Anatomic and surgical features of lower extremities blood vessels in case of major veins dysplasia in children with various type and severity of the disease according to data of contrast flebography

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Abstract

Dysplasia of the main veins (DMV) is known by the names of the authors who described this pathology as Klippel – Trenone syndrome. The clinical picture of Klippel – Trenone syndrome (CTS) in the classical version is characterized by a triad of symptoms: vascular spots, varicose atypical veins, hypertrophy of soft tissues and bones with an increase in the volume and length of the affected limb. The incidence of this defect from all vascular malformations is 49.6%. To diagnose a malformation, ultrasound is used – Dopplerography, MRI and MSCT with contrast, ascending venography. Vasocontrast methods reveal various options for surgical anatomy of the veins of the affected limbs. To assess the anatomy of deep veins, we used upward contrasting, since we consider this species to be more accurate and low-cost. The study is based on the results of examination and treatment of 200 patients. Long-term results were analyzed in 108 patients with congenital malformations of the vessels of the lower extremities aged 1 year to 18 years, who were in the angiomicrosurgical department of the Pediatric Medical University from 2005 to 2015. Patients were divided into 2 groups: the embryonic type – 70 people (of which 42 boys and 28 girls), and the fetal type – 38 people (16 of them boys, 22 girls). In this paper, we analyze the results of ascending phlebography of the extremities, which is considered the gold standard for examining vein malformations. Contrast phlebography in 108 patients with dysplasia of the main veins, hypo- and dysplasia of segments of the deep and superficial veins were detected 63%; atypical location of superficial or deep veins with the presence of congenital pathways of the collateral outflow 30%, aplasia of the veins – 7%. Conclusions: phlebography data accurately determine the surgical anatomy of blood vessels in patients with dysplasia of the main veins. The data obtained during the ascending phlebography, in almost 100% of cases coincide with intraoperative data thus allowing the development of optimal tactics of operations in the veins, and also scrutinize the pathology of musculoskeletal system.

About the authors

Mikhail V. Azarov

St. Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Author for correspondence.
Email: Azarov_89@mail.ru

Postgraduate Student, Department of Surgical Diseases of Childhood

Russian Federation, Saint Petersburg

Dmitry D. Kupatadze

St. Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Email: ddkupatadze@gmail.com

MD, PhD, Dr Med Sci, Professor, Head, Department of Surgical Diseases of Childhood

Russian Federation, Saint Petersburg

Viktor V. Nabokov

St. Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Email: vn59@mail.ru

MD, PhD, Associate Professor, Head, Microsurgical Department, Department of Surgical Diseases of Childhood

Russian Federation, Saint Petersburg

Suren M. Kocharyan

St. Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Email: surik_05@mail.ru

Postgraduate Student, Department of Surgical Diseases of Childhood

Russian Federation, Saint Petersburg

References

  1. Васютков В.Я. Выбор метода лечения трофических язв голени у больных с хронической венозной недостаточностью нижних конечностей // Хирургия. Журнал им. Н.И. Пирогова. – 1986. – Т. 62. – № 10. – С. 103–108. [Vasyutkov VYa. Vybor metoda lecheniya troficheskikh yazv goleni u bol’nykh s khronicheskoy venoznoy nedostatochnost’yu nizhnikh konechnostey. Khirurgiia (Mosk). 1986;62(10)103-108. (In Russ.)]
  2. Веденский А.Н. Варикозная болезнь. – Ленинград: Медицина, 1983. – 208 с. [Vedenskiy AN. Varikoznaya bolezn’. Leningrad: Meditsina; 1983. 208 p. (In Russ.)]
  3. Дан В.Н., Сапелкин С.В. Ангиодисплазии (врожденные пороки развития сосудов). – М.: Вердана, 2009. – 200 с. [Dan VN, Sapelkin SV. Angiodisplazii (vrozhdennye poroki razvitiya sosudov). Moscow: Verdana; 2009. 200 p. (In Russ.)]
  4. Дан В.Н., Сапелкин С.В. Ангиодисплазии (глава 7). В кн.: Лучевая диагностика болезней сердца и сосудов: национальное руководство. – М.: ГЭОТАР-Медиа, 2011. – С. 641–668. [Dan VN, Sapelkin SV. Angiodisplazii (glava 7). In: Luchevaya diagnostika bolezney serdtsa i sosudov: natsional’noe rukovodstvo. Moscow: GEOTAR-Media; 2011. P. 641-668. (In Russ.)]
  5. Дан В.Н., Сапелкин С.В., Щеголев А.И. Современные классификации врожденных пороков развития сосудов (ангиодисплазий) // Ангиология и сосудистая хирургия. – 2006. – Т. 12. – № 4. – С. 28–33. [Dan VN, Sapelkin SV, Shchegolev AI. Present-day classifications of congenital vascular malformations (angiodysplasias). Angiology and vascular surgery. 2006;12(4):28-33. (In Russ.)]
  6. Исаков Ю.Ф., Тихонов Ю.А. Врожденные пороки периферических сосудов у детей. – М.: Медицина, 1974. – 116 с. [Isakov YuF, Tikhonov YuA. Vrozhdennye poroki perifericheskikh sosudov u detey. Moscow: Meditsina; 1974. 116 p. (In Russ.)]
  7. Исаков Ю.Ф., Водолазов Ю.Ф., Поляев Ю.А., и др. Эндоваскулярная окклюзия сосудов в лечении некоторых хирургических заболеваний у детей // Хирургия. – 1987. – Т. 63. – № 8. – С. 3–5. [Isakov YuF, Vodolazov YuF, Polyaev YuA, et al. Endovaskulyarnaya okklyuziya sosudov v lechenii nekotorykh khirurgicheskikh zabolevaniy u detey. Khirurgiia (Mosk). 1987;63(8):3-5. (In Russ.)]
  8. Кармазановский Г.Г., Дан В.Н., Скуба Н.Д. Компьютерно-томографическая характеристика ангиодисплазий с ангиоматозом в мягких тканях // Вестник рентгенологии и радиологии. – 1993. – Т. 68. – № 2. – С. 37–43. [Karmazanovskiy GG, Dan VN, Skuba ND. Komp’yuterno-tomograficheskaya kharakteristika angiodisplaziy s angiomatozom v myagkikh tkanyakh. Vestn Rentgenol Radiol. 1993;68(2):37-43. (In Russ.)]
  9. Купатадзе Д.Д. Ангиомикрохирургия в педиатрии. Монография. – СПб., 2016. [Kupatadze DD. Angiomikrokhirurgiya v pediatrii. Monografiya. Saint Petertsburg; 2016. (In Russ.)]
  10. Купатадзе Д.Д., Азаров М.В., Набоков В.В. Клиника, диагностика и лечение детей с дисплазией магистральных вен // Педиатр. – 2017. – Т. 8. – № 3. – С. 101–107. [Kupatadze DD, Azarov MV, Nabokov VV. Clinic, diagnosis and treatment of children with dysplasia of the main veins. Pediatrician (St. Petersburg). 2017;8(3):101-107. (In Russ.)]. https://doi.org/10.17816/PED83101-106.
  11. Купатадзе Д.Д., Набоков В.В., Азаров М.В. Дисплазия магистральных вен. В кн.: Ангиомикрохирургия в педиатрии. – СПб: Ассоциация графических искусств, 2016. – С. 5–57. [Kupatadze DD, Nabokov VV, Azarov MV. Displaziya magistral’nykh ven. V kn.: Angiomikrokhirurgiya v pediatrii. Saint Petersburg: Assotsiatsiya graficheskikh iskusstv; 2016. P. 5-57. (In Russ.)]
  12. Милованов А.П. Патоморфология ангиодисплазий конечностей. – М.: Медицина, 1978. – 144 с. [Milovanov AP. Patomorfologiya angiodisplaziy konechnostey. Moscow: Meditsina; 1978. 144 p. (In Russ.)]
  13. Рубашов С.М. Врожденные артериовенозные свищи. «Macrosomia partialis congenital» // Труды Белорусского Университета. – 1928. [Rubashov SM. Congenital arterio-venous fistulas. “Macrosomia partialis congenital”. Trudy Belorusskogo Universiteta. 1928. (In Russ.)]
  14. Шалимов А.А., Дрюк Н.Ф., Полищук Ю.Э., и др. Диагностика и хирургическое лечение артериовенозных ангиодисплазий периферических сосудов // Клиническая хирургия. – 1982. – № 7. – С. 5–10. [Shalimov AA, Dryuk NF, Polishchuk YuE, et al. Diagnostika i khirurgicheskoe lechenie arteriovenoznykh angiodisplaziy perifericheskikh sosudov. Klin Khir. 1982;(7):5-10. (In Russ.)]
  15. Cherry KJ, Gloviczki P, Stanson AW. Persistent sciatic vein: Diagnosis and treatment of a rare condition. J Vasc Surg. 1996;23(3):490-497. https://doi.org/10.1016/s0741-5214(96)80016-4.
  16. Greene AK, Goss JA. Vascular Anomalies: From a Clinicohistologic to a Genetic Framework. Plast Reconstr Surg. 2018;141(5):709e-717e. https://doi.org/10.1097/PRS.0000000000004294.
  17. Gloviczki P, Driscoll DJ. Klippel-Trenaunay syndrome: current management. Phlebology. 2007;22(6):291-298. https://doi.org/10.1258/026835507782655209.
  18. Husmann DA, Rathburn SR, Driscoll DJ. Klippel-Trenaunay syndrome: incidence and treatment of genitourinary sequelae. J Urol. 2007;177(4):1244-1249. https://doi.org/10.1016/j.juro.2006.11.099.
  19. Jacob AG, Driscoll DJ, Shaughnessy WJ, et al. Klippel-Trénaunay Syndrome: Spectrum and Management. Mayo Clin Proc. 1998;73(1):28-36. https://doi.org/10.1016/s0025-6196(11)63615-x.
  20. Klippel M, Trenaunay P. Naevus variqueux osteo-hypertrophique. J Prat Rev Gen Clin Ther. 1900;14:65-70.
  21. Klippel M, Trenaunay P. Du naevus variqueux osteo-hypertrophique. Arch Gen Med (Paris). 1900;3:641-672.
  22. Luks VL, Kamitaki N, Vivero MP, et al. Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA. J Pediatr. 2015;166(4):1048-1054.e5. https://doi.org/10.1016/j.jpeds.2014.12.069.
  23. Mulliken & Young’s Vascular Anomalies Hemangiomas and Malformations. 2nd ed. Ed. by J.B. Mulliken, P.E. Burrows, S.J. Fishman. Oxford: Oxford University Press; 2013. https://doi.org/10.1093/med/9780195145052.001.0001.
  24. Oduber CE, Young-Afat DA, van der Wal AC, et al. The persistent embryonic vein in Klippel-Trenaunay syndrome. Vasc Med. 2013;18(4):185-191. https://doi.org/10.1177/1358863X13498463.
  25. John PR. Klippel-Trenaunay Syndrome. Tech Vasc Interv Radiol. 2019;22(4):100634. https://doi.org/10.1016/j.tvir.2019.100634.
  26. Servelle M, Bastin R, Loygue J, et al. Hematuria and rectal bleeding in the child with Klippel and Trenaunay syndrome. Ann Surg. 1976;183(4):418-428. https://doi.org/10.1097/00000658-197604000-00016.
  27. Servelle M. Pathologie Vasculaire. Paris: Masson; 1978.
  28. Uller W, Fishman SJ, Alomari AI. Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg. 2014;23(4):208-215. https://doi.org/10.1053/j.sempedsurg.2014.06.013.
  29. Vahidnezhad H, Youssefian L, Uitto J. Klippel-Trenaunay syndrome belongs to the PIK3CA-related overgrowth spectrum (PROS). Exp Dermatol. 2016;25(1):17-19. https://doi.org/10.1111/exd.12826.
  30. Wassef M, Blei F, Adams D, et al. Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies. Pediatrics. 2015;136(1):e203-214. https://doi.org/10.1542/peds.2014-3673.

Copyright (c) 2020 Azarov M.V., Kupatadze D.D., Nabokov V.V., Kocharyan S.M.

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