Hemangioma
Definition :Is a congenital benign proliferative tumor of endothelial cell; it is the most common tumor of infancy that exhibits rapid postnatal growth and slow regression during childhood. ,
It’s minimally visible at birth, and then undergo a period of rapid postnatal growth around 6 weeks of age and continuing until 6 to 20 months of age then undergo involution which may take 5 years until completion and there will be a certain amount of redundant, greyish skin which persists. Radiologically, there is well-circumscribed, abundant lobular parenchymal tissue.Histologically, there is rapid turnover of endothelial ,increased mast cells, and a multilaminated basement membrane
Types:
A- -cutaneous (superfecial & deep) ,(congintal & infantile )
B--Extra cutaneous
Extra cutaneous sites, including the liver, gastrointestinal tract, larynx, central nervous system, pancreas, gall bladder, thymus, spleen, lymph nodes, lung, urinary bladder, and adrenal glands.
A formal classification was officially adopted by the International Society for the Study of Vascular Anomalies (ISSVA )in 1996:
VASCULAR Tumors
Hemangioma --Hemangioendotheliomas
-Angiosarcoma
-Miscellaneous
VASCULAR Malformations
Slow-flow :
-Capillary
-Lymphatic
-Venous
Fast-flow
-Arterial
-Combined
Etiology:
The presence of Erythtocyte-type glucose transporter protein-1 GLUT1 (and other placenta-associated antigens) suggested that hemangioma might originate by embolized placental cells or involve an immunophenotypic alteration in primitive cells forming the tumor.
There is evidence that hemangioma-genesis begins as a somatic mutation in a single endothelial cell, leading to clonalexpansion.
Dose hemangioma resolve spontaneously?????
Incidence:
-Approximately 80% are solitary; 20% are multifocal. -females > males (3-5:1) The incidence is 10% to 12% in white infants and 23% in preterm infants who weigh < 1.000 g. -the frequency is lower in dark-skinned infants.
clinical feature:
Hemangioma appears in neonatalhood, usually within the first 2 weeks.
Deep subcutaneous tumors or visceral hemangioma may not manifest until 2 to 3 months of life.
superficial type barely visible pale area, telangiectatic, or macular red stain, or an ecchymotic spot.
Congenital hemangioma is a rare variant that grows in utero and presents completely formed at birth.
Phases of hemangioma -Proliferative Phase. -Involuting Phase. -Involuted Phase. dose every red lesion considered as hemangioma ??????
:Proliferative Phase
It grow rapidly during the first 6 to 8 months of infancy. If the tumor proliferates in superficial dermis, the skin becomes raised, bosselated, and a vivid crimson color. If the tumor proliferates in the lower dermis and subcutis, the overlying skin may be only slightly raised and of bluish color. Telangiectases and large superficial veins radiating from the hemang not Tender.
:
Involuting Phase
Hemangioma reaches its peak before the first year as the crimson color fades to a dull purple , the skin gradually pales, patchy grey until the child is 5 to 10 years and the tumor feels less tense. Usually the last traces of color disappear by 5 to 7 year of life.
Involuted Phase
Regression is complete & improvement until age 10 to 12 years. Nearly normal skin is restored in approximate 50% of children; otherwise there is a cutaneous telangiectasias, laxity, yellowish hypoelastic patche
Dose all hemangioma resolve completily ?????
Complications:
Ulceration. -
-Infection, Pain, Scarring
-Hemorrhage, external or internal bleeding
-High output cardiac (heart) failure resulting from a hemangioma in an internal organ.
-obstruction; obstruction of vital functions such as vision, hearing, or breathing; distortion of facial features life-threatening complicati lesions in “beard”distribution along the jaw are at increased risk of airway involvement & need to be monitored frequently for signs of respiratory distress.
-Visual complication (Amblyopia,Strabismus Astigmatisms) affects43% to 60% of patients with IH in this location.
Psychological -
diagnosis :
-History and physical examination.
-(Angiography; Ultrasonography ,CT scan,MRI).
-Biopsy is necessary when ever there is any suspicion of malignancy.
Out line of Management:
1-Observation
2-Local Treatment for Ulceration and Bleeding
3-Pharmacologic Therapy
4-Embolization
4-Laser Therapy
5-Surgical Management
Observation:
-Explanation-measurements and photographs
-follow-up
-Assurance
-More frequent visits are necessary if it large, ulcerated, multiple, or located in an anatomically critical area.
Management 0f breakdown and ulceration :
-occurs in 5%, commonly in lip or anogenital area.
-Daily use topical antibiotic or hydrocolloid dressing; lidocaine ,eschar, debridement or dressings are needed.
-pulsed dye laser treatment relieves pain & accelerates healing;
-Bleeding is a rare & it maneged by compressing the area with a clean pad for 10 minutes;Rarely is it require " to place a mattress suture to control a local bleeding site
Pharmacologic Therapy:
1-Corticosteroid.
2-Interferon.
Corticosteroid :
local :
-Intralesion injection (Triamcinolone 25mg/kg).
-For well localized (<2.5cm), injected slowly at low pressure 3 to5cc ; 3 to 5 Injection at 6 to 8 week interval.
-skin atrophy , periorbital area ptosis,3rd nerve palsy, necrosis and even blindness(due to Central retinal artery embolism).
Systemic Corticosteroid:
-is the first line of treatment of large endangering hemangioma
-Oral prednisolone 2 to 3mg/kg/day single morning dose for 4 to 6 week then tapered slowly over several months and stopped at 10 to 11 moth.
-Response rate is approximate y 85%, either accelerated regression or stabilization of growth.
-Discontinued if there is no effect such as lightening of color, softening, or diminished growth.
Rebound growth can occur if the drug level is lowered too rapidly.-
Response to Corticosteroid within 2-3 weeks as it become pale shrink
Interferon- Alfa:
second-line agent for endangering and life-threatening hemangiomas.
Indications:
1-failure to respond to corticosteroid;
2-contraindications to prolonged parenteral corticosteroid;
3-complications of corticosteroid;
4-parental refusal of corticosteroid
Corticosteroids and IFN should not be co-administered in therapeutic dosage.
The dose is 2 to 3 mU/m2, injected subcutaneously daily. The rate of response is >80% usually 6 to 10 months of sustained therapy is required.
Side effect of INF:
-fivefold induction in liver transamnase, transient neutropenia, and anemia
-fever for the first 1 to 2 weeks
-spastic diplegia
Side effect of Corticosteroid:
-Gastric irritation may developed so H2 receptor inhibitor is also given.
-Cushingoid faces occurs in virtually all treated infants;
-one- third exhabat diminished rate of gain of length and weight that returns to normal after discontinuing the drug.
-Rare complications include myopathy, cardiomyopathy, premature thelarche, and hirsutism
Embolization:
-Is the interventional radiological procedure in which abnormal vessels are closed off with various substances.
-with superselective arterial catheterization is considered if there is failure or slow Response to Pharmacologic Therapy
Chemotherapy:
-Vinicrestin,cyclophosphamid
-Vinicrestin is another second line treatment for endangering hemangioma in infant who fail to respond corticosteroid or develop serious corticosteroid complication.
-Administrated through central venous line; the response rate >80% .
-Side effect include peripheral neuropathy ,constipation ,minor hair loss , sepsis.
laser surgery:
The flash lamp pulsed-dye laser & pulsed Nd:YAG, frequency-doubled Nd:YAG, and KTP lasers, CO2 laser is useful for removing hemangioma inside the child's airway
Laser photocoagulation can lighten the involved skin; used in these situations:
1-Superficial facial hemangiomas
2-Refractory ulceration
3-Significant residual telangiectasia.
4-For cosmetic improvement.
6-Before the surgical resection.
Surgical Management:
Circular excision and purse-string closure & Elleptical excision,surgical excision & cover with local flap or use tissue expander.
Infancy (Proliferating Phase) Indications for resection of a well-localized tumor in the first year are :
1-obstruction
2-deformation
3-bleeding;
4-ulceration unresponsive to topical, intralesional, or systemic therapy;
5-predictable scar or hair loss.
Summery : -Hemangioma is a benign tumor which is a common tumor of infancy that exhibits rapid postnatal growth and slow regression during childhood. -It’s mainly diagnosed by History and physical examination.
-Management includes Local treatment ,medical therapy, embolization, Laser Therapy & Surgical interference .
Home work:-What are the types of hemangioma?-What are the out line management of hemangioma?