Vascular Anomalies
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Direct answer
Two families, not one: Mulliken and Glowacki's 1982 classification divides vascular anomalies by endothelial behaviour — haemangiomas, which appear in the first weeks, proliferate and then involute, versus malformations, present at birth, growing only with the child and never regressing. A proliferating haemangioma is treated today with propranolol 1–3 mg/kg/day, not steroids or laser; malformations divide by flow and channel into capillary, venous, lymphatic and arteriovenous, with slow-flow lesions managed by laser or sclerotherapy and fast-flow arteriovenous malformations by embolisation followed by excision — never by proximal ligation alone. Two separators beloved of examiners: GLUT1 stains infantile haemangioma and not malformation, and Kasabach-Merritt phenomenon belongs to kaposiform haemangioendothelioma, not to ordinary haemangioma.
What you must remember
- Infantile haemangioma timeline: precursor mark in the first weeks, proliferation through the first year, then involution — roughly 50 per cent involuted by age 5, 70 by 7, 90 by 9.
- Propranolol 1–3 mg/kg/day in divided doses is first-line, tapered after the proliferative phase; topical timolol suits thin superficial lesions; corticosteroids, vincristine and sirolimus are reserved for failures.
- Congenital haemangiomas (RICH, NICH): fully formed at birth, GLUT1 negative; the rapidly involuting type resolves by about a year.
- Danger distributions: segmental "beard-pattern" facial haemangiomas threaten the subglottic airway; large facial segmental lesions warrant a PHACES workup (posterior fossa, arterial, cardiac, eye, sternal); five or more cutaneous lesions trigger liver ultrasound, since multifocal hepatic disease causes consumptive hypothyroidism.
- Kasabach-Merritt phenomenon — profound thrombocytopenia with consumptive coagulopathy — complicates kaposiform haemangioendothelioma and tufted angioma, not infantile haemangioma.
- Capillary malformation (port-wine stain): present at birth, macular, darkens and thickens with age; pulsed-dye laser is first-line; a forehead-ophthalmic distribution raises Sturge-Weber syndrome (seizures, glaucoma).
- Venous malformation: soft, blue, compressible, empties on elevation, phleboliths on imaging; treated by image-guided sclerotherapy (ethanol, polidocanol or bleomycin, the last widely used in India) with surgery for localised residue.
- Lymphatic malformation: macrocystic and microcystic; sclerotherapy (OK-432, bleomycin, doxycycline) drains macrocysts, surgery excises residue; swelling with infection or bleed is typical.
- Arteriovenous malformation: warm, pulsatile with thrill and bruit around a nidus; Schobinger stages I to IV, stage IV adding cardiac decompensation; treatment is embolisation followed promptly by excision — proximal ligation or partial embolisation accelerates recruitment.
A fast-growing red plaque at six weeks
A six-week-old girl is referred for a "birthmark" over the upper eyelid that has doubled in size in three weeks and now blurs the lid margin. The history is the diagnosis: a lesion absent at birth, now briskly proliferating, is an infantile haemangioma; a port-wine stain was fully visible on day one and grows only with the child. Periorbital location raises the stakes — astigmatism and amblyopia — so ophthalmology is involved and posterior extension is imaged. Treatment is propranolol: baseline heart rate, blood pressure and glucose, titrating to 2–3 mg/kg/day in divided doses — most flatten visibly within weeks. If the same child had a rapidly enlarging, bruised truncal mass and a platelet count of 20,000, the diagnosis would be kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon, and the drug conversation would move to sirolimus or vincristine — the two scenarios share nothing but the word "haemangioma", which is the exam's point.
The strawberry that was lasered
The classic examination error is lasering a proliferating haemangioma: pulsed-dye laser then risks ulceration and scarring, and belongs (if at all) to residual telangiectasia after involution. The second is treating a port-wine stain with propranolol because it is "a haemangioma" — malformation endothelium does not respond; the stain's course is early pulsed-dye laser, which works better on pale infant skin. The third is the haematology trap: attributing Kasabach-Merritt to infantile haemangioma rather than kaposiform haemangioendothelioma — a distinction that changes the drug and the prognosis. And for arteriovenous malformations, the surgical sin is proximal ligation, which converts a treatable nidus into a recruiting lesion.
Frequently asked questions
How do you tell an infantile haemangioma from a port-wine stain at the bedside?
The haemangioma is absent at birth, proliferates in the first months, is raised and compressible, and involute; the malformation is fully present at birth, flat, and grows only with the child.
What monitoring does propranolol therapy for haemangioma require?
Heart rate and blood pressure at initiation, blood glucose in young infants, and photographic documentation. Airway, periorbital and perioral locations justify specialist input.
What is Kasabach-Merritt phenomenon, and which tumour causes it?
Consumptive coagulopathy — profound thrombocytopenia, low fibrinogen and raised D-dimers — arising within kaposiform haemangioendothelioma or tufted angioma, not within ordinary infantile haemangioma. Treatment is sirolimus or vincristine with correction of the coagulopathy.
Which haemangioma distributions mandate systemic workup?
Beard-pattern segmental facial lesions (airway risk), large facial segmental lesions (PHACES workup), and five or more cutaneous haemangiomas (hepatic ultrasound with thyroid function for consumptive hypothyroidism). Periorbital lesions need ophthalmological assessment.
Why is proximal ligation never adequate treatment for an AVM?
Because the nidus persists and recruits collaterals through vessels opened by the pressure drop, enlarging the lesion and complicating later embolisation. Correct management is embolisation of the nidus followed by complete excision.