weekly_question 29/8/2021

[Admin]

A ten week old former term infant presents with increasing abdominal girth and increased work of breathing. She has multiple cutaneous hemangiomas on her scalp and trunk. An echocardiogram is normal. On abdominal ultrasound she is found to have numerous lesions in the liver consistent with diffuse hemangioma. What is the best next course of action for this baby?

A Biopsy hepatic lesions

B Start propranolol

C Start interferon

D Observation

E Biopsy cutaneous lesions

[Admin]

Answer b

correct

This baby is presenting with infantile hemangioma of the liver, the most common benign liver tumor of childhood. Classically, infantile hemangiomas appear after birth when they begin their proliferative phase and then involute until 3-9 years of age. The clinical manifestations are variable. Infantile hepatic hemangioma may be asymptomatic or may present as hepatomegaly, abdominal distention, or a palpable abdominal mass. The presence of an arteriovenous shunt within the lesion may lead to congestive heart failure. Cases of hypothyroidism have also been reported due to increased activity of type 3 iodothyronine deiodinase that deactivates thyroid hormone.

These lesions are classified into three categories: focal, multifocal, and diffuse. Focal lesions involute rapidly by the age of 12–14 months and are usually asymptomatic. Multifocal lesions evolve after birth and present a proliferating phase of approximately 9–12-month duration, followed by a slow involution phase. In some cases, they are symptomatic. Diffuse lesions develop rapidly, may massively replace the hepatic parenchyma, and lead to hepatomegaly, respiratory distress, abdominal compartment syndrome, and severe hypothyroidism.

The management of infantile hepatic hemangioma is based on clinical presentation and includes observational, medical, surgical, and radiological intervention. Observation is recommended for asymptomatic infantile hepatic hemangiomas with serial US to document regression. Patients with severe symptoms may require medical management, primarily consisting of oral propranolol, a non-selective β adrenergic antagonist. Patients with multiple hepatic hemangioma should be screened for hypothyroidism and it should be treated when diagnosed. If life-threatening symptoms progress despite medical management, surgical or radiological intervention may be indicated. There is no role for routine biopsy in asymptomatic patients. These tumors stain positive for Glut-1 on immunohistochemistry, in contrast to congenital hepatic hemangiomas that do not. Iacobas et al. provided an excellent guidance document for evaluation and monitoring of pediatric hepatic hemangioma.

Interferon is no longer a primary therapy due to neurologic sequela such as spastic diplegia.

[Medhat Tello]

Answer b

[Admin]

Answer b

correct

This baby is presenting with infantile hemangioma of the liver, the most common benign liver tumor of childhood. Classically, infantile hemangiomas appear after birth when they begin their proliferative phase and then involute until 3-9 years of age. The clinical manifestations are variable. Infantile hepatic hemangioma may be asymptomatic or may present as hepatomegaly, abdominal distention, or a palpable abdominal mass. The presence of an arteriovenous shunt within the lesion may lead to congestive heart failure. Cases of hypothyroidism have also been reported due to increased activity of type 3 iodothyronine deiodinase that deactivates thyroid hormone.

These lesions are classified into three categories: focal, multifocal, and diffuse. Focal lesions involute rapidly by the age of 12–14 months and are usually asymptomatic. Multifocal lesions evolve after birth and present a proliferating phase of approximately 9–12-month duration, followed by a slow involution phase. In some cases, they are symptomatic. Diffuse lesions develop rapidly, may massively replace the hepatic parenchyma, and lead to hepatomegaly, respiratory distress, abdominal compartment syndrome, and severe hypothyroidism.

The management of infantile hepatic hemangioma is based on clinical presentation and includes observational, medical, surgical, and radiological intervention. Observation is recommended for asymptomatic infantile hepatic hemangiomas with serial US to document regression. Patients with severe symptoms may require medical management, primarily consisting of oral propranolol, a non-selective β adrenergic antagonist. Patients with multiple hepatic hemangioma should be screened for hypothyroidism and it should be treated when diagnosed. If life-threatening symptoms progress despite medical management, surgical or radiological intervention may be indicated. There is no role for routine biopsy in asymptomatic patients. These tumors stain positive for Glut-1 on immunohistochemistry, in contrast to congenital hepatic hemangiomas that do not. Iacobas et al. provided an excellent guidance document for evaluation and monitoring of pediatric hepatic hemangioma.

Interferon is no longer a primary therapy due to neurologic sequela such as spastic diplegia.