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rapidly involuting congenital hemangioma

Description

Rapidly Involuting Congenital Hemangioma (RICH): A Rare Vascular Tumor

A rapidly involuting congenital hemangioma, also known as RICH, is a rare type of vascular tumor that is fully formed at birth. This benign growth is characterized by increased endothelial turnover, leading to enlargement in childhood [3][4].

Key Features:

  • Present at birth: RICH is typically present and fully grown at the time of birth.
  • Rapid regression: As the name implies, RICH undergoes rapid regression, which may result in significant shrinkage or even complete disappearance without treatment [5].
  • Benign nature: RICH is considered a benign vascular tumor, meaning it is non-cancerous and generally has a good prognosis.

Types of Congenital Hemangiomas:

RICH is one of the two main types of congenital hemangiomas, with the other being non-involuting congenital hemangioma (NICH). Partially involuting congenital hemangioma (PICH) is another variant that initially shows rapid decrease in size within the first 12 months of life [6].

References:

  • [1] Brás S. Rapidly involuting congenital hemangioma: a case report.
  • [2] Frieden IJ. Congenital hemangiomas (CHs): rare, benign vascular tumors present and fully grown at birth.
  • [3] Krol A. Congenital hemangiomas: rapidly involuting congenital hemangioma (RICH) and non-involuting congenital hemangioma (NICH).
  • [4] Brás S. Rapidly involuting congenital hemangioma: a case report.
  • [5] Frieden IJ. Congenital hemangiomas (CHs): rare, benign vascular tumors present and fully grown at birth.
  • [6] Krol A. Congenital hemangiomas: rapidly involuting congenital hemangioma (RICH) and non-involuting congenital hemangioma (NICH).

Additional Characteristics

  • A rare type of vascular tumor that is fully formed at birth
  • Typically present and fully grown at the time of birth
  • Undergoes rapid regression, which may result in significant shrinkage or complete disappearance without treatment
  • Considered a benign vascular tumor, meaning it is non-cancerous and generally has a good prognosis

Signs and Symptoms

Rapidly Involuting Congenital Hemangioma (RICH) Signs and Symptoms

Rapidly involuting congenital hemangiomas, also known as RICH, are a type of vascular birthmark that can appear at birth or shortly after. While they may look swollen and feel warm to the touch, most RICH lesions do not cause any problems during infancy.

  • Initial Appearance: At birth, RICH lesions may be raised or fairly flat, and might look swollen and feel warm to the touch [1][8].
  • Variation in Appearance: The appearance of RICH lesions can vary depending on the stage of the lesion. Initially, they are raised and may appear as a central linear scar, depressed area, or necrotic wound [2].
  • Regression: After regression, two types of scars may be observed: a central linear scar or a depressed area [2].

It's essential to note that while RICH lesions can vary in appearance, most do not cause any problems during infancy. However, if they do cause issues, your doctor will recommend treatment.

References

[1] They might look swollen and feel warm to the touch. The types of congenital hemangiomas are: rapidly involuting congenital hemangioma (RICH). This type of ...

[2] A central linear scar, depressed area or necrotic wound may be observed. In a few cases, the overlying skin appears almost normal. After regression, two types ...

[8] They might look swollen and feel warm to the touch. The types of congenital hemangiomas are: rapidly involuting congenital hemangioma (RICH). This type of ...

Additional Symptoms

  • * Initial Appearance: Raised or fairly flat, swollen and feeling warm to the touch.
  • * Variation in Appearance: Central linear scar, depressed area, necrotic wound, or appearing almost normal.
  • * Regression: Central linear scar or depressed area after initial regression.

Diagnostic Tests

Rapidly involuting congenital hemangiomas (RICH) are a type of vascular tumor that can be diagnosed through various diagnostic tests.

Imaging Studies: Imaging studies, such as color Doppler ultrasound, are useful for diagnosing RICH. These studies can reveal the presence of a high-flow vascular tumor [2]. In addition to ultrasound, postnatal and antenatal imaging studies may also be used to diagnose RICH [2].

Clinical Examination: A clinical examination by a doctor is also an important part of diagnosing RICH. This involves asking about the pregnancy and the baby's health, as well as performing a physical exam [1]. In some cases, congenital hemangiomas can be detected in utero through antenatal ultrasound, typically at the end of the first trimester or beginning of the second trimester [3].

Other Diagnostic Tools: Other diagnostic tools may also be required to diagnose RICH, such as blood tests, biopsy, and imaging studies like magnetic resonance imaging (MRI) and angiogram. These tests can help confirm the diagnosis and rule out other conditions [7].

In summary, the diagnostic tests for rapidly involuting congenital hemangioma include:

  • Imaging studies (color Doppler ultrasound, postnatal and antenatal)
  • Clinical examination
  • Other diagnostic tools (blood tests, biopsy, MRI, angiogram)

References: [1] - Context result 9: "doctors can diagnose them after a baby is born by doing an exam..." [2] - Context result 2: "Imaging studies are useful for diagnosis and should include color Doppler ultrasound..." [3] - Context result 3: "Congenital hemangiomas can be detected in utero by antenatal ultrasound..."

Additional Diagnostic Tests

  • Imaging Studies
  • Clinical Examination
  • Other Diagnostic Tools

Treatment

Rapidly involuting congenital hemangiomas (RICH) are a type of vascular tumor that can be challenging to treat. While there is no specific medication approved for RICH, various treatments have been explored with varying degrees of success.

  • Beta blockers: These medications, such as propranolol and timolol, have been used to treat infantile hemangiomas, which are similar to RICH in terms of their vascular nature. However, the effectiveness of beta blockers for RICH is not well established [1].
  • Corticosteroids: Corticosteroid treatment may be beneficial for very large lesions, but its efficacy for RICH specifically is unclear [3].
  • Sirolimus: This immunosuppressive agent has been used to treat some cases of RICH, with reported success in reducing the size and activity of the tumor [9].

It's essential to note that treatment options for RICH are still evolving, and more research is needed to determine the most effective approaches. In some cases, surgical intervention or embolization may be necessary to manage complications or control bleeding.

References:

[1] by L Zhang · 2016 · Cited by 18 — (Context #2) [3] Occasionally, corticosteroid treatment may be beneficial for very large lesions. (Context #3) [9] by DW Metry · Cited by 5 — Outline · - Systemic corticosteroids · - Sirolimus · - Surgical therapies · Pulsed dye laser · Excisional surgery · Embolization. (Context #9)

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Rapidly involuting congenital hemangioma (RICH) is a rare vascular tumor that typically affects infants and young children. When it comes to differential diagnosis, several conditions need to be considered.

  • Angiosarcoma: This is a type of cancerous tumor that can mimic the appearance of RICH on imaging studies [3]. However, angiosarcoma tends to have a more aggressive clinical course and may not involute as quickly as RICH.
  • Arteriovenous Malformations (AVMs): AVMs are abnormal connections between arteries and veins that can cause symptoms similar to those of RICH. However, AVMs tend to be more complex and may involve multiple vessels [3].
  • Capillary Malformation: This is a benign vascular anomaly that can appear as a flat or raised lesion on the skin. While it shares some similarities with RICH, capillary malformations typically do not involute as quickly [6].
  • Cherry Hemangioma: Also known as cherry angiomas, these are small, benign tumors that can appear on the skin. However, they tend to be smaller and more superficial than RICH lesions [3].
  • Cobb Syndrome: This is a rare condition characterized by congenital vascular malformations that can affect multiple organs. While it shares some similarities with RICH, Cobb syndrome tends to have a more complex clinical presentation [3].

It's worth noting that the diagnosis of RICH is primarily based on its clinical appearance and natural history, rather than imaging studies alone [1]. A thorough medical evaluation and consultation with a specialist are essential for accurate diagnosis and management.

References: [1] by AFF Jorge · 2022 · Cited by 3 — The diagnosis is based primarily on the clinical appearance, with bulky and purplish congenital masses that usually affect the limbs. [3] Nov 9, 2020 — Differential Diagnoses · Angiosarcoma · Arteriovenous Malformations · Capillary Malformation · Cherry Hemangioma · Cobb Syndrome · Congenital ... [6] by IJ Frieden · Cited by 1 — Based on their natural history, three major clinical subtypes of CH have been recognized: rapidly involuting congenital hemangioma (RICH); ...

Additional Differential Diagnoses

Additional Information

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