Bile thickening syndrome in newborns and young children: risk factors, therapy, prognosis. А clinical case study
https://doi.org/10.21518/2079-701X-2020-18-134-141
Abstract
Bile thickening syndrome or biliary sludge causing obstruction of the common bile duct and impaired passage of bile into the duodenum ranks second among “surgical jaundice” in newborns and young children. The syndrome should be diagnosed early to conduct timely conservative treatment to avoid surgical intervention and complications such as acute pancreatitis, acute cholecystitis, cholelithiasis. Risk factors for bile thickening syndrome include prematurity, extremely low birth weight, longterm parenteral nutrition, administration of diuretics, third generation cephalosporins. If a threat of biliary sludge arises, repeat abdominal ultrasound imaging and identification of the following signs of this syndrome are indicated: dilatation of CBD more than 3 mm, the presence of heterogeneous sediment in the gallbladder and common bile duct. The article presents a clinical discussion of a bile thickening syndrome case in a an extremely low birth weight girl born at 29 weeks’ gestational age. The condition of the newborn at birth was very severe. The severity of the condition was caused by the type 1 respiratory distress syndrome, the girl was immediately intubated and mechanically ventilated. At the age of 1 month 10 days, the girl was transferred from the intensive care unit to the neonatal unit. The ultrasonic imaging showed an oval-shaped gallbladder of 26 x 6 mm in size, 0.5 ml in volume, instable angulation of the gallbladder body and neck, the walls of up to 2 mm in thickness, laminated, hyperechogenic, hyperechoic contents in the lumen (ointment-like bile), which allowed to draw a conclusion about the presence of bile thickening syndrome. Ursodeoxycholic acid at a dose of 20 mg/kg/day was prescribed immediately after ultrasonic imaging. 10 days later the dose of the drug was increased to 30 mg/kg/day. Two weeks later, the child was discharged home in satisfactory condition without signs of bile thickening syndrome. Knowledge of the risk factors for biliary sludge makes it possible to assign timely instrumental examination, establish early diagnosis and prescribe conservative therapy, and exclude surgical methods of treatment.
About the Authors
A. N. Goryaynova
Federal State Budgetary Educational Institution of Further Professional Education “Russian Medical Academy of Continuous Professional Education” of the Ministry of Healthcare of the Russian Federation
Russian Federation
Russian Federation
Cand. of Sci. (Med.), Assistant Professor, Department of Pediatrics with the course of polyclinic pediatrics named
after G.N. Speransky
2/1, Bldg. 1, Barrikadnaya St., Moscow, 125993, Russia
E. V. Belenovich
State Budgetary Institution of Healthcare of Moscow “Bashlyaeva City Children’s Clinical Hospital of the Department of Healthcare of Moscow“
Russian Federation
Russian Federation
Head of Neonatology Department No. 2
28, Geroev Panfilovtsev St., Moscow, 125373, Russia
A. A. Khudyakova
State Budgetary Institution of Healthcare of Moscow “Bashlyaeva City Children’s Clinical Hospital of the Department of Healthcare of Moscow“
Russian Federation
Russian Federation
Neonatologist, Neonatology Department No. 1
28, Geroev Panfilovtsev St., Moscow, 125373, Russia
Yu. A. Bronnikova
State Budgetary Institution of Healthcare of Moscow “Bashlyaeva City
Children’s Clinical Hospital of the Department of Healthcare of Moscow“
Russian Federation
Russian Federation
Ultrasonic Medical Investigation Specialist
28, Geroev Panfilovtsev St., Moscow, 125373, Russia
L. V. Churilova
State Budgetary Institution of Healthcare of Moscow “Bashlyaeva City
Children’s Clinical Hospital of the Department of Healthcare of Moscow“
Russian Federation
Russian Federation
Ultrasonic Medical Investigation Specialist
28, Geroev Panfilovtsev St., Moscow, 125373, Russia
References
1. Chen S.M., Liao Y.Y., Lin C.P. Inspissated bile syndrome: A rare cause of neonatal cholestasis. Pediatr Neonatol. 2020;61(4):449–450. doi: 10.1016/j.pedneo.2020.03.002.
2. Gottesman L.E., Del Vecchio M.T., Aronoff S.C. Etiologies of conjugated hyperbilirubinemia in infancy: a systematic review of 1692 subjects. BMC Pediatrics. 2015;15:192. doi: 10.1186/s12887-015-0506-5.
3. Fitzpatrick F., Jardine R., Farrant P., Karani J., Davenport M., Mieli-Vergani G., Baker A. Predictive Value of Bile Duct Dimensions Measured by Ultrasound in Neonates Presenting With Cholestasis. J Pediatr Gastroenterol Nutr. 2010;51(1):55–60. doi: 10.1097/MPG.0b013e3181d1b226.
4. Davenport M., Betalli P., D’Antiga L., Cheeseman P., Mieli-Vergani G., Howard E.R. The spectrum of surgical jaundice in infancy. J Pediatr Surg. 2003;38(10):1471–1479. doi: 10.1016/s0022-3468(03)00498-6.
5. Gubernick J.A., Rosenberg H.K., Ilaslan H., Kessler A. US approach to jaundice in infants and children. Radiographics. 2000;20(1):173–195. doi: 10.1148/radiographics.20.1.g00ja25173.
6. Ko C.W., Sekijim J.H., Lee S.P. Biliary sludge. Ann Intern Med. 1999; 130(4 Pt. 1):301–311. doi: 10.7326/0003-4819-130-4-199902160-00016.
7. Ladd W.E. Congenital obstruction of the bile ducts. Annals of Surgery. 1935;102(4):742–751. doi: 10.1097/00000658-193510000-00021.
8. Ko C.W., Murakami C., Sekijima J.H., Kim M.H., McDonald G.B., Lee S.P. Chemical composition of gallbladder sludge in patients after marrow transplantation. Am J Gastroenterol. 1996;91(6):1207–1210. Available at: https://pubmed.ncbi.nlm.nih.gov/8651172.
9. Kim Y.S., Kestell M.F., Lee SP. Gall-bladder sludge: lessons from ceftriaxone. J Gastroenterol Hepatol. 1992;7(6):618–621. doi: 10.1111/j.1440-1746.1992.tb01496.x.
10. Park H.Z., Lee S.P., Schy A.L. Ceftriaxone-associated gallbladder sludge. Identification of calcium-ceftriaxone salt as a major component of gallbladder precipitate. Gastroenterology. 1991;100(6):1665–1670. doi: 10.1016/0016-5085(91)90667-A.
11. Shiffman M.L., Keith F.B., Moore E.W. Pathogenesis of ceftriaxone-associated biliary sludge. in vitro studies of calcium-ceftriaxone binding and solubility. Gastroenterology. 1990;99(6):1772–1778. doi: 10.1016/0016-5085(90)90486-k.
12. Volodin N.N., Mukhina Yu.G., Chubarova A.I. (ed.). Neonatology. In: Volodin N.N., Mukhina Yu.G. (ed.). Childhood diseases. Moscow: Dinastiya; 2011. Vol. 1. 512 p. (In Russ.)
13. Bollu B.K., Dawrant M.J., Thacker K., Thomas G., Chenapragadda M., Gaskin K., Shun A. Inspissated bile syndrome; Safe and effective minimally invasive treatment with percutaneous cholecystostomy in neonates and infants. J Pediatr Surg. 2016;51(12):2119–2122. doi: 10.1016/j.jpedsurg.2016.09.053.
14. Miloh T., Rosenberg H.K., Kochin I., Kerkar N. Inspissated bile syndrome in a neonate treated with cefotaxime: sonographic aid to diagnosis, management, and follow-up. J Ultrasound Med. 2009;28(4):541–544. doi: 10.7863/jum.2009.28.4.541.
15. Rozmanic V., Banac S., Ivosevic D., Cace N. Biliary colic and sonographic evidence of pseudocholelithiasos 36 h after treatment with ceftriaxone. J Paediatr Child Health. 2006;42(10):658–661. Available at: https://www.researchgate.net/publication/6818041_Biliary_colic_and_sonographic_evidence_of_pseudocholelithiasos_36_h_after_treatment_with_ceftriaxone.
16. Schaad U.B., Tschäppeler H., Lentze M.J. Transient formation of precipitations in the gallbladder associated with ceftriaxone therapy. Pediatr Infect Dis. 1986;5(6):708–710. doi: 10.1097/00006454-198611000-00026.
17. Bor O., Dinleyici E.C., Kebapci M., Aydogdu S.D. Ceftriaxone-associated biliary sludge and pseudocholelithiasis during childhood: a prospective study. Pediatr Int. 2004;46(3):322–344. doi: 10.1111/j.1328-0867.2004.01884.x.
18. Klar A., Branski D., Akerman Y., Nadjari M., Berkun Y., Moise J. et al. Sludge ball, pseudolithiasis, cholelithiasis and choledocholithiasis from intrauterine life to 2 years: a 13-year follow- up. J Pediatr Gastroenterol Nutr. 2005;40(4):477–480. doi: 10.1097/01.mpg.0000151749.01631.21.
19. Jain A., Poddar U., Elhence P., Tripathi A., Shava U., Yachha S.K. Cholestasis in a neonate with ABO haemolytic disease of newborn following transfusion of ABO group-specific red cells compatible with neonatal serum: inspissated bile syndrome. Blood Transfus. 2014;12(4):621–623. doi: 10.2450/2014.0099-14.
20. Messing B., Bories C., Kuntslinger F., Bernier J.J. Does total parenteral nutrition induce gallbladder sludge formation and lithiasis? Gastroenterology. 1983;84(5 Pt. 1):1012–1019. Available at: https://pubmed.ncbi.nlm.nih.gov/6403401.
21. Chang S.H., Joo S.M., Yoon C.S., Lee K.H., Lee S.M. Percutaneous Transhepatic Biliary Drainage in a Two-Month-Old Infant with Inspissated Bile Syndrome. Yonsei Med J. 2018;59(7):904–907. doi: 10.3349/ymj.2018.59.7.904.
22. Hernanz-Schulman M., Ambrosino M.M., Freeman P.C., Quinn C.B. Common bile duct in children: sonographic dimensions. Radiology. 1995;195(1):193–195. doi: 10.1148/radiology.195.1.7892467.
23. Carroll B.A., Oppenheimer D.A., Muller H.H. High-frequency real-time ultrasound of the neonatal biliary system. Radiology. 1982;145(2):437–440. doi: 10.1148/radiology.145.2.7134449.
24. Heaton N.D., Davenport M., Howard E.R. Intraluminal biliary obstruction. Arch Dis Child. 1991;66(12):1395–1398. doi: 10.1136/adc.66.12.1395.
25. Berger S., Schibli S., Stranzinger E., Cholewa D. One-stage laparoscopic surgery for inspissated bile syndrome: case report and review of surgical techniques. Springerplus. 2013;2:648. doi: 10.1186/2193-1801-2-648.
26. Sideeka N, Shaikh R, Chaudry G. Single Procedure Saline Lavage for Treatment of Inspissated Bile. Case Rep Radiol. 2020;2020:8816599. doi: 10.1155/2020/8816599.
27. Gunnarsdóttir A, Holmqvist P, Arnbjörnsson E, Kullendorff CM. Laparoscopic aided cholecystostomy as a treatment of inspissated bile syndrome. J Pediatr Surg. 2008;43(4):e33–e55. doi: 10.1016/j.jpedsurg.2007.12.068.
28. Gao Z.G., Shao M., Xiong Q.X., Tou J.F., Liu W.G. Laparoscopic cholecystostomy and bile duct lavage for treatment of inspissated bile syndrome: a single-center experience. World J Pediatr. 2011;7(3):269–271. doi: 10.1007/s12519-011-0321-5.
29. Lieber J., Piersma F.E., Sturm E. et al. Surgical treatment of inspissated bile syndrome using a 2-stage pure laparoscopic approach: a case report. J Pediatr Surg. 2012; 47(12):e47–50. doi: 10.1016/j.jpedsurg.2012.09.043.
30. Helin R., Bhat R., Rao B. Ultrasound-guided percutaneous cholecystostomy for acute neonatal biliary obstruction. Neonatology. 2007;91(4):266–270. doi: 10.1159/000098174.
31. Dani C., Pratesi S., Raimondi F., Romagnoli C. Italian guidelines for the management and treatment of neonatal cholestasis. Ital J Pediatr. 2015;:41:69. doi: 10.1186/s13052-015-0178-7.
32. Jun W.Y., Cho M.J., Han H.S., Bae S.H. Use of Omega-3 Polyunsaturated Fatty Acids to Treat Inspissated Bile Syndrome: A Case Report. Pediatr Gastroenterol Hepatol Nutr. 2016;19(4):286–290. doi: 10.5223/pghn.2016.19.4.286.
33. Jonkers I.J., Smelt A.H., Princen H.M., Kuipers F., Romijn J.A., Boverhof R. et al. Fish oil increases bile acid synthesis in male patients with hypertriglyceridemia. J Nutr. 2006;136(4):987–991. doi: 10.1093/jn/136.4.987.
34. Bae S.H., Park H.S., Han H.S., Yun I.J. Omega-3 polyunsaturated fatty acid for cholestasis due to bile duct paucity. Pediatr Gastroenterol Hepatol Nutr. 2014;17(2):121–124. doi: 10.5223/pghn.2014.17.2.121.
35. Nandivada P., Carlson S.J., Chang M.I., Cowan E., Gura K.M., Puder M. Treatment of parenteral nutrition-associated liver disease: the role of lipid emulsions. Adv Nutr. 2013;4(6):711-717. doi: 10.3945/an.113.004770.
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For citations:
Goryaynova AN, Belenovich EV, Khudyakova AA, Bronnikova YA, Churilova LV. Bile thickening syndrome in newborns and young children: risk factors, therapy, prognosis. А clinical case study. Meditsinskiy sovet = Medical Council. 2020;(18):134-141. (In Russ.) https://doi.org/10.21518/2079-701X-2020-18-134-141
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