[Skip to Content]

Quiz

Dermatology

Persistent Vascular Lesion in a Young Boy

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA Ophthalmology

Published Online: July 7, 2022

JAMA Ophthalmology Clinical Challenge

Article Information and Disclosures

Tonya Lee, BS¹;

Mansoor Movaghar, MD^1,2;

Catherine Y. Liu, MD, PhD^1,2,3

Author Affiliations

¹Viterbi Family Department of Ophthalmology, University of California, San Diego, La Jolla
²Department of Ophthalmology, Rady Children’s Hospital, San Diego, California
³Division of Oculofacial Plastic and Reconstructive Surgery, Shiley Eye Institute, University of California, San Diego, La Jolla

Read article on JAMA Network

Read Case Take Quiz Answer

A 3-year-old boy presented with a vascular lesion involving the left orbit and face. He had a pink macule on his left cheek at birth, which became raised and discolored around age 3 to 4 weeks. He was diagnosed with a capillary hemangioma, and propranolol was started at age 1 month with progression despite dosage increases. Topical timolol was tried without improvement; oral prednisolone decreased the size of the lesion, but was poorly tolerated and discontinued. An ophthalmic evaluation at an outside hospital was concerning for amblyopia because of anisohypermetropia, greater on the left, and a left esotropia and hypertropia. A magnetic resonance image revealed an orbital mass surrounding the left lateral rectus muscle with mass effect on the globe and extension into the eyelid, pterygopalatine fossa, and cheek.

Take Quiz

Please finish quiz first before checking answer.

A 3-year-old boy presented with a vascular lesion involving the left orbit and face. He had a pink macule on his left cheek at birth, which became raised and discolored around age 3 to 4 weeks. He was diagnosed with a capillary hemangioma, and propranolol was started at age 1 month with progression despite dosage increases. Topical timolol was tried without improvement; oral prednisolone decreased the size of the lesion, but was poorly tolerated and discontinued. An ophthalmic evaluation at an outside hospital was concerning for amblyopia because of anisohypermetropia, greater on the left, and a left esotropia and hypertropia. A magnetic resonance image revealed an orbital mass surrounding the left lateral rectus muscle with mass effect on the globe and extension into the eyelid, pterygopalatine fossa, and cheek.

Sign in to take quiz and track your certificates

Buy This Activity

Article Information

Corresponding Author: Catherine Y. Liu, MD, PhD, Division of Oculofacial Plastic and Reconstructive Surgery, Shiley Eye Institute, University of California, San Diego, 9415 Campus Point Dr, La Jolla, CA 92093 (yul107@health.ucsd.edu).

Published Online: July 7, 2022. doi:10.1001/jamaophthalmol.2022.1541

Conflict of Interest Disclosures: Dr Liu reported a research grant from Horizon Therapeutics and royalties from Wolters Kluwer Health outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient’s family for granting permission to publish this information.

References

Cho YJ , Kwon H , Kwon YJ , Kim SC , Kim DY , Namgoong JM . Effects of sirolimus in the treatment of unresectable infantile hemangioma and vascular malformations in children: a single-center experience. J Vasc Surg Venous Lymphat Disord. 2021;9(6):1488-1494. doi:10.1016/j.jvsv.2021.03.014 PubMed Google Scholar Crossref

Hammill AM , Wentzel M , Gupta A , et al. Sirolimus for the treatment of complicated vascular anomalies in children. Pediatr Blood Cancer. 2011;57(6):1018-1024. doi:10.1002/pbc.23124 PubMed Google Scholar Crossref

Dávila-Osorio VL , Iznardo H , Roé E , Puig L , Baselga E . Propranolol-resistant infantile hemangioma successfully treated with sirolimus. Pediatr Dermatol. 2020;37(4):684-686. doi:10.1111/pde.14163 PubMed Google Scholar Crossref

Kaylani S , Theos AJ , Pressey JG . Treatment of infantile hemangiomas with sirolimus in a patient with PHACE syndrome. Pediatr Dermatol. 2013;30(6):e194-e197. doi:10.1111/pde.12023 PubMed Google Scholar Crossref

Hutchins KK , Ross RD , Kobayashi D , Martin A , Rajpurkar M . Treatment of refractory infantile hemangiomas and pulmonary hypertension with sirolimus in a pediatric patient. J Pediatr Hematol Oncol. 2017;39(7):e391-e393. doi:10.1097/MPH.0000000000000961 PubMed Google Scholar Crossref

Shoji MK , Shishido S , Freitag SK . The use of sirolimus for treatment of orbital lymphatic malformations: a systematic review. Ophthalmic Plast Reconstr Surg. 2020;36(3):215-221. doi:10.1097/IOP.0000000000001518 PubMed Google Scholar Crossref

Krowchuk DP , Frieden IJ , Mancini AJ , et al; Subcommittee on the Management of Infantile Hemangiomas. Clinical practice guideline for the management of infantile hemangiomas. Pediatrics. 2019;143(1):e20183475. doi:10.1542/peds.2018-3475 PubMed Google Scholar Crossref

Polites SF , Watanabe M , Crafton T , et al. Surgical resection of infantile hemangiomas following medical treatment with propranolol versus corticosteroids. J Pediatr Surg. 2019;54(4):740-743. doi:10.1016/j.jpedsurg.2018.08.001 PubMed Google Scholar Crossref

Men CJ , Ediriwickrema LS , Paik JS , et al. Surgical intervention of periocular infantile hemangiomas in the era of β-blockers. Ophthalmic Plast Reconstr Surg. 2020;36(1):70-73. doi:10.1097/IOP.0000000000001466 PubMed Google Scholar Crossref

Adams DM , Trenor CC III , Hammill AM , et al. Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics. 2016;137(2):e20153257. doi:10.1542/peds.2015-3257 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.