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Acta Radiológica Portuguesa

Print version ISSN 2183-1351

Acta Radiol Port vol.32 no.1 Lisboa Apr. 2020  Epub June 08, 2021

https://doi.org/10.25748/arp.18486 

Imagens de Interesse

Bone Scintigraphy in McCune-Albright Syndrome

Cintigrafia Óssea no Síndrome de McCune-Albright

Patrícia Gouveia1 

Ricardo Teixeira1 

Adriana Sá Pinto1 

Inês Amorim1 

1Department of Nuclear Medicine, Centro Hospitalar e Universitário do Porto, Porto, Portugal


Abstract

McCune-Albright Syndrome is a rare disease characterized by bone fibrous dysplasia, endocrine disorders, and café-au-lait skin pigmentation. We present a bone scan image of a 34-year-old man with McCune-Albright syndrome.

Keywords: Fibrous dysplasia; Polyostotic; McCune- Albright syndrome; Bone scintigraphy; Pirate sign.

Resumo

O Síndrome de McCune-Albright é uma doença rara caracterizada por fibrodisplasia óssea, alterações endócrinas e manchas de “café com leite” na pele. Nós apresentamos uma imagem de uma cintigrafia óssea de um homem de 34 anos com síndrome de McCune-Albright.

Palavras-chave: Displasia fibrosa; Poliostótica; Síndrome de McCune-Albright; Cintigrafia óssea; Sinal do pirata.

A 37-year-old man with McCune-Albright Syndrome and a past history of partial rib resection (9th and 11th left ribs) was admitted to our institution with pain in the left femur and in multiple ribs. He had no history of trauma. The patient underwent a technetium-99m-methylene diphosphonate (99mTcTc-MDP) bone scan. Planar whole-body images acquired 3 hours after injection in anterior and posterior views revealed intense tracer uptake in the pars orbitalis of the frontal bone, left sphenoid wing, nasal bones, zygomatics, maxillae, left temporal bone, and occipital bone. Increased uptake was also noted in several ribs bilaterally (1st, 3rd, 8th, 9th, 11th left ribs; 8th, 12th right rib) and in the left proximal femoral shaft. These findings were compatible with polyostotic fibrous dysplasia. Fibrous dysplasia involving the sphenoid wing has been described as the pirate sign as it resembles a pirate wearing an eye patch.

McCune-Albright syndrome is a rare congenital sporadic disorder characterized by bone fibrous dysplasia, endocrine disorders, and café-au-lait skin pigmentation.1 Fibrous dysplasia can affect one bone (monostotic form), or multiple bones (polyostotic form).2 This case illustrates the usefulness of 99mTcTc-MDP bone scan, a sensitive and relative low-cost examination of the whole body,3 in defining the extent of bone involvement by fibrous dysplasia, particularly in asymptomatic regions(Fig. 1).

References

1. Dumitrescu CE, Collins MT. McCune-Albright syndrome. Orphanet J Rare Dis. 2008;19:12. [ Links ]

2. Robinson C, Collins MT, Boyce AM. Fibrous dysplasia/McCune- Albright syndrome: clinical and translational perspectives. Curr Osteoporos Rep. 2016;14:178-86. [ Links ]

3. T. Van den Wyngaert, K. Strobel, W. U. Kampen, T. Kuwert, W. van der Bruggen, H. K. Mohan, et al. The EANM practice guidelines for bone scintigraphy. Eur J Nucl Med Mol Imaging. 2016;43:1723-38. [ Links ]

Ethical disclosures

Financing Support: This work has not received any contribution, grant or scholarship.

Received: August 23, 2019; Accepted: November 20, 2019

Address Patrícia Gouveia Serviço de Medicina Nuclear, Centro Hospitalar e Universitário do Porto Largo Prof. Abel Salazar, 4099-001 Porto, Portugal email: patirigouveia@gmail.com

Conflicts of interest: The authors have no conflicts of interest to declare.

Confidentiality of data: The authors declare that they have followed the protocols of their work center on the publication of data from patients.

Protection of human and animal subjects: The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki).

Creative Commons License This is an open-access article distributed under the terms of the Creative Commons Attribution License