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MRI Findings Common to Infantile Hemangiomas

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MRI Findings Common to Infantile Hemangiomas MRI Findings Common to Infantile Hemangiomas Item Type text; Electronic Thesis Authors Patel, Nirav Publisher The University of Arizona. Rights Copyright © is held by the author. Digital access to this material is made possible by the College of Medicine - Phoenix, University of Arizona. Further transmission, reproduction or presentation (such as public display or performance) of protected items is prohibited except with permission of the author. Download date 23/09/2021 18:48:55 Link to Item http://hdl.handle.net/10150/221633 MRI Findings Common to Infantile Hemangiomas Thesis submitted to the University of Arizona College of Medicine - Phoenix in partial fulfillment of the requirements for the degree of Doctor of Medicine Nirav Patel Class of 2012 Mentors: Judith O’Haver, PhD, RN, CPNP; Harper Price, MD; Richard Towbin, MD Dedication To my wonderful mentors Judith O’Haver, Richard Towbin, Ronald Hansen, and Harper Price. 2 Acknowledgements I would like to thank the Dermatology and Radiology departments at Phoenix Children’s Hospital for all their contributions. 3 Abstract Background: Infantile hemangiomas (IH) are the most common vascular tumors of infancy. Children may have Magnetic Resonance Imaging (MRI) to establish or confirm the diagnosis or to further evaluate lesions that do not improve with treatment. Objective: Describe specific MRI findings common to infantile hemangiomas. Compare the imaging diagnosis with the clinical diagnosis of IH to determine diagnostic accuracy. Methods: A descriptive retrospective chart review on a convenience sample. Twenty-six patients had a total of 31 MR studies in the group. From these 31 studies, 16 also had magnetic resonance angiography (MRA). Results: Clinical diagnosis matched imaging diagnosis 96.8% of the time. Findings from imaging of the infantile hemangiomas included increased signal intensity on T2-weighted sequences (96.8%), isointense or decreased signal with T1-weighted sequences (83.9%) and 4 moderate to marked contrast enhancement (78.5%). Lesions appeared to be high flow (64.5%), demonstrated lobulation (58.1%), and displayed central, low signal intensity dots on T2-weighted sequences (54.8%). In contrast, cystic spaces, intralesional DIC, phleboliths, focal intralesional inhomogenities, septation, edema, fat stranding, aneurysms, venous ectasia, and shunts were not features regularly seen in imaging of IHs in this study. Limitations: Small sample size on a convenience sample based at one institution. Conclusion: There are specific features to infantile hemangiomas on MR imaging that can be used for aid in diagnosis. 5 Table of Contents Introduction………………..8 Materials and Methods…..14 Results………………………19 Discussion……..……………24 Conclusion...........................28 Future Directions…………..29 References……………………30 6 List of Figures and Tables Table 1- Features on MRI……..15 Table 2- Features on MRA…….16 Appendix 1…………………….... 32 7 Introduction Infantile hemangiomas (IHs) are the most common tumor of infancy. These benign vascular tumors affect 4-10% of infants (1-2). They are more common in females, are usually found on the head and neck, and have an increased prevalence in children born prematurely (3-5). The differential diagnosis for vascular lesions in infants can include both vascular tumors and malformations and the continued use of outdated nomenclature can be confusing. In 1982, Mulliken and Glowacki attempted to clarify this by separating hemangiomas of infancy (classified as vascular tumors) and vascular malformations into two broad categories using histology, electron microscopy, and clinical findings. This classification is used by clinicians and has also been adopted by interventional radiologists (6). Vascular malformations are congenital anomalies of vessels that may be differentiated by vessel type into capillary, lymphatic, venous, arterial, or combined lesions, such as aterio-venous malformation or veno-lymphatic malformation. Vascular malformations are differentiated from hemangiomas by their equal predominance in 8 males and females, normal endothelial cycle (no proliferative or involution phase), and proportionate growth with the child (6). Hemangiomas of infancy are generally referred to as either infantile (present days to weeks after birth) or congenital and are classified according to the clinical depth of the lesion (superficial, deep or combined) and distribution (either segmental or focal). Generally, infantile lesions present with a precursor mark that may be seen at birth or the first few days to weeks of life followed by a “rapid growth phase” which may continue through 6-12 months of life. By one year of age most IH have reached maximum growth potential. Then lesions begin to involute over time entering the so called “involutional phase”, and the majority slowly resolves over the next 5-10 years (6-8). Most infantile hemangiomas involute with little remaining skin change left to be seen, e.g., telangiectasias. However, in some cases the residual skin overlying the lesion maybe altered due to stretching from rapid growth or scar may have resulted from ulceration. The residual fibrofatty infiltrate that results during the involution phase may also not regress adequately with time. Thus the composition of the underlying skin may also be altered and replaced with loose, fibro- fatty stroma. The impact of the physical alteration of the skin after 9 involution is dependent on the original size of the IH, the area of the body where the IH was located, and whether ulceration occurred. (8). Many IHs require no treatment, as they will naturally involute with an acceptable appearance. However, treatment is indicated when ulceration is present, functional impairment occurs (e.g., obstruction of airway), pain develops, and there is risk for permanent disfigurement (e.g. nose, ears). This represents an estimated 10% of IHs (9-10). Currently, there is a lack of evidence-based standards for treatment of hemangiomas that require intervention. Treatment options that have been studied include high dose systemic corticosteroids, vincristine sulfate, and interferon. All of these options pose risk due to systemic side-effects with the additional burden of close monitoring (1, 11-12). In 2008, a case series of 11 children published in the NEJM by Leaute- Labreze et al., described the use of propranolol hydrochloride, a nonselective β-adrenergic blocking agent, as effective in arresting the growth and precipitating the rapid involution of extensive IHs (13). This report and the subsequent discussion and literature that have followed have changed the practice of many physicians treating infantile hemangiomas. Propranolol is now being used as treatment 10 for IHs due to the high efficacy and low side-effect profile (1). In many cases, it is considered first-line treatment. A subset of patients with IHs also undergo imaging of their lesion. The reasons to order imaging vary. If the clinical diagnosis is in question, imaging may be done to obtain a support for a diagnosis. Some lesions are imaged to assess the extent of involvement, especially those around the orbits. Also, hemangiomas that fail to improve on treatment may be imaged to rule out another diagnosis that may mimic IH, such as a vascular malformation. Imaging can be done using ultrasound, computed tomography (CT), and MR. Modalities such as plain radiograph and CT expose the child to radiation and are less effective at soft-tissue differentiation. High-resolution ultrasound does not expose the patient to radiation, but is very operator dependent. Therefore, MR is currently the modality of choice as it minimizes radiation exposure to the child and is able to analyze many facets of the lesion. MR provides the optimal way to characterize and diagnose the lesion and assess the vascular supply. MR is considered standard testing for syndromes with associated hemangiomas such as PHACE syndrome (posterior fossa malformations of the brain, large facial hemangiomas, arterial 11 anomalies, cardiac anomalies and aortic coarctation, and eye abnormalities) or PELVIS syndrome (perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag) (15-16). To date, there have been few reports regarding the specific MRI/MRA features of just infantile hemangiomas (17-20). The findings are often reported along with other soft tissue vascular tumors and malformations such as arterio-venous malformations and include a small number of hemangiomas a part of larger study with patients of a wide age range (19-20), which makes comparison to other studies difficult. The purpose of this study is to further describe specific MR findings of clinically diagnosed infantile hemangiomas as well as to compare the imaging diagnosis with the clinical diagnosis to determine diagnostic accuracy. Specifically we hypothesize that on MR examination the majority of IHs would be seen as lobulated and enhancing lesions with T2 hyper-intensity and high flow. We would not expect to see cystic spaces or phleboliths, as these should be common to vascular malformations. 12 The results of this study will aid both clinicians and radiologists to properly identify a suspected lesion as an infantile hemangioma on MRI. 13 Materials/Methods Design: A descriptive retrospective chart review on a convenience sample. Sample: All infants who received propranolol for the treatment of an infantile hemangioma in the pediatric dermatology clinic at Phoenix Children’s Hospital from January 1, 2008 to
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