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Paeds Vivaspaediatric-dermatology

Paeds Vivas · paediatric-dermatology

Vascular birthmarks and infantile haemangioma — branching viva

Branching structured-oral viva on vascular birthmarks and infantile haemangioma: the Mulliken and ISSVA tumour-versus-malformation fork, the GLUT1-positive endothelial-proliferation biology and proliferate-then-involutive natural history, the GNAQ somatic-mosaic biology of port-wine stain and Sturge-Weber syndrome, propranolol 2 to 3 mg per kg per day as first-line therapy with topical timolol for small superficial lesions, the PHACE and LUMBAR syndrome screens, and the Kasabach-Merritt distinction from kaposiform haemangioendothelioma.

branching clinical structured oral5 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
You are the general paediatric registrar. A 2-month-old infant is referred with a large, bright-red segmental haemangioma covering the right forehead and cheek that has grown rapidly since appearing at three weeks of age. The examiner asks you to take the candidate through the classification, the syndrome screening, the propranolol pathway and the complications of vascular birthmarks.

Opening question

Examiner: Take me through this infant. What is the lesion, and how do you frame it? [2]

Candidate: This is an infantile haemangioma — a proliferating vascular tumour that was absent at birth, appeared in the first weeks of life, and is now in its rapid proliferative phase. My frame is the two-family fork: vascular tumours proliferate through endothelial growth and then involute, while vascular malformations are structural errors present at birth that grow only with the child. Because this lesion is large and segmental on the face, it carries the highest risk of a syndrome, so my priorities are to classify it, to screen it, and to treat it in the proliferative phase while the window for propranolol is open. [2] [11]

Examiner: Walk me through the classification. [6]

Candidate: The Mulliken and International Society for the Study of Vascular Anomalies framework divides vascular anomalies into two families. Vascular tumours include infantile haemangioma, the congenital haemangiomas (rapidly involuting, non-involuting and partially involuting), kaposiform haemangioendothelioma and tufted angioma, and pyogenic granuloma. Vascular malformations include the capillary port-wine stain, venous, lymphatic and arteriovenous malformations, and the combined syndromes such as Klippel-Trenaunay and Sturge-Weber. The decisive distinction is cellular behaviour: tumours proliferate, malformations do not. [6] [11]

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References7ShowHide
  1. [1]Léauté-Labrèze C; Dumas de la Roque E; Hubiche T; Boralevi F; et al Propranolol for severe hemangiomas of infancy. N Engl J Med, 2008.PMID 18550886
  2. [2]Krowchuk DP; Frieden IJ; Mancini AJ; Darrow DH; et al Clinical Practice Guideline for the Management of Infantile Hemangiomas. Pediatrics, 2019.PMID 30584062
  3. [4]Shirley MD; Tang H; Gallione CJ; Baugher JD; et al Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med, 2013.PMID 23656586
  4. [6]Wassef M; Blei F; Adams D; Alomari A; et al Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies. Pediatrics, 2015.PMID 26055853
  5. [7]Drolet BA; Frommelt PC; Chamlin SL; Haggstrom A; et al Initiation and use of propranolol for infantile hemangioma: report of a consensus conference. Pediatrics, 2013.PMID 23266923
  6. [8]Garzon MC; Epstein LG; Heyer GL; Frommelt PC; et al PHACE Syndrome: Consensus-Derived Diagnosis and Care Recommendations. J Pediatr, 2016.PMID 27659028
  7. [11]Sebaratnam DF; Rodríguez Bandera AL; Wong LF; Wargon O Infantile hemangioma. Part 2: Management. J Am Acad Dermatol, 2021.PMID 34419523
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