SURGICAL TREATMENT OF RARE COMBINATION OF INTESTINAL MALROTATION WITH SECONDARY LYMPHANGIECTASIA
- Authors: Morozov D.A.1, Pimenova E.S.1, Tatochenko V.K.2, Bakradze M.D.2, Gadliya D.D.2, Morozova O.L.3, Talalaev A.G.2
-
Affiliations:
- Scientific Centre of Children’s Health, Moscow, Russian Federation Sechenov First Moscow State Medical University, Russian Federation
- Scientific Centre of Children’s Health, Moscow, Russian Federation
- Sechenov First Moscow State Medical University, Russian Federation
- Issue: Vol 70, No 1 (2015)
- Pages: 56-62
- Section: PEDIATRICS: CURRENT ISSUES
- Published: 28.01.2015
- URL: https://vestnikramn.spr-journal.ru/jour/article/view/72
- DOI: https://doi.org/10.15690/vramn.v70i1.1232
- ID: 72
Cite item
Full Text
Abstract
The rare combination of intestinal lymphangiectasia with malrotation of the duodenum in a child of three months of life is described. Basing on the literature review only 3 similar cases were described in the world practice. The boy with protein-losing enteropathy was examined at Moscow Scientific Centre of Children’s Health. The child had vomiting, diarrhea, loss in body weight, hypoproteinemia, lymphopenia. The infectious nature of the disease was excluded. It had been suggested the Waldman desease (primary intestinal lymphangiectasia). The prognosis for such disease is unfavorable. An examination of the child was continued against the backdrop of ongoing symptomatic therapy. Complete physical examination included monitoring laboratory blood tests, X-ray examination with contrast, CT-scan, gastroduodenoscopy with biopsy of the mucosa of the small intestine. Malrotation duodenum with the recurrent mid-gut volvulus with the development of secondary intestinal lymphangiectasia was diagnosed. Modern methods of examination and multidisciplinary approach made it possible to diagnose the case. Operation to eliminate fixation duodenum resulted in the recovery of the patient. At the present time the child grows and develops according to age and does not require treatment. The prognosis for this disease is regarded as favorable.
Keywords
About the authors
D. A. Morozov
Scientific Centre of Children’s Health, Moscow, Russian FederationSechenov First Moscow State Medical University, Russian Federation
Author for correspondence.
Email: damorozov@list.ru
Дмитрий Анатольевич, доктор медицинских наук, профессор, директор НИИ детской хирургии Научного центра здоровья детей, заведующий кафедрой детской хирургии и урологии-андрологии Первого Московского государственного медицинского университета им. И.М. Сеченова
Адрес: 119991, Москва, Ломоносовский пр-т, д. 2, стр. 1, тел.: +7 (499) 134-13-17
E. S. Pimenova
Scientific Centre of Children’s Health, Moscow, Russian FederationSechenov First Moscow State Medical University, Russian Federation
Email: evgeniyapimenova@list.ru
кандидат медицинских наук, доцент кафедры детской хирургии и урологии-андрологии Первого Московского государственного медицинского университета им. И.М. Сеченова, врач отделения общей хирургии НИИ детской хирургии Научного центра здоровья детей
Адрес: 119991, Москва, Ломоносовский пр-т, д. 2, стр. 1, тел.: +7 (499) 134-14-55
V. K. Tatochenko
Scientific Centre of Children’s Health, Moscow, Russian Federation
Email: tatovk@yandex.ru
доктор медицинских наук, профессор, главный научный сотрудник отделения пульмонологии и аллергологии НИИ педиатрии Научного центра здоровья детей
Адрес: 119991, Москва, Ломоносовский пр-т, д. 2, стр. 1, тел.: +7 (495) 967-14-21 Россия
M. D. Bakradze
Scientific Centre of Children’s Health, Moscow, Russian Federation
Email: bakradze.md@yandex.ru
доктор медицинских наук, заведующая отделением диагностики и восстановительного лечения НИИ педиатрии Научного центра здоровья детей
Адрес: 119991, Москва, Ломоносовский пр-т, д. 2, стр. 1, тел.: +7 (495) 967-14-21
D. D. Gadliya
Scientific Centre of Children’s Health, Moscow, Russian Federation
Email: apsni@inbox.ru
аспирант отделения диагностики и восстановительного лечения НИИ педиатрии Науч-ного центра здоровья детей
Адрес: 119991, Москва, Ломоносовский пр-т, д. 2, стр. 1, тел.: +7 (499) 134-07-05 Россия
O. L. Morozova
Sechenov First Moscow State Medical University, Russian Federation
Email: morozova_ol@list.ru
доктор медицинских наук, профессор кафедры патологической физиологии Первого Московского государственного медицинского университета им. И.М. Сеченова
Адрес: 119048, Москва, ул. Трубецкая, д. 8, тел.: +7 (499) 622-96-47 Россия
A. G. Talalaev
Scientific Centre of Children’s Health, Moscow, Russian Federation
Email: damorozov@list.ru
доктор медицинских наук, профессор, заведующий патологической лабораторией Научного центра здоровья детей
Адрес: 119991, Москва, Ломоносовский пр-т, д. 2, стр. 1, тел.: +7 (495) 967-14-21 Россия
References
- Davis N.M., Kurpios N.A., Sun X., Gros J., Martin J.F., Tabin C.J. The chirality of gut rotation derives from left-right asymmetric changes in the architecture of the dorsal mesentery. Dev. Cell. 2008; 1: 134–145.
- McVay M.R., Kokoska E.R., Jackson R.J., Smith S.D. Jack Barney Award. The changing spectrum of intestinal malrotation: diagnosis and management. Am. J. Surg. 2007; 6: 712–717.
- Ingoe R., Lange P. The Ladd’s procedure for correction of intestinal malrotation with volvulus in children. AORN J. 2007; 85 (2): 300–308.
- Williams H. Green for danger! Intestinal malrotation and volvulus. Arch. Dis. Child. Educ. Pract. Ed. 2007; 92 (3): 87–91.
- Penco J.M., Murillo J.C., Hernández A., de La Calle Pato U., Masjoan D.F., Aceituno F.R. Anomalies of intestinal rotation and fixation: consequences of late diagnosis beyond two years of age. Pediatr. Surg. Int. 2007; 23 (8): 723–730.
- Torres A.M., Ziegler M.M. Malrotation of the intestine. World J. Surg. 1993; 3: 326–331.
- Eksarko P., Nazir S., Kessler E. Duodenal web associated with malrotation and review of literature. J. Surg. Case Rep. 2013; 12: 110.
- Mall F.T. Development of the human intestine and its position in the adult. Bull. Johns Hopkins Hosp. 1898; 9: 197–210.
- Ladd W.E. Congenital obstruction of the duodenum in children. N. Engl. J. Med. 1932; 206: 277–283.
- Bairov G.A. Khirurgiya porokov razvitiya u detei [Surgery Malformations in Children]. Leningrad, Meditsina, 1968. 688 p.
- Bairov G.A., Doroshevskii Yu.L., Nemilova T.K. Atlas operatsii u novorozhdennykh [Atlas of Operations in Newborns]. Leningrad, Meditsina, 1984. 256 p.
- Doletskii S.Ya., Gavryushov V.V., Akopyan V.G. Khirurgiya novorozhdennykh [Neonatal Surgery]. Moscow, 1976. 320 p.
- Littman I. Operativnaya khirurgiya [Operative Surgery]. Budapesht,1985. 1176 p.
- Ashkraft K.U., Kholder T.M. Detskaya khirurgiya.Per. s angl [Pediatric Surgery. Translated from English]. St. Petersburg, 1996. 384 p.
- Puri P., Höllwarth M. E. Pediatric Surgery. Berlin: Springer. 2006. 646 p.
- Martin V., Shaw-Smith C. Review of genetic factors in intestinal malrotation. Pediatr. Surg. Int. 2010; 26 (8): 769–781.
- Kellogg E.L., Kellogg W.A. Chronic duodenal obstruction with duodeno-jejunostomy as a method of treatment report of forty one operations Ann. Surg. 1921; 73 (5): 578–608.
- Michael H. Childress. Duodenum Inversum. J. Natl. Med. Assoc. 1979; 71 (5): 515–516.
- Wayne E.R., Burrington J.D. Extrinsic duodenal obstruction in children. Surg. Gynecol. Obstet. 1973; 136 (1): 87–91.
- Waldmann T.A., Steinfeld J.L., Dutcher T.F., Davidson J.D., Gordon R.S. The role of the gastrointestinal system in «idiopathic hypoproteinemia». Gastroenterology. 1961; 41: 197–207.
- Proujansky R. Protein losing enteropathy. In: Pediatric gastrointestinal disease. J. Walker (ed.). Hamilton: BC Becker. 2000. Р. 89–95.
- Toshovski V. Ostrye protsessy v bryushnoi polosti u detei [Acute Processes in the Abdominal Cavity in Children]. Praga, Avitsenum, 1987. 472 p.
- Geras'kin A.V., Mokrushina O.G., Morozov D.A., Akhunzyanov A.A., Gumerova A.A. Status and prospects for improving surgical care to infants with developmental disabilities. Rossiiskii vestnik perinatologii i pediatrii = Russian bulletin of perinatology and pediatrics. 2009; 54 (6): 7–12.
- Murphy F.L., Sparnon A.L. Long term complications following intestinal malrotation and the Ladd’s procedure: a 15 year review. Pediatr. Surg. Int. 2006; 22 (4): 326–329.
- Orzech N., Navarro O.M., Langer J.C. Is ultrasonography a good screening test for intestinal malrotation? J. Pediatr. Surg. 2006; 41 (5): 1005–1009.
- Ziegler M.M., Azizkhan R.G., Weber T.R. Operative pediatric surgery. New York: McGraw-Hill Professional. 2003. 1340 p.
- Davidson J.D., Waldmann T.A., Goodman D.S., Gordon R.S. Protein losing gastroenteropathy in congestive heart-failure. Lancet. 1961; 1 (7183): 899–902.
- Ostrow A.M., Freeze H., Rychik J. Protein losing enteropathy after fontan operation: investigations into possible pathophysiologic mechanisms. Ann. Thorac. Surg. 2006; 82 (2): 695–700.
- Iida F., Wada R., Sato A., Yamada T. Clinicopathologic consideration of protein-losing enteropathy due to lymphangiectasia of the intestine. Surg. Gynecol. Obstet. 1980; 151 (3): 391–395.
- Tamamoto F., Takeuchi N., Shindou N., Sumi Y., Katayama H. A case of infantile protein losing gastroenteropathy due to chronic volvulus. Rinsho Hoshasen. 1988; 33 (4): 511–514.
- Zellos A., Zarganis D., Ypsiladis S., Chatzis D., Papaioannou G., Bartsocas C. Malrotation of the intestine and chronic volvulus as a cause of protein-losing enteropathy in infancy. Pediatrics. 2012; 129 (2): 515–518.