinical course of a premature baby, who was treated with prednisone and propanolol due to concerns of cardiac failure. The team plans to get a biopsy to confirm the diagnosis.
Interpreting AFP Levels and Vascular Anomalies
The importance of interpreting AFP levels in the context of a premature baby's health was discussed. A single AFP measurement can be difficult to interpret, but subsequent measurements can provide more clarity. The team also discussed the potential for intralesional bleeding in vascular anomalies, distinguishing it from the Kasabach-Merritt phenomenon, which is more severe and occurs in Kaposiform Hemangioendothelioma (KHE), highlighting the importance of distinguishing between AFP levels in hepatic hemangiomas and hepatoblastoma. It was suggested that a biopsy should be considered if the imaging and lab results do not follow the standard pattern.
Congenital Hemangiomas Diagnosis and Treatment Challenges
The team discussed the complexities of diagnosing and treating congenital and infantile hemangiomas, particularly in the liver. They expressed hesitance towards performing a biopsy due to the risks of bleeding and the potential for coagulopathy. However, they agreed that a percutaneous needle biopsy could be safer than an open biopsy. The team also discussed the possibility of embolization techniques for these lesions, but noted that bland embolization has not been particularly successful in shrinking them down. They concluded that further investigation and tissue confirmation are necessary for accurate diagnosis and treatment planning.
Liver Tumor Treatment Challenges and Options
The challenges of treating liver tumors were discussed, particularly in older patients, and the potential impact on the remaining liver. A healthy liver can regenerate even after significant removal, and it was suggested that the use of pharmacotherapy, such as Serolimus, as an option for congenital hemangiomas, should be considered. The team also discussed the potential use of laser therapy and ligating the hepatic artery for treating infantile hemangiomas. The consensus was that these treatments should be approached with caution and considered only in critical situations. The team agreed on the need for a standardized approach and prospective studies to better understand the effectiveness of these treatments.