Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 1999  |  Volume : 5  |  Issue : 1  |  Page : 40-42
Spherocytosis presenting with choledocholithiasis in early childhood: A case report and review of the literature


Department of Pediatrics, University of Jordan, Amman, Jordan

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Date of Submission12-Nov-1997
Date of Acceptance23-Jun-1998
 

How to cite this article:
El-Halabi IM. Spherocytosis presenting with choledocholithiasis in early childhood: A case report and review of the literature. Saudi J Gastroenterol 1999;5:40-2

How to cite this URL:
El-Halabi IM. Spherocytosis presenting with choledocholithiasis in early childhood: A case report and review of the literature. Saudi J Gastroenterol [serial online] 1999 [cited 2019 Dec 5];5:40-2. Available from: http://www.saudijgastro.com/text.asp?1999/5/1/40/33526


Choledocholithiasis is a recognized condition throughout childhood. Predisposing factors include hemolytic anemia [1],[2] including spherocytosis [3],[4] and sickle cell disease [5],[6],[7] . Total parenteral nutrition for more than a week can predispose to bile sludge and stasis [8] . Ceftriaxone is also implicated as a lithogenic factor [9],[10] and infestation of the common bile duct by ascaris lumbricoides [11] is a well recognized predisposing factor. This case is reported because of the young age at presentation of spherocytosis as choledocholithiasis.


   Case presentation Top


SK is a thirteen-month-old female who presented to our clinic with one month history of jaundice with tea-colored urine but no definite change in the color of the stool. No history of vomiting or diarrhea. No similar condition was reported in family members or contacts. No history of blood transfusion, surgery or hospitalization. There is a history of similar but milder episode of jaundice, of undocumented severity and of undetermined nature, which according to the parents resolved spontaneously about two months before this presentation. No history of skin rash or joint pain. No history of drug intake. The patient's development is appropriate for age. Family history revealed that the mother had a history of anemia when she was young for which she took some pills, the nature of which was unclear to her and to her husband. Physical examination showed an alert conscious young female with vital signs within normal limits. Weight, height and head circumference were within one standard deviation below the mean. Yellowish discoloration of the sclera and skin was noted, and there were no dysmorphic features. No murmurs were heard on cardiac auscultation. Liver span was within normal limits for age and the spleen was not felt below the costal margin. The kidneys were not palpable and no masses, distension or ascitis were detected. No skin lesions, vertebral or limb anomaly.

Investigation included hemoglobin: 10.5 gm/dl, WBC:6.5K, no eosinophilia. Blood film showed macrocytic anemia with marked spherocytosis. Reticulocyte count 0.034 number fraction (normal 0.005-0.015 number fraction). Total serum iron and binding capacity were within normal. Total bilirubin: 174.5 micromol/L (normal 3.4-17.1 micromol/L), Direct 85.2 micromol/L (normal <3.4 micromol/L). ALT 95 U/L (normal 5-45 U/L), AST 85 U/L (normal 4/45 U/L), GGT 195 U/L (normal 5/32 U/L). Stool examination for ova and parasites, culture and sensitivity were negative. Hepatitis profile was negative for A. B & C viruses Coombs test was negative. Incubated osmotic fragility test at 37 C with NaCl concentration of 8.5 g/L resulted in hemolyzed fraction of 0.82 (normal is 0.00 at this concentration). The same test was performed on RBCs obtained from her siblings and parents. The only one that showed significant fragility was the one from the mother (0.2 hemolyzed fraction at 8.5 g/L NaCl, normal is 0.00). Hemoglobin electropheresis showed a normal pattern. Hepatobiliary/pancreatic ultrasound showed dilated common bile duct, no gall bladder pathology, normal pancreas Endoscopic Retrograde Cholangiopancreaticography (ERCP) showed multiple soft stones in the common bile duct, sphincterotomy was done, the stones were extremely fragile and disintegrated during retrieval [Figure - 1].

The patient was started on folic acid supplements and showed remarkable improvement on subsequent visits. Ultrasound was repeated on two of the subsequent visits and showed normal structure of the biliary tree.


   Discussion Top


In this case the age of presentation was felt to be unusually young. Choledocholithiasis was reported at the seventh and ninth year of life associated with spherocytosis [12] . Although Pappis et al reported 419 children with chronic hemolytic anemia 53 of whom developed choledocholitiasis, the youngest patient was 61/2 years [2] . Neonatal hemolysis rather than red cell membrane defect was reported by Debray et al as a predisposing factor for infancy Choledocholithiasis in two patients [1] .

Choledocholithiasis due to hemolysis is of the black pigment stones type. Increased bilirubin excretion secondary to hemolysis leads to super abundance of unconjugated bilirubin [13] . When the solubility of bile is exceeded, the excess unconjugated bilirubin precipitates as calcium bilirubinate, which polymerizes, binds to mucin produced by the gallbladder mucosa and is retained. The black color results from the structure of this polymer which absorbs light throughout the physical spectrum [14] . Besides supersaturation with unconjugated bilirubin, calcium salts and mucin act as nidus to initiate stone growth [15] . Enhanced betaglucuronidase activity may originate from bacteria present in infected bile. Increased pH from defective acidification of bile by the gallbladder increases the available free calcium [16] . These stones become radiopaque when significant quantities of calcium carbonates and phosphates are present. Black pigment stones do not respond to medical therapy and usually need endoscopic intervention by ERCP or cholecystectomy [14] . It is concluded that spherocytosis may present with choledocholithiasis in infants. Accordingly, the search for stones and predisposing factors should be done in all infants and children presenting with obstructive jaundice.

 
   References Top

1.Debray D, Pariente D. Myara A, Bernard O. Cholelithiasis in infancy: a study of 40 cases. J Pediatr 1993;122:385-91.  Back to cited text no. 1    
2.Pappis CH, Galanakis S, Moussatos G, Keramidas D, Kattamis C. Experience of splenectomy and cholecystectomy in children with chronic hemolytic anemia. J Pediatr Surg 1989;24:543-6.  Back to cited text no. 2    
3.Young LE, Izzo MJ, Platzer RF. Hereditary spherocytosis, clinical hematologic & genetic features in 28 cases with particular reference to the osmotic and mechanical fragility of incubated erythrocytes. Blood 1951;6:1073-7.  Back to cited text no. 3    
4.Hurst D, Vichinsky EP. Splenectomy indications in childhood, in: Pochedlly C, Sills RH, Schwartz AD, (Eds). Disorders of the spleen pathophysiology & management. New York. Marcel Decker 1989.  Back to cited text no. 4    
5.Winter SS, Kinney TR, Ware RE. Gallbladder sludge in children with sickle cell disease. J Pediatr 1994;125:747-9.  Back to cited text no. 5    
6.Serjeant GR. The clinical features of sickle cell disease. Baillieres Clin Haematol 1993;6:93-115.  Back to cited text no. 6    
7.Okuonghae HO, Szlachetka R, Sagay AS. Cholelithiasis in children with homozygous sickle cell anaemia in northern Nigeria. Trop Geogr Med 1993;45:44-55.  Back to cited text no. 7    
8.Komura J, Yano H, Tanaka Y, Tsuru T. Increased incidence of cholestasis during total parenteral nutrition in children factors affecting stone formation. Kureme Med J 1993;40:7-11.  Back to cited text no. 8    
9.Stabile A, Ferrara P, Maietti G, Maresca G. Ceftriaxone associated gallbladder lithiasis in children (Letter). Fur J Pediatr 1995;154:590.  Back to cited text no. 9    
10.Blais C, Duperval R. Biliary psuedolthiasis in a child associated with 2 days of ceftriaxone therapy. Pediatr Radiol 1994;24:218-9.  Back to cited text no. 10    
11.Schulman A. Intrahepatic biliary stones: imaging features and a possible relationship with Ascaris lumbricoides. Clin Radiol 1993;47:325-32.  Back to cited text no. 11    
12.Iwai N. Tokiwa K, Tsuto T, Yanagihara J, Takahashi T. Cholelithiasis in children with congenital spherocytosis. Z Kinderchir 1986;41:308-10.  Back to cited text no. 12    
13.Ostrow JD. The etiology of pigment gallstones. Hepatology 1984;4(suppl.5):215-22.  Back to cited text no. 13    
14.Eldon A. Shaffer. Gallbladder disease, in: Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watkins JB (Eds) Pediatric Gaastrointestinal disease. Philadelphia: BC decker, 1991.  Back to cited text no. 14    
15.Cahalane MJ, Neudrand MW, Carey MC. Physical-chemical pathogenesis of pigment gallstones. Semin Liver Dis 1988;8:317-28.  Back to cited text no. 15    
16.Rege RV, Moore EW. Pathogenesis of calcium-containing gallstones. Canine ductular bile, but not gallbladder bile is supersaturated with calcium carbonate. J Clin Invest 1986;77:21-6.  Back to cited text no. 16    

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Correspondence Address:
Issam M El-Halabi
Assistant Professor & Director Division of Pediatric Gastroenterology, University of Jordan, Amman
Jordan
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Source of Support: None, Conflict of Interest: None


PMID: 19864760

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