Lympho Scintigraphy and Lymphangiography of Lymphangiectasia

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Lympho Scintigraphy and Lymphangiography of Lymphangiectasia supplement to qualitative interpretation of scintiscans, pulmo 13. Hirose Y, lmaeda T, Doi H, Kokubo M, Sakai 5, Hirose H. Lung perfusion SPECT in nary perfusion scintigraphy will become a more useful tech predicting postoperative pulmonary function in lung cancer. Ann Nuc/ Med 1993:7: 123—126. nique for clinical evaluation of treatment and assessment of 14. Hosokawa N, Tanabe M, Satoh K. et al. Prediction of postoperative pulmonary breathlessness and respiratory failure than the usual one. function using 99mTcMAA perfusion lung SPECT. Nippon Ada Radio/ 1995;55:414— 422. 15. Richards-Catty C, Mishkin FS. Hepatic activity on perfusion lung images. Semi,, Nuci REFERENCES Med l987;l7:85—86. I. Wagner UN Jr. Sabiston DC ir, McAee JG, Tow D, Stem HS. Diagnosis of massive 16. Kitani K, Taplin GV. 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Lympho scintigraphy and Lymphangiography of Lymphangiectasia Douglas Howarth and Peter Gloviczki Department ofNuclear Medicine, John Hunter Hospital, Newcastle, Australia; and Division of Vascular Surgery, Mayo Clinic, Rochester, Minnesota syndrome (1,2). The more common congenital cardiovascular Chronic genital edema secondary to lymphangiectasia and chylous abnormalities include pulmonary valvular stenosis, hypertro reflux in a 23-yr-old man with Noonan syndrome was investigated by 90'@'Tcsulfur nanocolloid lymphoscintigraphy and bipedal con phic cardiomyopathy and atrial septal defect; however, abnor trast lymphangiography. Lymphoscintigraphy showed a delayed malities ofthe lymphatic system are also well recognized (3—7). lymphatic flow pattern in the pelvis, abdomen and chest consistent In some patients, peripheral lymphoscintigraphy may be helpful with lymphangiectasia and abnormal lymphatic flow dynamics. in distinguishing primary lymphedema from secondary Lymphangiography showed dilated and tortuous abnormal lym lymphedema and further evaluating congenital lymphatic ab phatics in the abdomen and pelvis. Ligation of incompetent retro normalities (8). Peripheral lymphoscintigraphy demonstrates peritoneal lymph vessels and lymphaticovenous anastamosis were normal or abnormal lymphatic transport of radiolabeled nano performed, resulting in clinical improvement. Lymphangiectasia has colloid in patterns that may be diagnostic, but it more often been described previously in Noonan syndrome, but it is relatively provides additional information confirming or refuting the uncommon below the diaphragm. This case demonstrates the use clinical diagnosis. Accurate anatomical detail is not, however, of lymphoscintigraphy and lymphangiography in providing impor tant physiological and anatomical information before surgical inter provided by lymphoscintigraphy and, under some circum vention. Careful presurgical planning using such tests also allows stances, when lymphatic surgery is planned lymphangiography the most appropriate operation to be performed. may also be helpful. We describe a patient with retroperitoneal Key Words: Noonan syndrome; lymphangiectasia; lymphoscintig lymphangiectasia in whom lymphoscintigraphy and lym raphy; lymphangiography phangiography were complementary in planning lymphatic surgery. J NucI Med I998 39'.1635—I638 CASE REPORT Noonansyndromeischaracterizedbywide-rangingphenoA 23-yr-old man with a sporadic form of Noonan syndrome typic features, many of which are also seen in Turner's characterized by slightly wide-spaced eyes and low-set ears, mild pectus excavatum, previous surgically corrected right Received Oct. 1, 1997; accepted Jan. 12, 1998. cryptorchidism and moderate pulmonary stenosis/regurgitation Forcorrespondenceor reprintscontact:Dot4asHowarth,MD, Departmentof Nuclear Medicine, John Hunter Hospftal, Locked Bag No. 1, NewcasUe Reg@nal Mail presented with a 7-yr history of genital edema and chronic Centre,2310NewSouthWales,AUStr@. painless eruption of scrotal vesicles associated with fluid LYMPHOSCINTIGRAPHY AND LYMPHANGIOGRAPHY OF LYMPHANGIECTASIA •Howarth and Gloviczki 1635 A Ant •Post :B.@Ant PostS. @ •,- .@. @ . • . ,@‘.@ @.t. .@ @ .. • . — ::,@C @ . .-@ a; .. @ . - . :@•@ @ .. .:..‘@ :@..4 I @ • ...@i.- . @ t.' ‘4 4 4' @ . @@;‘@:: : :1: @ . ,. a .. .‘‘@ @;k /@••., .-...,@ .@:3Hr FiGURE1. @A4Lymphoscintigraphyat 1 hr shows radiotracerat injectionsites in both feet and relativelysymmetrical,rapkl radiOtraCeruptake in inguinal regionsbilaterally.MilddiffuselyabnormalradiOtraCerdistributionisseen inpelvisand scrotumconsistentwithIymphangiectaSla.Inaddition,unusualpattern of muftipladiscrete lymphnodes seen along lymphaticchannels of lowerextremitiesis of uncertalnsignificance.No radiotraceractivityis seen in liver, indicatingdelayed flowof lymphfromdilated peMc and thoracic lymphaticchannels intovenous circulation.(B)Lymphoscintigraphyat 3 hr shows a@Ad radiotracer uptake in iliaclymphatics, and refltndngradiotracer is seen inscrotum, petineum, retroperitoneal and verylikely in mesenteric lymph vessels. More diffuseradiOtraceruptake is seen in central abdomen and thorax consistent wfthlymphangiectasia.Uver is now visualized,indicatingfree passage of radiotracer from lymphatics into systemic circulation. Persistent uptake of radiOtraCeris seen in unusual pattern of lymph nodes in lower extremities. leaking from the scrotal skin surface. Physical examination bifurcation were ligated. A microscopically guided end-to-end revealed erythema and skin thickening of the scrotum. In lymphovenous anastomosis between a large retroperitoneal addition, there were multiple scrotal vesicles that discharged a lymphatic vessel and one of the smaller mesenteric veins was whitish fluid. MRI performed 3 yr earlier showed diffuse performed. Excellent lymphatic flow was seen through the lymphangiectasia in the pelvis extending bilaterally into the anastamosis. Despite very high lymphatic pressures, there was scrotum and superiorly
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