Alt Gastrointestinal Kanaması Olan bir Yenidoğanda Bağırsak Lenfanjiyomatozu: Tanı Zorluğu

Öz

Doğumsal kistik lenfatik malformasyonlar, yumuşak dokular, akciğerler, abdominal organlar ve kemiklerin lenfatik dokusunu etkileyen nadir bozukluklardır. Biz, doğum sonrası kanlı dışkı ile başvuran ve daha sonra jejunumda lenfanjiyom tanısı konan bir yenidoğan kız olguyu sunuyoruz. Olgumuzun ilginç yönü, şiddetli aktif kanamanın medikal tedavi ve kan transfüzyonu ile kontrol altına alınamaması ve bunun pediatrik cerrahi ekibi tarafından acil cerrahi müdahale gerektirmesiydi. Operasyon sırasında jejunal bağırsak segmentinde lenf sıvısı ile dolu iki kistik yapı tespit edildi ve rezeksiyon ile çıkarıldı. Doğumsal kistik intestinal lenfanjiyom tanısı, yenidoğan döneminde rektal kanama ile başvuran, yaşamı tehdit eden bir durum olarak düşünülmelidir.

Anahtar Kelimeler

• Yenidoğan
• konjenital kistik intestinal lenfanjiom
• kanlı dışkı
• kistektomi

Intestinal Lymphangiomatosis in a Newborn with Lower Gastrointestinal Bleeding: Diagnostic Challenge

Öz

Congenital cystic lymphatic malformations are rare disorders of the lymphatic tissue lining the soft tissues, lungs, abdominal organs, and bones. We report a case of a female neonate who presented with bloody stools and was subsequently diagnosed with a jejunal lymphangioma. An interesting aspect of our case was that the severe active hemorrhage could not be controlled with medical management and blood transfusion, necessitating urgent surgical intervention by the pediatric surgery team. During the operation, two cystic structures filled with lymphatic fluid were identified in the jejunal bowel loop and were removed by resection. The diagnosis of congenital cystic intestinal lymphangioma, a life-threatening condition presenting with rectal bleeding in the neonatal period, should be considered.

Anahtar Kelimeler

• Newborn
• congenital cystic intestinal lymphangioma
• bloody faeces
• cystectomy

Kaynakça

1. 1.Al Laham O, Abdul Khalek G, Almaydaani M, Abazid E,Abazeed O, Alshalabi A. A rare occurrence of an incidental primary intra-abdominal Cystic Lymphangioma in a Middle Eastern adult female: A case report. Ann Med Surg (Lond) 2023;18: 231-235.
2. 2.Tran D, Fallat ME, Buchino JJ. Lymphangiomatosis: A casereport. South Med J. 2005; 98: 669-671.
3. 3.Ding XL, Yin XY, Yu YN, et al. Lymphangiomatosisassociated with protein-losing enteropathy: A case report.World J Clin Cases. 2021; 9: 3758-3764.
4. 4.Baskın D, Narcı A, Okur N, et al. Cystic lymphangiomatosiswith severe intra-abdominal bleeding in a newborn: case report. J Clin Ultrasound. 2013; 41: 261-4.
5. 5.Valakada J, Madhusudhan KS, Ranjan G, et al. Abdominal lymphangiomatosis with intestinal lymphangiectasia diagnosed by magnetic resonance lymphangiography: A case report. CurrProbl Diagn Radiol. 2018; 47: 200–202.
6. 6.Ilhan M, Oner G, Alibeyoglu A, et al. Primary intestinal lymphangiomatosis of the ileum in an adult—the role ofsurgical approach. J Surg Case Reports. 2016; 8: rjw133.
7. 7.Giuliani A, Romano L, Coletti G, et al. Lymphangiomatosis of the ileum with perforation: A case report and review of the literature. Ann Med Surg. 2019; 41:6–10.
8. 8.Chan WYS, Kwan KEL, Teo LT. A rare case of retroperitoneal and mesenteric lymphangiomatosis. Radiol Case Reports. 2020; 15:11–14.

9. 9.Suthiwartnarueput W, Kiatipunsodsai S, Kwankua A, et al. Lymphangioma of the small bowel mesentery: A case report and review of the literature. World J Gastroenterol. 2012; 18: 6328–6332.
10. 10.Alqahtani A, Nguyen LT, Flageole H, et al. 25 Years’experience with lymphangiomas in children. J Pediatr Surg.1999; 34:1164–1168.
11. 11.Güvenç BH, Ekingen G, Tuzlaci A, et al. Diffuse neonatal abdominal lymphangiomatosis: management by limitedsurgical excision and sclerotherapy. Pediatr Surg Int. 2005;21: 595-598.
12. 12.Tuncer A, Karavelioğlu A, Yavaş B, et al. Mesenteric cystic lymphangioma causes with abdominal pain: Case report and review of the literature. Turkish Association of PediatricSurgeons. 2013: 66-99.
13. 13.Blask AN, Fagen K. Prenatal Imaging of the Gastrointestinal Tract with Postnatal Imaging Correlation. Ultrasound Q. 2016; 32:15-24.
14. 14.Iwabuchi A, Otaka M, Okuyama A, et al. Disseminated intra-abdominal cystic ymphangiomatosis with severe intestinal bleeding. A case report. J Clin Gastroenterol. 1997; 25: 383-386.
15. 15.Ho M, Lee CC, Chang YY, et al. Prenatal diagnosis of lymphangiomas at unusual locations: report of three cases. JMed Ultrasound 2002; 10: 32.
16. 16.Puligandla PS, Nguyen LT, St-Vil D, et al. Gastrointestinal duplications. J Pediatr Surg. 2003;38(5):740-744.