SURGICAL TREATMENT OF RARE COMBINATION OF INTESTINAL MALROTATION WITH SECONDARY LYMPHANGIECTASIA

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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.

 

About the authors

D. A. Morozov

Scientific Centre of Children’s Health, Moscow, Russian Federation
Sechenov 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

Russian Federation

E. S. Pimenova

Scientific Centre of Children’s Health, Moscow, Russian Federation
Sechenov 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

Russian Federation

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 Russian Federation

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

Russian Federation

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 Russian Federation

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 Russian Federation

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 Russian Federation

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