weekly_question 29/11/2020

[Admin]

A 1-month old baby is being seen in the clinic for follow up of a liver mass. The mass was seen on ultrasound prenatally. Postnatally, the baby remained hemodynamically normal. Alpha fetoprotein was normal for age and is appropriately decreasing with time. Ultrasound at birth showed a 4cm hepatic hemangioma. The ultrasound today was interpreted as unchanged in size.

This baby would be at risk for

A Heart failure

B Thrombocytopenia

C Hypothyroidism

D Development of metachronous lesions

E Involution of the lesion

[Admin]

correct answer
E Involution of the lesion

The baby has a congenital hemangioma and the natural course of the lesion is involution postnatally.

In 2018, the American Society of Pediatric Hematology Oncology Vascular Anomalies Special Interest Group published a landmark paper on hepatic hemangiomas, identifying this clinical entity as knowledge gap among physicians. The paper recommended the use of the International Society of the Study of Vascular Anomalies classification when discussing hepatic lesions, specifying whether a hepatic hemangioma is congenital or infantile.

Congenital and infantile hemangiomas are both benign endothelial tumors. Congenital hemangiomas (CH) proliferate in utero and are fully formed at birth. There are three subtypes of evolution: the rapidly involuting congenital hemangioma (RICH), partially involuting congenital hemangioma (PICH), and non-involuting congenital hemangioma (NICH). In contradistinction, infantile hemangiomas (IH) have their proliferative phase after birth, rapidly growing until 6-12 months of age, with a gradual involution until 3-9 years of age. Glucose transporter-1 (GLUT-1) protein is an immunologic marker that has allowed pathologists to designate accurately the type of vascular tumor and predict the natural course. Congenital hemangiomas do not express GLUT-1 or podoplanin (a marker for lymphatic malformation), but infantile hemangiomas do express GLUT-1.

Most CH are unifocal. IH may be unifocal, multifocal or diffuse. Ultrasonography should be the imaging study performed first. MRI can be used if there are doubts regarding the diagnosis. Radiologic findings are beyond the scope of this discussion. However, the following characteristics should be specified in the diagnostic imaging report to ensure close longitudinal follow up: focality; number of lesions; presence of hemorrhage, calcification, and/or necrosis; assessment of hepatic vasculature; documentation of flow, enhancement, shunting, and/or tapering of aorta.

CH are high flow vascular tumors that may led to high output cardiac failure. At birth, the shift in circulation can lead to bleeding, anemia, thrombocytopenia, and low fibrinogen levels. The bleeding is typically self-limited, but may require transfusion. CBC, coagulation parameters, hepatic ultrasound, and echocardiogram should be performed.

IH are typically small at birth and grow quickly in the first 6-12 months. Cardiac failure will typically not be present at birth, but may develop during the proliferative phase. Consumptive hypothyroidism may be seen due to high levels of type 3 idothyronine deiodinase. In the diffuse form, there is higher likelihood of cardiac failure, abdominal compartment syndrome, failure to thrive. At diagnosis, CBC, hepatic ultrasound, and thyroid function tests (TSH, free T4) should be obtained. Cardiac ECHO can be considered if symptoms are present.

At diagnosis, either during birth or at any time in infancy, serum AFP should be obtained to rule out hepatoblastoma. Follow up for both CH and IH includes serial ultrasounds every two weeks, adding two weeks to the interval between imaging if there is a stable size of the lesion. For CH, US is recommended until at least one year of age, or when a stable size of lesion is reached. For IH, US should be performed until the involution is complete.

The treatment of vascular anomalies may be managed best by a multidisciplinary team that may include dermatology, surgery, interventional radiology, and pathology. The goal of treatment is to minimize morbidity and mortality, optimize functionality, and ultimately, have the best aesthetic result. Infantile hemangiomas may be managed by observation, medication, or surgical intervention, with the age of the child, symptoms, size and location of the lesion that would guide treatment. Propranolol has been used for infantile hemangioma, but is not effective in CH. Propanolol may have side effects of bradycardia, hypotension, bronchospasm and hypoglycemia. There is a 25% recurrence of IH after cessation of propranolol therapy. Steroids have been used as well.

[ashrarur]

Hemangiomas are characterized by proliferation, static phase followed by regression as a natural process. If this a case of hemangioma, then follow up by USG may be the right way to manage. However, if rapid growth is observed, beta blockers may be tried as well. Very big lesions may cause high output heart failure & thrombocytopenia. Keep us updated.

[Dr Ammar]

E

[Abusnaina mohammed]

B

[Hananmahdy]

B

[Admin]

correct answer
E Involution of the lesion

The baby has a congenital hemangioma and the natural course of the lesion is involution postnatally.

In 2018, the American Society of Pediatric Hematology Oncology Vascular Anomalies Special Interest Group published a landmark paper on hepatic hemangiomas, identifying this clinical entity as knowledge gap among physicians. The paper recommended the use of the International Society of the Study of Vascular Anomalies classification when discussing hepatic lesions, specifying whether a hepatic hemangioma is congenital or infantile.

Congenital and infantile hemangiomas are both benign endothelial tumors. Congenital hemangiomas (CH) proliferate in utero and are fully formed at birth. There are three subtypes of evolution: the rapidly involuting congenital hemangioma (RICH), partially involuting congenital hemangioma (PICH), and non-involuting congenital hemangioma (NICH). In contradistinction, infantile hemangiomas (IH) have their proliferative phase after birth, rapidly growing until 6-12 months of age, with a gradual involution until 3-9 years of age. Glucose transporter-1 (GLUT-1) protein is an immunologic marker that has allowed pathologists to designate accurately the type of vascular tumor and predict the natural course. Congenital hemangiomas do not express GLUT-1 or podoplanin (a marker for lymphatic malformation), but infantile hemangiomas do express GLUT-1.

Most CH are unifocal. IH may be unifocal, multifocal or diffuse. Ultrasonography should be the imaging study performed first. MRI can be used if there are doubts regarding the diagnosis. Radiologic findings are beyond the scope of this discussion. However, the following characteristics should be specified in the diagnostic imaging report to ensure close longitudinal follow up: focality; number of lesions; presence of hemorrhage, calcification, and/or necrosis; assessment of hepatic vasculature; documentation of flow, enhancement, shunting, and/or tapering of aorta.

CH are high flow vascular tumors that may led to high output cardiac failure. At birth, the shift in circulation can lead to bleeding, anemia, thrombocytopenia, and low fibrinogen levels. The bleeding is typically self-limited, but may require transfusion. CBC, coagulation parameters, hepatic ultrasound, and echocardiogram should be performed.

IH are typically small at birth and grow quickly in the first 6-12 months. Cardiac failure will typically not be present at birth, but may develop during the proliferative phase. Consumptive hypothyroidism may be seen due to high levels of type 3 idothyronine deiodinase. In the diffuse form, there is higher likelihood of cardiac failure, abdominal compartment syndrome, failure to thrive. At diagnosis, CBC, hepatic ultrasound, and thyroid function tests (TSH, free T4) should be obtained. Cardiac ECHO can be considered if symptoms are present.

At diagnosis, either during birth or at any time in infancy, serum AFP should be obtained to rule out hepatoblastoma. Follow up for both CH and IH includes serial ultrasounds every two weeks, adding two weeks to the interval between imaging if there is a stable size of the lesion. For CH, US is recommended until at least one year of age, or when a stable size of lesion is reached. For IH, US should be performed until the involution is complete.

The treatment of vascular anomalies may be managed best by a multidisciplinary team that may include dermatology, surgery, interventional radiology, and pathology. The goal of treatment is to minimize morbidity and mortality, optimize functionality, and ultimately, have the best aesthetic result. Infantile hemangiomas may be managed by observation, medication, or surgical intervention, with the age of the child, symptoms, size and location of the lesion that would guide treatment. Propranolol has been used for infantile hemangioma, but is not effective in CH. Propanolol may have side effects of bradycardia, hypotension, bronchospasm and hypoglycemia. There is a 25% recurrence of IH after cessation of propranolol therapy. Steroids have been used as well.

[Khizer Mansoor]

B

[Khizer Mansoor]

The answer you have selected is wrong as per your question. You asked for the risk, not the fate of the lesion. Also it depends whether it's a RICH or NICH.

[Admin]

This is the question and answer as it is from the question bank of APSA, thanks for your contribution.

[paupaubb2005]

E