AAP's First-ever Guideline for Mx of Infantile Hemangioma

AAP's First-ever Guideline for Mx of Infantile Hemangioma


The American Academy of Pediatrics (AAP) has recently released it's first clinical practice guideline regarding the management of infantile hemangiomas (IHs). The guideline emphasizes several key concepts and defines those IHs that are potentially higher risk and should prompt concern, it further underlines the importance of increased vigilance, consideration of active treatment and, when appropriate, specialty consultation. 

“IH is a disease with a window of opportunity in which to intervene and prevent poorer outcomes, and this critical time frame for optimizing outcomes can be missed if there are delays in referral or treatment“, the authors write while laying stress on the timely diagnosis and treatment of IHs. “Even for the most experienced clinicians, it can be difficult to predict the degree of IH growth until several weeks to months after the lesion is first noticed. By that time, damage to the dermis and subcutaneous tissues as well as permanent distortion of important anatomic landmarks, such as the nose or lips, may already have occurred”, the authors further added.

There are 5 major indications for consideration of early treatment or need for further evaluation of IHs

• Life-threatening complications
Airway obstruction, high-output congestive heart failure (associated with IHs on the liver), or profuse bleeding from ulceration
• Functional impairment or risk thereof
Vision disturbance resulting from the location of the lesion near the eyes, or feeding problems when they involve the lips or mouth.
• Ulceration or risk thereof
Ulceration occurs most commonly in infants younger than 4 months during the period of rapid IH proliferation. Ulceration is especially common in IHs that are of the superficial or mixed type, segmenta, and those located on the scalp, neck, perioral, perineal, perianal, or intertriginous sites.
• Evaluation to identify important associated structural anomalies
PHACE syndrome, in which large IHs of the face, head, and/or neck are associated with one or more developmental defects of the eyes, heart, major arteries, and brain.
• Risk of leaving permanent scarring or distortion of anatomic landmarks
Superficial strawberry-appearing lesions that can cause skin changes, and other lesions in visible locations on the head and neck that can distort anatomic landmarks, such as cartilage of the nose or ear or shape of the lip.

Definitions

A. Infantile hemangiomas
• Benign vascular tumors of infancy and childhood with unique clinical and histopathologic characteristics that distinguish them from other vascular tumors (eg, congenital hemangiomas) or malformations. 
• These characteristics include development during the first weeks or months of life, a typical natural history of rapid growth followed by gradual involution, and immunohistochemical staining of biopsy specimens with erythrocyte-type glucose transporter protein and other unique markers not present on other benign vascular tumors. 
• Many other entities are also called hemangiomas. Some are true vascular tumors, and others are vascular malformations. Therefore, it is important to use the adjective “infantile” when referring to true IHs.
• IHs are classified on the basis of soft-tissue depth and the pattern of anatomic involvement.

B. Soft-tissue depth
• Superficial: red with little or no evidence of a subcutaneous component (formerly called strawberry” hemangiomas)
• Deep: blue and located below the skin surface (formerly called “cavernous” hemangiomas)
• Combined (mixed): both superficial and deep components are present

C. Anatomic appearance
• Localized: well-defined focal lesions (appearing to arise from a central point)
• Segmental: IH involving an anatomic region that is often plaque-like and often measuring at >5 cm in diameter
• Indeterminate (undetermined): neither clearly localized or segmental (often called partial segmental)
• Multifocal: multiple discrete IHs at disparate sites.

The recommendations are:-

1. Risk stratification (Strong recommendation)
A. Classify an IH as high risk if there is evidence of or potential for the following: 

  • life-threatening complications
  • functional impairment or ulceration
  • structural anomalies (eg, in PHACE syndrome or LUMBAR syndrome), or
  • permanent disfigurement

B.    After identifying an IH as high risk, facilitate evaluation by a hemangioma specialist as soon possible

2. Imaging
A. Do not perform imaging unless the diagnosis of IH is uncertain, there are ≥5 cutaneous IHs, or associated anatomic abnormalities are suspected (Moderate recommendation)
B. Perform ultrasonography as the initial imaging modality when the diagnosis of IH is uncertain (Weak recommendation)
C. Perform MRI when concerned about associated structural abnormalities (eg, PHACE syndrome or LUMBAR syndrome) (Moderate recommendation)

3. Pharmacotherapy
A. 
Oral propranolol is the first-line agent for IHs requiring systemic treatment (Strong recommendation)
B. Dose of propranolol should be between 2 and 3 mg/kg per d unless there are comorbidities (eg, PHACE syndrome) or adverse effects (eg, sleep disturbance) that necessitate a lower dose (Moderate recommendation)
C. Propranolol should be administered with or after feeding and doses should be held at times of diminished oral intake or vomiting to reduce the risk of hypoglycaemia (Strong recommendation)
D. Evaluate patients for and educate caregivers about potential adverse effects of propranolol, including sleep disturbances, bronchial irritation, and clinically symptomatic bradycardia and hypotension (Strong recommendation)
E. Oral prednisolone or prednisone may be prescribed for Rx of IHs if there are contraindications or an inadequate response to oral propranolol (Moderate recommendation)
F.  Intralesional injection of triamcinolone and/or betamethasone may be recommen to treat focal, bulky IHs during proliferation or in certain critical anatomic locations (eg, the lip) (Moderate recommendation)
G. Topical timolol maleate may be used for thin and/or superficial His (Moderate recommendation)

4. Surgery and laser therapy in managing selected His (Moderate recommendation)

5. Educate caregivers of infants with an IH about the condition, including the expected natural history and its potential for causing complications or disfigurement (Strong recommendation)

High-Risk IHs


About AAP
The American Academy of Pediatrics (AAP) is an American professional association of paediatricians. It is an organization of 67,000 paediatricians committed to the optimal physical, mental, and social health and well-being for all infants, children, adolescents, and young adults.

Note: This list is a brief compilation of some of the key points included in the guideline and is not exhaustive and does not constitute medical advice. Kindly refer to the original publication here: https://pxmd.co/TXwcU


About Author
Dr. Prachi Chhimwal
Dr. Prachi Chhimwal is an Editor at PlexusMD and is a part of the Engagment Team. She curates the Technical Content posted daily on the news feed. She graduated from Army College of Dental Sciences (B.D.S) and went on to pursue her post-graduation (M.D.S) in Oral & Maxillofacial Pathology. After a decade in the field of dentistry she took a leap of faith and joined PlexusMD. A badminton enthusiast, when not working you can find her reading, Netflixing or enjoying stand-up comedy shows.
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Dr. U●●●v P●●●l
Dr. U●●●v P●●●l Paediatrics
The guideline is really helpful and exhaustive. Thanks.
Dec 29, 2018Like1