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Research ArticleHead and Neck Imaging

A Radiologic Grading System for Assessing the Radiographic Outcome of Treatment in Lymphatic and Lymphatic-Venous Malformations of the Head and Neck

R. De Leacy, D.V. Bageac, S. Manna, B.S. Gershon, D. Kirke, T. Shigematsu, C. Sinclair, D. Chada, P. Som, A. Doshi, K. Nael and A. Berenstein
American Journal of Neuroradiology August 2021, DOI: https://doi.org/10.3174/ajnr.A7260
R. De Leacy
aFrom the Department of Neurosurgery (R.D.L., D.V.B., S.M., B.S.G., T.S., D.C., A.B.)
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D.V. Bageac
aFrom the Department of Neurosurgery (R.D.L., D.V.B., S.M., B.S.G., T.S., D.C., A.B.)
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S. Manna
aFrom the Department of Neurosurgery (R.D.L., D.V.B., S.M., B.S.G., T.S., D.C., A.B.)
bOtolaryngology-Head and Neck Surgery (S.M., D.K., C.S., P.S.)
cRadiology (S.M., P.S., A.D., K.N.), Icahn School of Medicine at Mount Sinai, New York
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B.S. Gershon
aFrom the Department of Neurosurgery (R.D.L., D.V.B., S.M., B.S.G., T.S., D.C., A.B.)
dCollege of Medicine (B.S.G.), SUNY Downstate, Brooklyn, New York, New York
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D. Kirke
bOtolaryngology-Head and Neck Surgery (S.M., D.K., C.S., P.S.)
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T. Shigematsu
aFrom the Department of Neurosurgery (R.D.L., D.V.B., S.M., B.S.G., T.S., D.C., A.B.)
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C. Sinclair
bOtolaryngology-Head and Neck Surgery (S.M., D.K., C.S., P.S.)
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D. Chada
aFrom the Department of Neurosurgery (R.D.L., D.V.B., S.M., B.S.G., T.S., D.C., A.B.)
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P. Som
bOtolaryngology-Head and Neck Surgery (S.M., D.K., C.S., P.S.)
cRadiology (S.M., P.S., A.D., K.N.), Icahn School of Medicine at Mount Sinai, New York
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A. Doshi
cRadiology (S.M., P.S., A.D., K.N.), Icahn School of Medicine at Mount Sinai, New York
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K. Nael
cRadiology (S.M., P.S., A.D., K.N.), Icahn School of Medicine at Mount Sinai, New York
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A. Berenstein
aFrom the Department of Neurosurgery (R.D.L., D.V.B., S.M., B.S.G., T.S., D.C., A.B.)
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  • FIG 1.
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    FIG 1.

    BDL grade 1 response. A 50-year-old man with dysphagia was found to have a de Serres stage II macrocystic LM of the head. A, Preprocedural T2-weighted axial MR imaging at the level of the hard palate shows a lesion primarily centered within the right parapharyngeal space (white arrow). B, Four  months later, following 1 sclerotherapy treatment, T2-weighted axial MR imaging at the same level shows BDL grade 1 complete regression of the lesion on cross-sectional imaging (white arrow).

  • FIG 2.
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    FIG 2.

    BDL grade 2 response. A 7-year-old girl with a left cheek mass was found to have a de Serres stage II mixed macro-/microcystic LM of the head. A, Preprocedural T2 fat-suppressed axial MR imaging at the level of the maxillary alveolar process shows involvement of the left buccal and parotid spaces (white arrow). B, Seven  months later, following 2 sclerotherapy treatments, T2 fat-suppressed axial MR imaging at the same level shows BDL grade 2 near-complete resolution of the LM with trace residuals (white arrow).

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    FIG 3.

    BDL grade 3B response. A 6-year-old boy with dysphagia was found to have a de Serres stage II mixed macro-/microsystic LM of the head. A, Preprocedural T2-weighted axial MR imaging at the level of the submandibular glands shows involvement of the left submandibular, sublingual, and parapharyngeal mucosal (not shown) spaces (white arrow). B, Thirty-seven months later, following 1 sclerotherapy treatment, T2-weighted axial MR imaging at the same level shows BDL grade 3B partial regression, with <50% estimated volume of residual malformation (white arrow) and intermediate signal granulation tissue formation within the treatment bed (white arrowhead). C, At the follow-up time point, note precontrast axial T1-weighted MR imaging isointensity of the suspected granuloma with muscle (white arrowhead). D, Postcontrast axial T1 at the same level demonstrates enhancement of the suspected granuloma (white arrowhead), confirming 3B grading.

  • FIG 4.
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    FIG 4.

    BDL grade 4 response. An 18-year-old woman with an extensive bilateral de Serres stage IV mixed macro-/microcystic LVM of the supra- and infrahyoid neck presents for sclerotherapy after receiving multiple treatments at an outside institution. A, Preprocedural T2-weighted axial MR imaging at the level of the alveolar process of the maxilla shows widespread LVM bilaterally (white arrow). B, Preprocedural T1 fat-saturated postcontrast axial MR imaging at the same level demonstrates enhancement of venous structures within the LVM (white arrow). C, Thirty-five  months later, following 1 sclerotherapy treatment targeting the left buccal region, repeat T2-weighted axial MR imaging at the same level shows BDL grade 4 partial regression (white arrow) with >50% estimated volume of residual malformation.

  • FIG 5.
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    FIG 5.

    BDL grade 5 response. A 2-month-old boy with tongue swelling was found to have a de Serres stage II mixed macro-/microcystic sublingual LM. A, Preprocedural T2 fat-suppressed axial MR imaging at the level of the mandibular symphysis shows involvement of the right and middle aspects of the sublingual space (white arrows). B, Five years later, following 1 sclerotherapy treatment, T2 fat-suppressed axial MR imaging at the same level shows BDL grade 5 recurrence of the LM without a gross interval change from baseline (white arrows).

  • FIG 6.
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    FIG 6.

    BDL grade 6 response. An 8-year-old boy with right facial prominence was found to have a de Serres stage II mixed macro-/microcystic LM. A, Preprocedural T2 fat-suppressed axial MR imaging at the level of the oropharynx shows trans-spatial involvement of the right neck extending from the right parotid and masticator spaces to the pharyngeal mucosal space (white arrow), sparing the retropharyngeal space. B, One  year later, following 2 sclerotherapy treatments, T2 fat-suppressed axial MR imaging at the same level shows BDL grade 6 regression within the treatment bed and extension into the retropharyngeal space, which was previously uninvolved (white arrow).

  • FIG 7.
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    FIG 7.

    BDL grade 7 progression. A neonate boy with right facial prominence was found to have a de Serres stage II macrocystic LM. A, Initial T2 fat-suppressed axial MR imaging at the level of the hard palate shows involvement of the right buccal space (white arrow). B, Three  years later, before any intervention, T2 fat-suppressed axial MR imaging at the same level shows BDL grade 7 progression with clear volume increase and extension into the masticator and parapharyngeal (white arrow).

Tables

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    Table 1:

    Grading system for assessing the radiographic outcome of treatment in LM-LVM malformations of the head and neck

    GradeDescription
    1Complete regression of the lesion on cross-sectional imaging
    2Near-complete regression with trace residual of the lesion on cross-sectional imaging
    3Partial regression with <50% estimated volume of residual malformation
    4Partial regression with >50% estimated volume of residual malformation
    5Minimal or no gross interval change
    6Regression of malformation in 1 region with progression into a previously uninvolved/untreated area
    7Gross interval progression
    Modifier BGranulation tissue formation in treatment bed
    • View popup
    Table 2:

    Patient age and average treatments

    MedianRange
    Age (yr)6.38(0.33–73.92)
    Imaging interval (mo)32.50(1–131)
    Treatments within imaging interval (mo)1.50(0–13)
    • View popup
    Table 3:

    Patient demographics and LV-LVM characteristics

    No.(%)
    Total56
    Sex
     Male2850.0%
     Female2850.0%
    Classification
     LM3766.1%
     LVM1933.9%
    Architecture
     Macrocystic916.1%
     Microcystic1933.9%
     Mixed2850.0%
    Lesion laterality
     Right1832.1%
     Left1628.6%
     Bilateral2239.3%
    de Serres stage
     I (unilateral infrahyoid)11.8%
     II (unilateral suprahyoid)2748.2%
     III (unilateral suprahyoid and infrahyoid)814.3%
     IV (bilateral suprahyoid)916.1%
     V (bilateral suprahyoid and infrahyoid)1119.6%
    • View popup
    Table 4:

    Frequency of grading outcomes

    Final GradeCountFrequency (%)
    135.4%
    2814.3%
    31730.4%
    4814.3%
    5712.5%
    6610.7%
    758.9%
    B Modifier23.6%
    • View popup
    Table 5:

    BDL interrater reliability

    Interrater Reliability MeasureStatistical ValueCI or P Value
    Krippendorff α statistic0.93(95% CI, 0.89–0.95)
    Intraclass coefficienta0.97(95% CI, 0.96–0.98)
    Spearman ρ (K.N. vs A.D.)0.90P < .001
    Spearman ρ (K.N. vs R.D.L.)0.93P < .001
    Spearman ρ (A.D. vs R.D.L.)0.95P < .001
    • ↵a Intraclass coefficients calculated for both consistency and absolute agreement yielded identical values.

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R. De Leacy, D.V. Bageac, S. Manna, B.S. Gershon, D. Kirke, T. Shigematsu, C. Sinclair, D. Chada, P. Som, A. Doshi, K. Nael, A. Berenstein
A Radiologic Grading System for Assessing the Radiographic Outcome of Treatment in Lymphatic and Lymphatic-Venous Malformations of the Head and Neck
American Journal of Neuroradiology Aug 2021, DOI: 10.3174/ajnr.A7260

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A Radiologic Grading System for Assessing the Radiographic Outcome of Treatment in Lymphatic and Lymphatic-Venous Malformations of the Head and Neck
R. De Leacy, D.V. Bageac, S. Manna, B.S. Gershon, D. Kirke, T. Shigematsu, C. Sinclair, D. Chada, P. Som, A. Doshi, K. Nael, A. Berenstein
American Journal of Neuroradiology Aug 2021, DOI: 10.3174/ajnr.A7260
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