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Miri A, Abdessalam S, Powers AM, Quiros‐Tejeira RE, Dike CR. Gallbladder remnant: A potential source for biliary stones postcholecystectomy; a case report in a child with sickle cell disease. JPGN REPORTS 2024; 5:162-165. [PMID: 38756117 PMCID: PMC11093913 DOI: 10.1002/jpr3.12039] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 08/28/2023] [Revised: 11/28/2023] [Accepted: 12/24/2023] [Indexed: 05/18/2024]
Abstract
Stone formation in a gallbladder remnant is a rare postcholecystectomy complication. This report describes the case of gallstones in a gallbladder remnant of an adolescent with sickle cell disease (SCD) years after laparoscopic cholecystectomy. A 15-year-old female with SCD presented to our gastroenterology clinic with concerns of recurrent choledocholithiasis despite cholecystectomy 2 years before presentation. About 4 months before presentation to our clinic, she was evaluated at the referring physician's emergency department for recurrent severe abdominal pain of 1 month duration. After admission to the hospital, common bile duct stones were seen on magnetic resonance cholangiopancreatography (MCRP) imaging and subsequently removed via endoscopic retrograde cholangiopancreatography (ERCP). On review of her MRCP and ERCP at our hospital, a remnant of gallbladder containing multiple stones was identified. She subsequently underwent a laparoscopic resection of the gallbladder remnant. Clinicians should consider biliary duct imaging in children with biliary colic following cholecystectomy, especially those with history of chronic hemolysis.
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Affiliation(s)
- Ahmad Miri
- Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and NutritionUniversity of Nebraska Medical CenterOmahaNebraskaUSA
| | | | - Andria M. Powers
- Department of Pediatrics, Division of Pediatric RadiologyUniversity of Nebraska Medical CenterOmahaNebraskaUSA
| | - Ruben E. Quiros‐Tejeira
- Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and NutritionUniversity of Nebraska Medical CenterOmahaNebraskaUSA
| | - Chinenye R. Dike
- Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and NutritionUniversity of Nebraska Medical CenterOmahaNebraskaUSA
- Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and NutritionUniversity of Alabama at BirminghamBirminghamAlabamaUSA
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Al Shamousi K, Al Maimani M. Prevalence and Risk Factors of Choledocholithiasis in Omani Patients With Sickle Cell Disease Undergoing Endoscopic Retrograde Cholangiopancreatography: A Retrospective Analysis. Cureus 2023; 15:e51133. [PMID: 38274900 PMCID: PMC10810723 DOI: 10.7759/cureus.51133] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/26/2023] [Indexed: 01/27/2024] Open
Abstract
Background Sickle cell disease (SCD) is a prevalent genetic disorder in the Middle East, particularly in Oman, leading to significant morbidity. It is caused by a mutation in the gene encoding hemoglobin (Hb) molecules, resulting in the formation and polymerization of hemoglobin S (HbS), which subsequently leads to hemolysis. Chronic hemolysis in SCD patients often results in various complications, including increased bilirubin levels in the gallbladder and the formation of pigmented biliary stones, which may obstruct the biliary tract system. In such cases, endoscopic retrograde cholangiopancreatography (ERCP) is often employed as a diagnostic and therapeutic tool to manage biliary complications. Objectives and rationale Considering the lack of studies on the Omani population with SCD, our study aims to determine the incidence of biliary stone formation in SCD patients undergoing ERCP and identify associated risk factors. Subjects and methods This retrospective study included 79 SCD patients aged over 12 years who underwent ERCP at Sultan Qaboos University Hospital, Muscat, Oman, between January 2010 and January 2023. Patient data were extracted from medical records. Continuous variables were analyzed using mean and standard deviation calculations, with independent sample t-tests for mean comparisons. The chi-square test assessed associations between categorized variables, with a p-value of ≤0.05 denoting statistical significance. Results The prevalence of choledocholithiasis in SCD patients undergoing ERCP was 67.1%. The incidence was higher in females (68.9%) than males (65.9%), in patients aged 12-29 (71.2%) compared to those aged ≥29 (59.3%), in patients with SCD (70.6%) versus sickle cell thalassemia (66.1%), and in those who had undergone gallbladder removal (80.0%) compared to those who did not (61.3%). Prevalence was also higher in patients not using hydroxyurea and folic acid (70.6% and 84.6%, respectively); however, chi-square analysis showed no significant association (p-value > 0.05). Additionally, t-test comparisons of HbS and HbF levels showed no significant differences. Conclusion This study documents a high prevalence of choledocholithiasis (67.1%) in SCD patients undergoing ERCP. Although the prevalence is notable, the examined risk factors did not show a significant association with stone formation.
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Affiliation(s)
| | - Maimoona Al Maimani
- Internal Medicine, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, OMN
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Shah R, Taborda C, Chawla S. Acute and chronic hepatobiliary manifestations of sickle cell disease: A review. World J Gastrointest Pathophysiol 2017; 8:108-116. [PMID: 28868180 PMCID: PMC5561431 DOI: 10.4291/wjgp.v8.i3.108] [Citation(s) in RCA: 78] [Impact Index Per Article: 9.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/21/2017] [Revised: 06/02/2017] [Accepted: 07/24/2017] [Indexed: 02/06/2023] Open
Abstract
Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as “sickle cell hepatopathy”. This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis. In this review, we detail the pathophysiology, clinical presentation and biochemical features of various acute and chronic hepatobiliary manifestations of SCD and present and evaluate existing evidence with regards to management of this disease process. We also discuss recent advances and controversies such as the role of liver transplantation in sickle cell hepatopathy and highlight important questions in this field which would require further research. Our aim with this review is to help increase the understanding, aid in early diagnosis and improve management of this important disease process.
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Muroni M, Loi V, Lionnet F, Girot R, Houry S. Prophylactic laparoscopic cholecystectomy in adult sickle cell disease patients with cholelithiasis: A prospective cohort study. Int J Surg 2015; 22:62-6. [PMID: 26278661 DOI: 10.1016/j.ijsu.2015.07.708] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/01/2015] [Revised: 07/09/2015] [Accepted: 07/29/2015] [Indexed: 12/15/2022]
Abstract
INTRODUCTION Prophylactic laparoscopic cholecystectomy remains controversial and has been discussed for selected subgroups of patients with asymptomatic cholelithiasis who are at high risk of developing complications such as chronic haemolytic conditions. Cholelithiasis is a frequent condition for patients with sickle cell disease (SCD). Complications from cholelithiasis may dramatically increase morbidity for these patients. Our objective was to evaluate the effectiveness of prophylactic cholecystectomy in SCD patients with asymptomatic gallbladder stones. METHODS From January 2000 to June 2014, we performed 103 laparoscopic cholecystectomies on SCD patients. Fifty-two patients had asymptomatic cholelithiasis. The asymptomatic patients were prospectively enrolled in this study, and all underwent a prophylactic cholecystectomy with an intraoperative cholangiography. The symptomatic patients were retrospectively studied. Upon admission, all patients were administered specific perioperative management including intravenous hydration, antibiotic prophylaxis, oxygenation, and intravenous painkillers, as well as the subcutaneous administration of low-molecular-weight heparin. During the same period, 51 patients with SCD underwent a cholecystectomy for symptomatic cholelithiasis. We compared these 2 groups in terms of postoperative mortality, morbidity, and hospital stay. RESULTS There were no postoperative deaths or injuries to the bile ducts in either group. In the asymptomatic group, we observed 6 postoperative complications (11.5%), and in the symptomatic group, there were 13 (25.5%) postoperative complications. DISCUSSION Regarding the SCD complications, we observed 1 case (2%) of acute chest syndrome in an asymptomatic cholelithiasis patient, while there were 3 cases (6%) in the symptomatic group. Vaso-occlusive crisis was observed in 1 patient (2%) with asymptomatic cholelithiasis, and in 4 patients (8%) in the other group. The mean hospital stay averaged 5.8 (4-17) days for prophylactic cholecystectomy and 7.96 (4-18) days for the comparative symptomatic group. CONCLUSIONS Postoperative complications related to SCD were less frequent for asymptomatic patients who had a laparoscopic prophylactic cholecystectomy. This intervention, if performed with perioperative specific management, is safe and helps avoid emergency operations for acute complications including cholecystitis, choledocholithiasis, and cholangitis. For SCD patients, a prophylactic cholecystectomy reduces hospital stays.
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Affiliation(s)
- Mirko Muroni
- Hôpital Tenon, Department of Surgery, 4 rue de la Chine, 75020, Paris, France.
| | - Valeria Loi
- Hôpital Tenon, Department of Surgery, 4 rue de la Chine, 75020, Paris, France.
| | - François Lionnet
- Hôpital Tenon, Department of Hematology, 4 rue de la Chine, 75020, Paris, France.
| | - Robert Girot
- Hôpital Tenon, Department of Hematology, 4 rue de la Chine, 75020, Paris, France.
| | - Sidney Houry
- Hôpital Tenon, Department of Surgery, 4 rue de la Chine, 75020, Paris, France.
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Gale HI, Setty BN, Sprinz PG, Doros G, Williams DD, Morrison TC, Kalajian TA, Tu P, Mundluru SN, Mehta MN, Castro-Aragon I. Implications of radiologic-pathologic correlation for gallbladder disease in children and young adults with sickle cell disease. Emerg Radiol 2015; 22:543-51. [PMID: 26109240 DOI: 10.1007/s10140-015-1326-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2015] [Accepted: 05/19/2015] [Indexed: 11/25/2022]
Abstract
The purpose of this study is to describe gallbladder imaging findings in patients with sickle cell disease, and to determine how they correspond with occurrence of complications, need for cholecystectomy, and surgical pathology. This study is IRB approved and HIPAA compliant. Informed consent requirements were waived. We reviewed records of 77 children with sickle cell disease ages 0-18 years at the time of their first gallbladder imaging study. Demographics, hospital courses, and radiologic and pathologic reports were collected. Two pediatric radiologists independently and retrospectively reviewed the imaging studies. Statistical analysis was performed using kappa statistic, chi-squared test, and ANOVA F-test. Continuous variables were described with mean, median, variance, and range. Patients who underwent cholecystectomy (N = 25) were more likely than the patients who did not undergo cholecystectomy (N = 52) to have gallstones or sludge (100 versus 36.5 %, p = <0.0001) or other gallbladder or biliary abnormality (70.8 versus 1.9 %, p = <0.0001). Patients who did not undergo cholecystectomy more frequently had normal-appearing gallbladders and biliary tracts (63.5 versus 0 %, p = <0.0001). Ninety-two percent of patients with cholecystectomy had chronic cholecystitis on pathology, and 96 % had a complication, including chronic cholecystitis and sequelae of biliary obstruction. Young patients with sickle cell disease, cholelithiasis, and any other biliary imaging abnormality will almost certainly require cholecystectomy, and many will experience complications. The most common surgical pathologic diagnosis in this group is chronic cholecystitis, which has a variable radiologic appearance. Our findings support recommendations to perform elective cholecystectomy for children and young adults with sickle cell disease and cholelithiasis or gallbladder sludge.
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Affiliation(s)
- Heather I Gale
- Boston University/Boston Medical Center, 820 Harrison Avenue FGH Building 3rd Floor, Boston, MA, 02118, USA.
| | - Bindu N Setty
- Boston University/Boston Medical Center, 820 Harrison Avenue FGH Building 3rd Floor, Boston, MA, 02118, USA
| | - Philippa G Sprinz
- Boston University/Boston Medical Center, 850 Harrison Avenue, Yawkey, 4S01, Boston, MA, 02118, USA
| | - Gheorghe Doros
- Boston University, 801 Massachusetts Avenue, Boston, MA, 02118, USA
| | - Don D Williams
- Boston University/Boston Medical Center, 820 Harrison Avenue FGH Building 3rd Floor, Boston, MA, 02118, USA
| | - Trevor C Morrison
- Boston University/Boston Medical Center, 820 Harrison Avenue FGH Building 3rd Floor, Boston, MA, 02118, USA
| | - Tyler A Kalajian
- Medical Clinical Sciences/Graduate Medical Studies, Boston University School of Medicine, 820 Harrison Avenue FGH Building 3rd Floor, Boston, MA, 02118, USA
| | - Powen Tu
- Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA, 02215, USA
| | - Shankar N Mundluru
- Boston University School of Medicine, 820 Harrison Avenue FGH Building 3rd Floor, Boston, MA, 02118, USA
| | - Manisha N Mehta
- Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA, 02215, USA
| | - Ilse Castro-Aragon
- Boston University/Boston Medical Center, 820 Harrison Avenue FGH Building 3rd Floor, Boston, MA, 02118, USA
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Amoako MO, Casella JF, Strouse JJ. High rates of recurrent biliary tract obstruction in children with sickle cell disease. Pediatr Blood Cancer 2013; 60:650-2. [PMID: 23255346 PMCID: PMC3977003 DOI: 10.1002/pbc.24413] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2011] [Accepted: 11/01/2012] [Indexed: 11/11/2022]
Abstract
BACKGROUND Individuals with sickle cell disease (SCD) have an increased risk of cholelithiasis from bilirubin stones. Symptomatic biliary tract disease (BTD) includes acute and chronic cholecystitis, obstruction of the common bile duct (CBD), cholangitis, and gallstone pancreatitis. Cholecystectomy is the main treatment strategy for symptomatic patients; however, the prevalence of recurrent BTD following cholecystectomy has not been systematically evaluated. We conducted a retrospective cohort study to describe the recurrence of BTD after cholecystectomy and characterize risk factors for recurrent disease. PROCEDURE We identified patients <22 years of age who presented to the Johns Hopkins Children Center with symptomatic BTD from July 1993 to June 2008. RESULTS We identified 56 patients with a total of 76 episodes of symptomatic BTD (median age at first event 15.9, range 4.6-21.5 years). Eleven of the 56 patients (19.6%) had at least one episode of recurrent symptomatic BTD (median follow-up of 5.3 years). Baseline characteristics were similar between the patients with a single episode of BTD and those with recurrent BTD. CONCLUSIONS These results demonstrate that recurrent BTD is a frequent complication of SCD (20% by age 4 years) and often presents as CBD obstruction by stone, despite cholecystectomy. In our cohort, recurrence was not associated with age at first episode, baseline total bilirubin, gender, or genotype of SCD.
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Affiliation(s)
| | - James F. Casella
- Division of Pediatric Hematology, Dept of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD
| | - John J. Strouse
- Division of Pediatric Hematology, Dept of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD,Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD,Corresponding author: John J. Strouse, MD, PhD, Ross 1125, 720 Rutland Ave, Baltimore, MD 21205 USA,
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Laparoscopic cholecystectomy in children with sickle cell anemia and the role of ERCP. Surg Laparosc Endosc Percutan Tech 2012; 22:139-42. [PMID: 22487628 DOI: 10.1097/sle.0b013e3182471b1c] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Patients with sickle cell anemia (SCA) have a high incidence of cholelithiasis and choledocholithiasis. This report is an analysis of our experience with laparoscopic cholecystectomy (LC) for children with SCA and the role of endoscopic retrograde cholangiopancreatography (ERCP). PATIENTS AND METHODS The records of children with SCA who had cholecystectomy were retrospectively reviewed for age, sex, hemoglobin level, hemoglobin electrophoresis, indication for cholecystectomy, operative time, hospital stay, and postoperative complications. They were divided into 2 groups, open cholecystectomy (OC) group and LC group, and the 2 were compared in terms of operative time, hospital stay, and postoperative complications. RESULTS Over a period of 15 years (January 1995 and December 2009), 94 children with SCA had cholecystectomy. Thirty-five (19 males and 16 females) had OC, 52 (28 males and 24 females) had LC, and 7 (4 males and 3 females) had LC and splenectomy. Their age ranged from 4 to 15 years (mean, 11.4 y). The indications for cholecystectomy were biliary dyspepsia and biliary colic (55), acute cholecystitis (7), obstructive jaundice (17), asymptomatic (12), and biliary pancreatitis (3). All those who had OC underwent intraoperative cholangiogram, 9 of them (25.7%) had common bile duct (CBD) exploration and 2 transduodenal sphincterotomy. Of those who had LC, 13 (25%) underwent preoperative ERCP, which was normal in 1, showed dilated CBD with no stones in 2, and dilated CBD with stones in 7. In 3, ERCP showed dilated CBD with enlarged, inflammed papilla suggestive of recent stone passage. Nine underwent endoscopic sphincterotomy and stone extraction followed by LC. There was no mortality; 1 (2.1%) required conversion to OC and another underwent postoperative exploration because of bleeding from an accessory cystic artery. In the LC group, 4 (7.7%) developed minor postoperative complications, whereas 8 (22.9%) in the OC group developed complications. CONCLUSIONS With proper perioperative management, LC is feasible, safe, and superior to OC in children with SCA with regard to postoperative complications, duration of hospital stay, cosmetic appearance, and postoperative recovery. LC should be the treatment of choice for both symptomatic and asymptomatic cholelithiasis in children with SCA. ERCP is a valuable diagnostic and therapeutic investigation both preoperatively and postoperatively. The sequential approach of endoscopic sphincterotomy and stone extraction followed by LC is a safe and effective approach for the management of cholelithiasis and choledocholithiasis in children with SCA.
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Impact of hydroxyurea on perioperative management and outcomes in children with sickle cell anemia. J Pediatr Hematol Oncol 2011; 33:487-90. [PMID: 21941139 DOI: 10.1097/mph.0b013e318230b2f4] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
Hydroxyurea has enhanced the treatment for children with sickle cell anemia. The objectives of this study were to compare perioperative transfusions and outcomes for children taking hydroxyurea versus those not taking hydroxyurea. We retrospectively reviewed perioperative management and outcomes for 51 children with sickle cell anemia (HbSS genotype) who underwent surgery in our center between January 2003 and April 2008. Of the 51 patients, 30 (59%) were taking hydroxyurea and 21 (41%) were not taking hydroxyurea. Eight of 30 (27%) in the hydroxyurea group were not transfused preoperatively, 12 of 30 (40%) received a single transfusion and 10 of 30 (33%) received serial transfusions, compared with 1 of 21 (5%) children in the nonhydroxyurea group who was not transfused, 2 of 21 (10%) who received a single transfusion and 18 of 21 (85%) who received serial transfusions or pheresis (P=0.004; for comparison across groups). One patient not taking hydroxyurea developed a delayed hyperhemolytic transfusion reaction, and 2 children taking hydroxyurea developed acute chest syndrome. Overall, children taking hydroxyurea had substantially fewer transfusions compared with children not taking hydroxyurea. Both groups of children had a low complication rate. Further research should be done to optimize perioperative management of children taking hydroxyurea.
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Issa H, Al-Salem AH. Role of ERCP in the era of laparoscopic cholecystectomy for the evaluation of choledocholithiasis in sickle cell anemia. World J Gastroenterol 2011; 17:1844-7. [PMID: 21528058 PMCID: PMC3080719 DOI: 10.3748/wjg.v17.i14.1844] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/15/2010] [Revised: 08/06/2010] [Accepted: 08/13/2010] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) for choledocholithiasis in patients with sickle cell anemia (SCA) in the era of laparoscopic cholecystectomy (LC).
METHODS: Two hundred and twenty four patients (144 male, 80 female; mean age, 22.4 years; range, 5-70 years) with SCA underwent ERCP as part of their evaluation for cholestatic jaundice (CJ). The indications for ERCP were: CJ only in 97, CJ and dilated bile ducts on ultrasound in 103, and CJ and common bile duct (CBD) stones on ultrasound in 42.
RESULTS: In total, CBD stones were found in 88 (39.3%) patients and there was evidence of recent stone passage in 16. Fifteen were post-LC patients. These had endoscopic sphincterotomy and stone extraction. The remaining 73 had endoscopic sphincterotomy and stone extraction followed by LC without an intraoperative cholangiogram.
CONCLUSION: In patients with SCA and cholelithiasis, ERCP is valuable whether preoperative or postoperative, and in none was there a need to perform intraoperative cholangiography. Sequential endoscopic sphincterotomy and stone extraction followed by LC is beneficial in these patients. Endoscopic sphincterotomy may also prove to be useful in these patients as it may prevent the future development of biliary sludge and bile duct stones.
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Ballas SK. Sickle Cell Pain. Pain Manag 2011. [DOI: 10.1016/b978-1-4377-0721-2.00026-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022] Open
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Gastrointestinal and hepatic complications of sickle cell disease. Clin Gastroenterol Hepatol 2010; 8:483-9; quiz e70. [PMID: 20215064 DOI: 10.1016/j.cgh.2010.02.016] [Citation(s) in RCA: 86] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2009] [Revised: 02/12/2010] [Accepted: 02/21/2010] [Indexed: 02/07/2023]
Abstract
Sickle cell disease (SCD) is an autosomal recessive abnormality of the beta-globin chain of hemoglobin (Hb), resulting in poorly deformable sickled cells that cause microvascular occlusion and hemolytic anemia. The spleen is almost always affected by SCD, with microinfarcts within the first 36 months of life resulting in splenic atrophy. Acute liver disorders causing right-sided abdominal pain include acute vaso-occlusive crisis, liver infarction, and acute hepatic crisis. Chronic liver disease might be due to hemosiderosis and hepatitis and possibly to SCD itself if small, clinically silent microvascular occlusions occur chronically. Black pigment gallstones caused by elevated bilirubin excretion are common. Their small size permits them to travel into the common bile duct but cause only low-grade obstruction, so hyperbilirubinemia rather than bile duct dilatation is typical. Whether cholecystectomy should be done in asymptomatic individuals is controversial. The most common laboratory abnormality is an elevation of unconjugated bilirubin level. Bilirubin and lactate dehydrogenase levels correlate with one another, suggesting that chronic hemolysis and ineffective erythropoiesis, rather than liver disease, are the sources of hyperbilirubinemia. Abdominal pain is very common in SCD and is usually due to sickling, which resolves with supportive care. Computed tomography scans might be ordered for severe or unremitting pain. The liver typically shows sickled erythrocytes and Kupffer cell enlargement acutely and hemosiderosis chronically. The safety of liver biopsies has been questioned, particularly during acute sickling crisis. Treatments include blood transfusions, exchange transfusions, iron-chelating agents, hydroxyurea, and allogeneic stem-cell transplantation.
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Issa H, Al-Haddad A, Al-Salem AH. ERCP in Patients With Sickle Cell Disease: Diagnostic and Therapeutic Dilemmas. Gastroenterology Res 2010; 3:74-78. [PMID: 27956989 PMCID: PMC5139873 DOI: 10.4021/gr2010.03.177w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/17/2010] [Indexed: 11/03/2022] Open
Abstract
BACKGROUND Cholestatic jaundice (CJ) in patients with sickle cell disease (SCD) poses diagnostic and therapeutic dilemmas. This is an evaluation of the role of ERCP in SCD. METHODS A total of 224 SCD patients with CJ had ERCP. The indications for ERCP were based on clinical and biochemical evidence of CJ and ultrasound findings. RESULTS The indications were: CJ only in79, CJ and dilated ducts in 103, and CJ and biliary stones in 42. The ERCP findings were: (A) For those with CJ only: ERCP was normal in 45, showed dilated ducts with no stones in 13, dilated ducts with stones in 16, normal CBD with a stone in 1; (B) For those with CJ, dilated ducts: ERCP was normal in 17, showed dilated ducts with stones in 47, dilated ducts without stones in 28, normal CBD with a stone in 1, a choledochoduodenal fistula in 2; (C) For those with CJ and duct stones: ERCP was normal in 2, showed dilated ducts with stones in 21, dilated ducts without stones in 14, normal CBD with a stone in 1. CONCLUSIONS ERCP was unnecessary in a significant number (27%) of patients. This is especially so for those with CJ only (57%). These should be evaluated further prior to ERCP. There was also a significant number (19%) who had ES for duct dilatation without an obstruction. The reason for this dilatation is not known and the value of ES in this group needs to be investigated further.
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Affiliation(s)
- Hussain Issa
- Department of internal medicine, Qatif Central Hospital, Qatif, Saudi Arabia; Current affiliation: King Fahad Specialist Hospital-Dammam, Saudi Arabia
| | - Ali Al-Haddad
- Department of internal medicine, Qatif Central Hospital, Qatif, Saudi Arabia
| | - Ahmed H Al-Salem
- Department of internal medicine, Qatif Central Hospital, Qatif, Saudi Arabia; Current affiliation: Maternity and Children Hospital-Dammam, Saudi Arabia
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Abstract
Sickle cell anemia is one of the common hemoglobinopathies around the world. It results from a single change of one amino acid valine instead of glutamic acid in the hemoglobin beta change. This change leads to polymerization of the hemoglobin when the oxygen saturation is lowered, resulting in deformity of the red blood cells and microvascular occlusion. Sickle cell anemia can affect any part of the body and one of the main organs to be affected is the hepatobiliary system either directly from the sicklening process or indirectly as a result of chronic hemolysis and multiple blood transfusions. This manifests in several clinical conditions which poses diagnostic and therapeutic dilemmas to the treating physicians. These hepatobiliary manifestations will be outlined in this review.
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Affiliation(s)
- Hussain Issa
- Department of Internal Medicine, Division of Gastroenterology, King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Ahmed H Al-Salem
- Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia
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Issa H, Al-Haddad A, Al-Salem A. Sickle cell cholangiopathy: An endoscopic retrograde cholangiopancreatography evaluation. World J Gastroenterol 2009; 15:5316-20. [PMID: 19908340 PMCID: PMC2776859 DOI: 10.3748/wjg.15.5316] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
AIM: To evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) in patients with sickle cell disease (SCD).
METHODS: Two hundred and twenty four SCD patients with cholestatic jaundice (CJ) had ERCP. The indications for ERCP were based on clinical and biochemical evidence of CJ and ultrasound findings.
RESULTS: Two hundred and forty ERCPs were performed. The indications for ERCP were: CJ only in 79, CJ and dilated bile ducts without stones in 103, and CJ and bile duct stones in 42. For those with CJ only, ERCP was normal in 42 (53.2%), and 13 (16.5%) had dilated bile ducts without an obstructive cause. In the remaining 22, there were bile duct stones with or without dilation. For those with CJ, dilated bile ducts and no stones, ERCP was normal in 17 (16.5%), and 28 (27.2%) had dilated bile ducts without an obstructive cause. In the remaining 58, there were bile ducts stones with or without dilation. For those with CJ and bile duct stones, ERCP was normal in two (4.8%), and 14 (33.3%) had dilated bile ducts without an obstructive cause. In the remaining 26, there were bile duct stones with or without dilatation.
CONCLUSION: Considering the high frequency of biliary sludge and bile duct stones in SCD, endoscopic sphincterotomy might prove helpful in these patients.
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Lebensburger J, Esbenshade A, Blakely M, Hankins J, Wang W. Biloma and pneumobilia in sickle cell disease. Pediatr Blood Cancer 2008; 51:288-90. [PMID: 18421713 DOI: 10.1002/pbc.21575] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
Spontaneous biloma and pneumobilia developed in a patient with sickle cell disease (SCD) who presented with acute abdominal pain, jaundice, and fever. Bilomas are fluid-filled cysts that develop from a bile leak and are located outside of the biliary system. Pneumobilia is characterized by gas within the biliary system due to an abnormal connection between the biliary tree and adjacent organs, particularly the gastrointestinal tract. Although hepatobiliary complications are common in SCD, we report on biloma and pneumobilia in a young patient with this condition.
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Affiliation(s)
- Jeffrey Lebensburger
- Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
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Irizarry K, Rossbach HC, Ignacio JRA, Winesett MP, Kaiser GC, Kumar M, Gilbert-Barness E, Wilsey MJ. Sickle cell intrahepatic cholestasis with cholelithiasis. Pediatr Hematol Oncol 2006; 23:95-102. [PMID: 16651237 DOI: 10.1080/08880010500456964] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Sickle cell intrahepatic cholestasis (SCIC) is a rare complication seen in sickle cell patients who present with sudden onset of RUQ pain, progressive hepatomegaly, mild elevation of transaminases, coagulopathy, and extreme hyperbilirubinemia. Early recognition of this entity is essential to avoid life-threatening complications. Diagnosis can be challenging given the overlap in clinical presentation with other conditions affecting the hepatobiliary biliary system in sickle cell anemia such as hepatitis, cholecystitis, and hepatic crisis. Treatment is currently limited to exchange transfusion. The authors present two patients with SCIC and cholelithiasis; the clinical picture of one is complicated by choledocholithiasis.
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Affiliation(s)
- Karina Irizarry
- Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA.
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17
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Seleem MI, Al-Hashemy AM, Meshref SS. Mini-laparoscopic cholecystectomy in children under 10 years of age with sickle cell disease. ANZ J Surg 2005; 75:562-5. [PMID: 15972048 DOI: 10.1111/j.1445-2197.2005.03437.x] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
BACKGROUND Cholelithiasis is very common in patients with sickle cell disease (SCD) and is responsible for recurrent attacks of abdominal pain. The ideal management, especially for children, remains controversial. The purpose of the present study was to evaluate the safety and outcome of mini-laparoscopic cholecystectomy (MLC) in young children under age of 10 years with SCD. METHODS A prospective study was carried out of 75 children with SCD under 10 years of age with recurrent abdominal pains seen between August 2001 and March 2004 at Armed Forces Hospital, Khamis Mushayt, Saudi Arabia, who were screened for cholelithiasis. Twelve (16%) of the 75 children were found to have gallstones. The mean age was 7.8 years (range 4-9 years). All 12 children underwent MLC. Anaemia was corrected preoperatively in all the patients. Operative time, intraoperative complications, hospital stay, and postoperative recurrent abdominal pain were recorded. RESULTS The mean operating time was 46.5 min (range: 35-65 min). Intraoperative cholangiogram failed in two children due to narrow cystic ducts. The mean hospital stay was 2.1 days (range: 2-4 days). No patient required intra-abdominal drain. The mean follow-up period was 13.4 months (range: 4-24 months). The only postoperative complication was deep jaundice 1 month postoperatively due to cholestasis, and this responded to medical treatment. None of the children had recurrent abdominal pain after MLC. CONCLUSION Mini-laparoscopic cholecystectomy is a safe surgical procedure for the management of cholelithiasis in children with SCD and leads to improvement in the quality of life by decreasing the frequency of recurrent abdominal pain.
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Affiliation(s)
- Mohamed I Seleem
- Department of Surgery, Armed Forces Hospitals, Southern Region, Khamis Mushayt, Saudi Arabia.
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Abstract
Sickle cell disease is a serious and life threatening disease that affects approximately 1 in 600 African-Americans. Since its first description in 1910 by Herrick, our understanding and treatment of this disease has grown tremendously. Most pediatricians will encounter one or more children with sickle cell disease and should have a thorough understanding of the disease, its complications, and treatment. This article summarizes present day knowledge about the complications and potential treatments for patients with sickle cell disease.
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Affiliation(s)
- Jason Fixler
- Children's Hospital and Research Center at Oakland, Department of Hematology/Oncology, 747 52nd Street, Oakland, CA 94609, USA
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20
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Duncan ND, McDonald AH, Mitchell DI. Cholecystectomy in Jamaican children with homozygous sickle-cell disease. Trop Doct 2000; 30:214-6. [PMID: 11075654 DOI: 10.1177/004947550003000411] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Twenty-seven children aged 18 years and under with homozygous sickle-cell disease had open cholecystectomy for symptomatic gallstones over the 12-year period 1985-1997. Emergency procedures (done during period of acute exacerbation of symptoms) were performed on 16 patients. Four with haemoglobin levels greater than 1 g/dl below their steady state received a simple blood transfusion preoperatively designed to raise haemoglobin levels to 10 g/dl. All had acute or acute on chronic cholecystitis based on histological examination of gallbladder specimens. Twelve had common bile duct stones. In two patients calculi were missed intraoperatively but these subsequently passed into the duodenum after a period saline irrigation via an in-situ t-tube. Six developed the acute chest syndrome (aetiology not determined) and this progressed to multi system failure and death in one. This high level of postoperative mobility and mortality may in part be due to the high proportion of emergency procedures.
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Affiliation(s)
- N D Duncan
- Department of Surgery, Radiology, Anaesthesia and Intensive Care, University Hospital of the West Indies, Kingston 7, Jamaica.
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Affiliation(s)
- A H Al-Salem
- Department of Surgery, Division of Pediatric Surgery, Qatif Central Hospital, Qatif, Saudi Arabia.
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22
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Abstract
The prevalence, incidence, risk factors, clinical associations, and morbidity of gallstones were studied in 311 patients with homozygous sickle cell disease and 167 patients with sickle cell-hemoglobin C disease in a cohort study from birth. Gallstones developed in 96 patients with homozygous sickle cell disease and 18 patients with sickle cell-hemoglobin C disease; specific symptoms necessitating cholecystectomy occurred in only 7 patients with homozygous sickle cell disease.
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Affiliation(s)
- T M Walker
- Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston, Jamaica
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23
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Diagne I, Badiane M, Moreira C, Signate-Sy H, Ndiaye O, Lopez-Sall P, Preira-Sylla G, Camara B, Diouf S, Diack-Mbaye A, Fall M. [Cholelithiasis and homozygote drepanocytosis in children in Dakar (Senegal)]. Arch Pediatr 1999; 6:1286-92. [PMID: 10627899 DOI: 10.1016/s0929-693x(00)88890-9] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023]
Abstract
BACKGROUND Cholelithiasis is a well recognized complication of sickle-cell anemia (SCA) because of chronic hemolysis. It is usually asymptomatic but may result in acute cholecystitis or cholangitis. The aim of this study was to assess prevalence of cholelithiasis and its associated factors among Senegalese children and adolescents with SCA. PATIENTS AND METHODS We analyzed the follow-up records and results of systematic clinical, biological and ultrasonographic examinations in 106 patients with SCA aged 11 months to 22 years (median: 10 years, 6 months), followed up in the Albert-Royer Children's Hospital (Dakar). RESULTS Cholelithiasis was detected in 9.4% of the patients. Gender, ethnic group, geographic origin and socioeconomic conditions did not have any association with cholelithiasis frequency. The youngest patient with cholelithiasis was 7 years old, and prevalence of cholelithiasis increased with age. The mean age at the beginning of SCA follow-up was higher among patients with cholelithiasis. They were more frequently transfused and tended to present more frequent pain crises and other complications of SCA. We found no spontaneous clinical manifestations attributed to cholelithiasis. Nevertheless, provoked right upper quadrant pain was more frequently observed in patients with cholelithiasis. Fetal hemoglobin, steady-state hemoglobin, reticulocytes count, serum bilirubin, alkaline phosphatase and transaminase levels were not significantly different in these patients, compared to the others. However, those with cholelithiasis had significantly higher mean red cell volume. CONCLUSION Prevalence of cholelithiasis appears relatively low in Senegalese patients with SCA, probably owing to the tolerance of the Senegal haplotype. Advanced age and severe chronic hemolysis are etiologic factors in evidence. We recommend systematic abdominal ultrasound at least once a year for patients older than 5 years, or when right upper quadrant pain is observed. Cholecystectomy should be performed in cases of cholelithiasis in order to prevent complications.
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Affiliation(s)
- I Diagne
- Chaire de pédiatrie, Université Cheikh Anta Diop de Dakar (UCAD), Hôpital d'enfants Albert-Royer, Sénégal
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Nourallah H, Issa H, Al-Salem AH. The role of ERCP in the evaluation, diagnosis, and therapy of biliary and pancreatic diseases in children. Ann Saudi Med 1999; 19:163-6. [PMID: 17337963 DOI: 10.5144/0256-4947.1999.163] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Affiliation(s)
- H Nourallah
- Departments of Medicine and Surgery, Qatif Central Hospital, Qatif, Saudi Arabia
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Tarnasky PR, Tagge EP, Hebra A, Othersen B, Adams DB, Cunningham JT, Cotton PB, Hawes RH. Minimally invasive therapy for choledocholithiasis in children. Gastrointest Endosc 1998; 47:189-92. [PMID: 9512289 DOI: 10.1016/s0016-5107(98)70357-8] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Affiliation(s)
- P R Tarnasky
- Digestive Disease Center, Medical University of South Carolina, Charleston, USA
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26
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Al-Salem AH, Nourallah H. Sequential endoscopic/laparoscopic management of cholelithiasis and choledocholithiasis in children who have sickle cell disease. J Pediatr Surg 1997; 32:1432-5. [PMID: 9349762 DOI: 10.1016/s0022-3468(97)90555-8] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
BACKGROUND/PURPOSE Cholelithiasis and choledocholithiasis are common complications of sickle cell disease (SCD). With the recent advances in laparoscopic cholecystectomy (LC), which has been used successfully for the management of cholelithiasis in children who have SCD, exclusion of choledocholithiasis before LC is of great importance. METHODS Eighteen children who had SCD, cholelithiasis, and choledocholithiasis were treated at our hospital. Seven were treated with open cholecystectomy (OC) and common bile duct (CBD) exploration, and two were treated with transduodenal sphincteroplasty. The remaining 11 patients underwent endoscopic retrograde cholangiopancreatography (ERCP), sphincterotomy, and stone extraction followed by laparoscopic cholecystectomy (LC). RESULTS A dilated CBD noted on ultrasound, elevated alkaline phosphatase, elevated total bilirubin of more than 5 mg/dL, history of pancreatitis, either singly or in combination, should raise suspicion of choledocholithiasis, and these patients together with those who have choledocholithiasis detected on ultrasound should undergo ERCP to confirm and extract the stones before LC. CONCLUSION This sequential approach of endoscopic sphincterotomy and stone extraction followed by LC is a safe and effective approach for the management of cholelithiasis and choledocholithiasis in children who have SCD.
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Affiliation(s)
- A H Al-Salem
- Department of Surgery, Qatif Central Hospital, Saudi Arabia
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Newman KD, Powell DM, Holcomb GW. The management of choledocholithiasis in children in the era of laparoscopic cholecystectomy. J Pediatr Surg 1997; 32:1116-9. [PMID: 9247246 DOI: 10.1016/s0022-3468(97)90411-5] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Although laparoscopic cholecystectomy has become the procedure of choice for gallbladder removal in children, the treatment of children who have choledocholithiasis remains unclear. For adults who have suspected choledocholithiasis, preoperative endoscopic retrograde cholangiopancreatography (ERCP) is a well-described and effective approach, however, its use for common bile duct stones in children has not been defined. The authors reviewed the records of 131 consecutive children undergoing laparoscopic cholecystectomy on two surgical services to define the efficacy of ERCP followed by laparoscopic cholecystectomy in managing choledocholithiasis in children. Fourteen children were suspected of having common duct stones noted on preoperative ultrasound scan and laboratory data. At ERCP, six children had no stones visualized; eight had stones and underwent stone extraction and sphincter dilation or sphincterotomy. All 14 underwent laparoscopic cholecystectomy a mean of 3.8 days after ERCP. None of the 14 had evidence of retained stones. Only one of 117 children undergoing primary laparoscopic cholecystectomy had unsuspected common bile duct stones and was treated with laparoscopic common bile duct exploration and stone removal. A management plan incorporating ERCP followed by early laparoscopic cholecystectomy is a safe and effective strategy for children who have choledocholithiasis.
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Affiliation(s)
- K D Newman
- Department of Surgery, George Washington University School of Medicine and Children's Hospital, Washington, DC 20010, USA
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Gamba PG, Zancan L, Midrio P, Muraca M, Vilei MT, Talenti E, Guglielmi M. Is there a place for medical treatment in children with gallstones? J Pediatr Surg 1997; 32:476-8. [PMID: 9094022 DOI: 10.1016/s0022-3468(97)90610-2] [Citation(s) in RCA: 20] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
BACKGROUND Medical treatment of gallstones with ursodeoxycholic acid (UDCA) or chenodeoxycholic acid (CDCA) has not been evaluated in children. AIM The purpose of this study was to assess the effectiveness of UDCA in the treatment of gallstones in children. METHODS UDCA was used to treat 15 patients, (7 boys and 8 girls; mean age, 7.8 years; range, 3 months to 15 years) for 1 year. All had radiolucent stones with a maximum diameter of 10 mm and a normally contractile gallbladder. RESULTS The stones disappeared completely in two children but returned later. All symptomatic patients became symptom free. CONCLUSION UDCA is ineffective in the treatment of gallstones in children except in terms of relieving symptoms while on treatment.
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Affiliation(s)
- P G Gamba
- Department of Pediatric Surgery, University of Padova, Italy
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Tagge EP, Tarnasky PR, Chandler J, Tagge DU, Smith C, Hebra A, Hawes RH, Cotton PB, Othersen HB. Multidisciplinary approach to the treatment of pediatric pancreaticobiliary disorders. J Pediatr Surg 1997; 32:158-64; discussion 164-5. [PMID: 9044114 DOI: 10.1016/s0022-3468(97)90171-8] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
A multidisciplinary approach using traditional open surgery, endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic surgery has revolutionized the care of the adult with pancreaticobiliary disease. This study focuses on a similar collaborative effort to diagnose and treat children with pancreaticobiliary disorders. Charts of all patients treated on the pediatric surgery service between June 1990 and May 1995, who also underwent ERCP, were abstracted for disease process, presenting symptoms, laboratory evaluation, surgical or endoscopic procedures, and eventual outcome. Twenty-six children were identified, ranging from 6 months to 19 years of age. Pancreaticobiliary disorders included pancreas divisum (n = 1), choledochal cyst (n = 4), pancreaticobiliary trauma (n = 4), cholelithiasis and choledocholithiasis (n = 17). The pancreaticobiliary tree was successfully visualized by ERCP in 25 of 26 (96%) patients. Fifteen of these patients also underwent attempted therapeutic endoscopic procedures, with 13 (87%) performed successfully. Three patients with choledochal cyst had stents placed preoperatively for cholangitis, all of whom have undergone successful choledochal cyst excision. Two trauma patients underwent attempted stenting of a bile leak and bile duct stricture, respectively, both of which were unsuccessful, necessitating surgical correction. Seventeen patients with cholelithiasis underwent ERCP to rule out choledocholithiasis. Ten patients were found to have common duct stones, and all stones were endoscopically extracted, including those in a 6-month-old child. Overall survival rate was 96% (25 of 26), with the one death occurring in a trauma patient unrelated to his pancreaticobiliary disorder. A multidisciplinary approach using traditional open surgery, ERCP and laparoscopic surgery can successfully treat even young children with pancreaticobiliary disorders. In experienced hands, diagnostic ERCP and therapeutic endoscopic intervention can be performed successfully in most pediatric patients, greatly simplifying the surgical management of these potentially complex problems.
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Affiliation(s)
- E P Tagge
- Department of Surgery, Medical University of South Carolina, Charleston 29425, USA
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Al-Salem AH, Qaisaruddin S, Al-Abkari H, Nourallah H, Yassin YM, Varma KK. Laparoscopic versus open cholecystectomy in children. Pediatr Surg Int 1997; 12:587-90. [PMID: 9354731 DOI: 10.1007/bf01371905] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Twenty-one consecutive laparoscopic cholecystectomies (LC) were compared with 29 consecutive open cholecystectomies (OC). Sickle-cell disease (SCD) was the most common reason for cholecystectomy in both groups. The average length of operative time for LC was significantly longer than that of OC (P=0.0149). In 1 patient there was conversion from LC to OC due to severe adhesions. Common bile duct (CBD) stones were diagnosed in 8 (27.6%) of the OC group; in 4 of them the diagnosis was made preoperatively by ultrasound, in 4 by intraoperative cholangiogram. All 8 patients required CBD exploration, and 2 had additional transduodenal sphincteroplasties. In the LC group 5 patients (23.8%) had CBD stones. All had (ERCP) endoscopic retrograde cholangiopancreatography sphincterotomy, and stone extraction followed by LC. ERCP is a necessary adjunct to treatment if LC is to be contemplated. Six patients in the OC group developed complications, while only 4 patients in the LC group developed minor complications. The length of hospitalization after LC was significantly shorter than after OC (P=0.0150). LC is the procedure of choice in the management of cholelithiasis in children, especially those with SCD.
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Affiliation(s)
- A H Al-Salem
- Division of Pediatric Surgery, Qatif Central Hospital, Qatif, Saudi Arabia
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Winter SS, Kinney TR, Ware RE. Gallbladder sludge in children with sickle cell disease. The journal The Journal of Pediatrics 1994. [DOI: 10.1016/s0022-3476(06)80174-5] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/10/2023]
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Abstract
Sickle hemoglobinopathies include sickle cell disease, sickle-C disease, and sickle-beta thalassemia. Patients with these disorders commonly suffer a multitude of destructive events to vital organs, especially to the central nervous system, the spleen, the kidney, the lung, and the heart as a result of microvascular plugging by the sickled erythrocytes. Thoughtful preparation for anesthesia and operation, especially when directed by experienced individuals, can greatly reduce the hazard of inducing the sickle crises that formerly plagued individuals with sickle hemoglobinopathies who faced major operations under general anesthesia. The patient must be free of any acute illness, especially one involving the respiratory system. Adequate hydration preoperatively combined with avoiding perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will go far toward avoiding sickle-induced complications. Modern transfusion therapy, consisting of multiple small transfusions of Hb A erythrocytes administered over several weeks prior to the operation, not only corrects the chronic anemia but suppresses erythropoiesis of cells containing Hb S in the patient's bone marrow and leaves him or her with a majority of cells containing Hb A. This provides a safety net in case a sickle-inducing insult occurs despite the best efforts to avoid one. Individuals with sickle hemoglobinopathies may require any of the operations common to all children, for example, herniorrhaphy, appendectomy, tonsillectomy, and circumcision, but a significant number will develop calcium bilirubinate cholelithiasis and possibly cholecystitis as a result of the continual increased load of bile salts resulting from the shortened lifespan of the cells containing Hb S. Also, although most individuals with Hb S will gradually suffer splenic infarction by late childhood, a significant number of infants will experience acute splenic sequestration crisis, a life-threatening entity, the recurrence of which is prevented by splenectomy. Several publications have demonstrated that such surgical procedures can be performed in large numbers of patients with sickle hemoglobinopathies without deaths and with minimal morbidity.
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Affiliation(s)
- R E Ware
- Department of Pediatrics, Duke University Medical Center, Durham, North Carolina
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