1060
Clinical notes
The Journal ofPediatrics December 1978
Intrapancreatic compression of the common bile duct in cystic fibrosis We report an infant with cystic fibrosis presenting at birth with meconium ileus who developed prolonged cholestatic jaundice from compression of the common bile duct and the pancreatic duct by advanced pancreatic fibrosis. Extrahepatic biliary obstruction of this nature has not been previously described in cystic fibrosis.
- C8 D
CASE REPORT Patient P.R., a male infant of a term gestation, presented with meconium ileus following birth. Cystic fibrosis Was diagnosed subsequently. At 3 months of age, a laparotomy was performed because of progressive jaundice (bilirubin direct [6.2 and indirect 3.8 mg/ dl), An intraoperative cholangiogram showed only filling of the gallbladder. An open liver biopsy revealed marked cholestasis with bile plugging, reduction in the number of bile ducts in portal triads, and giant cell transformation. At this time, the diagnosis of intrahepatic and extrahepatic biliary atresia was entertained. At 5 months of age, tbe infant died of overwhelming sepsis. Prior to death, the direct-reacting bilirubin concentration Was 21.6 mg/dl and indirect, 6 rng/dl. At autopsy, from the level of the ampulla of Vater, serial sections revealed a common opening of the common bile duct and pancreatic duct (Figure, A). This common pancreatlcobiliary channel and the intrapancrcatic portion of the common bile duct (Figure B) were compressed by fibrotic pancreatic tissue, with resulting internal diameters of 1 and 1.5 rnrn, respectively, Proximally, the common bile duct was grossly dilated and filled with bile. Histology of the liver revealed proliferation of bile ductucles, bile retention, and portal fibrosis.
DISCUSSION The histopathology of the hepatobiliary system in newborn infants with cystic fibrosis has recently been reviewed.': The hepatic lesions are characterized by proliferation of bile ductules, with or without portal fibrosis and periportal inflammation. Examination of the extrahepatic biliary system has demonstrated mucoviscid material within the cystic duct, large bile ducts, and gallbladder, i , , and has been implicated as a cause of prolonged neonatal jaundice in cystic fibrosis.>" However, extraluminal obstruction of the common bile duct or extrahepatic biliary atresia has not been reported in cystic fibrosis. Therefore, clinically, the underlying hepatobiliary disorder may present as cholestasis, but it is uncommon for this mode of presentation to be rapidly progressive or fatal early in life. The unusual feature in this patient was the development of progressive cholestasis, which soon overshadowed the other clInical manifestations of cystic fibrosis. We suggest that if severe, progressive cholestasis develops in an infant with cystic fibrosis, compression of the intra pancreatic portion of the common bile duct mimicking extrahepatic biliary atresia should be considered, The authors express their appreciation to G, Gauthier and P.
0022-3476178/121060 + 02$00.20/0 © 1978 The C. V. Mosby Co.
Figure. A, SChematic diagram summarizing the autopsy findings of the extrahepatic biliary tree, Compression of the intrapancreatic portion of the common bile duct (CBD) and the common pancreaticobiliary channel by pancreatic fibrosis. B, Common bile duct (large arrow) showing intraluminal obstruction by PAS-positive mucoid material and compression by fibrosis (F). The common bile duct is surrounded by fibrotic pancreatic tissue (PAN) and bile duct proliferation (small arrows) (X 30), Nelson, Medical Illustrations Department, for preparation of the illustration, the Dominique Belcourt Liver Research Fund, and Mrs. L. Thewaldt for typing this manuscript. Benedetto B. Vitullo, M.D. Fellow of the Medical Research Council of Canada The Montreal Children's Hospital DIvision of Gastroenterology C-1220 Montreal. PQ, Canada 1J3HIP3 Louise Rochon. M.D. Thomas /I, Secmaycr, M.D. Harvey Beardmore. M.D. Ross C. de Belle, M.D,
REFERENCES I. Oppenheimer EH, and Esterly JR: Pathology of cystic fibrosis, Perspect Pediatr Pathol 2:241, 1975.
Clinical notes
Vol ume 93
1061
Number 6
2.
Oppenheimer EH, and Esterly JR: Hepatic changes in young infants with cysti c flbro sls : Possible relation to foc al bili ary cirrhosis, I Pediatr 86:683, 1975. 3. T alamo RC, and Hendren WH : Prolonged o bstructive jaundice. Am J D is Child 115 :74. 1968. 4. Jean R. Bonnet H, Emberger JM . Bose R , and Rieu D : Cholestatic icterus in in fants ind icat ive of m ucoviscidosis, Arch F Pediatr 27:849. 1970. 5, Valman HB. France N E. and W allis PG : Prol onged neonatal jaundice in cyst ic fibrosis, Arch Dis Child 46:806,
1971.
merited in a patient with hemoglobin SC disease..' the diagnosis appears unlikely in this case alth ough it Cannot be entire ly ruled Olll.
Ch oreiform activity in our patient may po ssibly have been due to th e beneficia l effects of h yper tra nsfusion on a n occult vascu lo occlusive lesion in the diencephalon. With improvemen t in cortical funct ion, the lesion would then be man ifest. Lon g-term follow-up of such pat ients will help determine whether o r not thes e events are causally rel ated . S. Kaiser Ali, M,B.• B,S.
Fellow In Hematology-Oncology St. Jude Children's Research Hospital 332 N. Lauderdale Memphis, TN 38101 .Judilh Wili mas, M.D. Research Associate St . Jude Children's Research Hospital Assistant Professo r oj Clinical Pediatrics University of Tenn essee Cent er for the H ealth Scien ces John Goff. PIT. D. Res earch Ass ociate in Neuropsychology SI. Ju de Children 's Research Hospit al
Choreiform activity in hemoglobin SC disease Neurologic complications in hem oglobin SC disea se include hemiplegia,' recurrent massive seizures, ver tigo. and blindness,' We wish to report a case ofchorea-Iike ac tivity in a pa tient with this diseu se , CASE REPORT Patie nt R. S.• a 7!1l-year-old black . boy, presented with a two-week his tory of weak ness in his right leg. His att ention spun had decreased and he appeared to have amn esia for recent events, Family history was negative for chorea. Physical examination revea led right hemiparesis with hyp erreflexia . A Grade 2/6 systolic now murmur was audible along the left sternal border, The spleen was palpated 4 ern below the costal margin. Hemoglobin level WllS 11.4 gm /dl. White blood cell and platelet counts were normal. Hemoglobin electrophoresis revealed a hemoglobin S level of 51%, hemoglobin C of 46%. and hemoglobin F of 2%. Ce rebral arteriogram was abnormal. He was starred on hypertransfuslon for his cerebrovascular accident. Evalu ation one year later showed a full scale IQ of 79 on the Wechsle r Intelligence Scale for Children. One a nd one-half years afte r the first admission, while on hypertransfusion, he developed a n acute onset of swa ying . unstable gait and sl urred speech. There was no prodroma of a respiratory tract infection. Choreiform movements co nsist ing of facial twitching. flicking of the tongue. a nd jerking of all extremities were noted. A right Bab inski sign was present. Cardiovascul ar exam ination was unc ha nged. T ile hemoglobi n va lue was 11.5 grn /dl, hemog lobi n S level was 13.6%, a nd hem oglobin C 18.5%. C hest radiograph. clcctrocardiograrn. sLrep lOzyme :lssa y. C -rcactivc protein. uri nalysis, levels of alkali n e phosphntase. SGOT and SGPT. a nd bra in sca n were n ormal. He was started o n da ily oral peni cilli n un,d continued oil hypertransfusion . Repeat evalu ation sho wed an increuse in his IQ 10 91 with ov era ll im pro vem en t of left hemisphere fu nctio n. Over the next four months, his chorea reso lved . DISCUSSION On set of chorea after 17 months of hypertransfusion for a cerebrovascular accident is unusual , since prophylactic transfusions have been shown to improve the clinical status of such p atients." While rheumatic fever has been previously docu-
00 22-3476/78/ 12 106 1+01$00.1010 (CJ 1978 The C. V. Mosby Co.
REFERENCES
I. Port noy EA, a nd Hcrion IC: Neurol og ical manifestatio ns in sickle cell disease. Ann Intern Med 76:643, 1972. Greer M , and Schotland D : Abnorma l hemoglobin as a ca use of neurologic disease. Neu rology 12: 114, 1962. 3. Lusher JM . Hnghighat H, and Khalifa AS: A prophylactic transfusion program for children with sickle cell anemia complicated by CNS infarc tio n. Am I Hernatol 1:265. 1976, 4. Lisker SA, et al: The coexi stence of acute rheumatic fever and sickle cell-hemoglobin C d isease, Circulation 31: 108. 2.
1965.
Neisseria lactamica meningitis Neisseria lactamica is a lactose-ferment ing strain of N eisseria whi ch has been reco vered fro m tracheal aspirates, tonsils, sputum. lung tissue , amnio tic fluid. vagina , a nd nasopharynx. " t It has frequen tly been misidenti fied as N. meningitldis or N, subflava a nd has been considered to be of no clinical sign ificance.' The isolation of th is organism from the cerebr osp inal fluid or blood in associa tion wit h clinical illness has previous ly been repo r ted in two ch ildren." We report a case of N. lactamica, men ingitis in a 6-month-old infan t. M iddle-ear colonization is hypothesized as the foc us of th is pa tient's meningitis. CASE REPORT This 6-month-old white girl was othe rwise well when she developed signs of conjunctivitis . About 24 hours later she dev eloped fever, irritability, and bilateral otitis media. The CSF
0022-3476178/ 121061 +02$00.2010
Clinical notes
The Journal ofPediatrics December 1978
Intrapancreatic compression of the common bile duct in cystic fibrosis We report an infant with cystic fibrosis presenting at birth with meconium ileus who developed prolonged cholestatic jaundice from compression of the common bile duct and the pancreatic duct by advanced pancreatic fibrosis. Extrahepatic biliary obstruction of this nature has not been previously described in cystic fibrosis.
- C8 D
CASE REPORT Patient P.R., a male infant of a term gestation, presented with meconium ileus following birth. Cystic fibrosis Was diagnosed subsequently. At 3 months of age, a laparotomy was performed because of progressive jaundice (bilirubin direct [6.2 and indirect 3.8 mg/ dl), An intraoperative cholangiogram showed only filling of the gallbladder. An open liver biopsy revealed marked cholestasis with bile plugging, reduction in the number of bile ducts in portal triads, and giant cell transformation. At this time, the diagnosis of intrahepatic and extrahepatic biliary atresia was entertained. At 5 months of age, tbe infant died of overwhelming sepsis. Prior to death, the direct-reacting bilirubin concentration Was 21.6 mg/dl and indirect, 6 rng/dl. At autopsy, from the level of the ampulla of Vater, serial sections revealed a common opening of the common bile duct and pancreatic duct (Figure, A). This common pancreatlcobiliary channel and the intrapancrcatic portion of the common bile duct (Figure B) were compressed by fibrotic pancreatic tissue, with resulting internal diameters of 1 and 1.5 rnrn, respectively, Proximally, the common bile duct was grossly dilated and filled with bile. Histology of the liver revealed proliferation of bile ductucles, bile retention, and portal fibrosis.
DISCUSSION The histopathology of the hepatobiliary system in newborn infants with cystic fibrosis has recently been reviewed.': The hepatic lesions are characterized by proliferation of bile ductules, with or without portal fibrosis and periportal inflammation. Examination of the extrahepatic biliary system has demonstrated mucoviscid material within the cystic duct, large bile ducts, and gallbladder, i , , and has been implicated as a cause of prolonged neonatal jaundice in cystic fibrosis.>" However, extraluminal obstruction of the common bile duct or extrahepatic biliary atresia has not been reported in cystic fibrosis. Therefore, clinically, the underlying hepatobiliary disorder may present as cholestasis, but it is uncommon for this mode of presentation to be rapidly progressive or fatal early in life. The unusual feature in this patient was the development of progressive cholestasis, which soon overshadowed the other clInical manifestations of cystic fibrosis. We suggest that if severe, progressive cholestasis develops in an infant with cystic fibrosis, compression of the intra pancreatic portion of the common bile duct mimicking extrahepatic biliary atresia should be considered, The authors express their appreciation to G, Gauthier and P.
0022-3476178/121060 + 02$00.20/0 © 1978 The C. V. Mosby Co.
Figure. A, SChematic diagram summarizing the autopsy findings of the extrahepatic biliary tree, Compression of the intrapancreatic portion of the common bile duct (CBD) and the common pancreaticobiliary channel by pancreatic fibrosis. B, Common bile duct (large arrow) showing intraluminal obstruction by PAS-positive mucoid material and compression by fibrosis (F). The common bile duct is surrounded by fibrotic pancreatic tissue (PAN) and bile duct proliferation (small arrows) (X 30), Nelson, Medical Illustrations Department, for preparation of the illustration, the Dominique Belcourt Liver Research Fund, and Mrs. L. Thewaldt for typing this manuscript. Benedetto B. Vitullo, M.D. Fellow of the Medical Research Council of Canada The Montreal Children's Hospital DIvision of Gastroenterology C-1220 Montreal. PQ, Canada 1J3HIP3 Louise Rochon. M.D. Thomas /I, Secmaycr, M.D. Harvey Beardmore. M.D. Ross C. de Belle, M.D,
REFERENCES I. Oppenheimer EH, and Esterly JR: Pathology of cystic fibrosis, Perspect Pediatr Pathol 2:241, 1975.
Clinical notes
Vol ume 93
1061
Number 6
2.
Oppenheimer EH, and Esterly JR: Hepatic changes in young infants with cysti c flbro sls : Possible relation to foc al bili ary cirrhosis, I Pediatr 86:683, 1975. 3. T alamo RC, and Hendren WH : Prolonged o bstructive jaundice. Am J D is Child 115 :74. 1968. 4. Jean R. Bonnet H, Emberger JM . Bose R , and Rieu D : Cholestatic icterus in in fants ind icat ive of m ucoviscidosis, Arch F Pediatr 27:849. 1970. 5, Valman HB. France N E. and W allis PG : Prol onged neonatal jaundice in cyst ic fibrosis, Arch Dis Child 46:806,
1971.
merited in a patient with hemoglobin SC disease..' the diagnosis appears unlikely in this case alth ough it Cannot be entire ly ruled Olll.
Ch oreiform activity in our patient may po ssibly have been due to th e beneficia l effects of h yper tra nsfusion on a n occult vascu lo occlusive lesion in the diencephalon. With improvemen t in cortical funct ion, the lesion would then be man ifest. Lon g-term follow-up of such pat ients will help determine whether o r not thes e events are causally rel ated . S. Kaiser Ali, M,B.• B,S.
Fellow In Hematology-Oncology St. Jude Children's Research Hospital 332 N. Lauderdale Memphis, TN 38101 .Judilh Wili mas, M.D. Research Associate St . Jude Children's Research Hospital Assistant Professo r oj Clinical Pediatrics University of Tenn essee Cent er for the H ealth Scien ces John Goff. PIT. D. Res earch Ass ociate in Neuropsychology SI. Ju de Children 's Research Hospit al
Choreiform activity in hemoglobin SC disease Neurologic complications in hem oglobin SC disea se include hemiplegia,' recurrent massive seizures, ver tigo. and blindness,' We wish to report a case ofchorea-Iike ac tivity in a pa tient with this diseu se , CASE REPORT Patie nt R. S.• a 7!1l-year-old black . boy, presented with a two-week his tory of weak ness in his right leg. His att ention spun had decreased and he appeared to have amn esia for recent events, Family history was negative for chorea. Physical examination revea led right hemiparesis with hyp erreflexia . A Grade 2/6 systolic now murmur was audible along the left sternal border, The spleen was palpated 4 ern below the costal margin. Hemoglobin level WllS 11.4 gm /dl. White blood cell and platelet counts were normal. Hemoglobin electrophoresis revealed a hemoglobin S level of 51%, hemoglobin C of 46%. and hemoglobin F of 2%. Ce rebral arteriogram was abnormal. He was starred on hypertransfuslon for his cerebrovascular accident. Evalu ation one year later showed a full scale IQ of 79 on the Wechsle r Intelligence Scale for Children. One a nd one-half years afte r the first admission, while on hypertransfusion, he developed a n acute onset of swa ying . unstable gait and sl urred speech. There was no prodroma of a respiratory tract infection. Choreiform movements co nsist ing of facial twitching. flicking of the tongue. a nd jerking of all extremities were noted. A right Bab inski sign was present. Cardiovascul ar exam ination was unc ha nged. T ile hemoglobi n va lue was 11.5 grn /dl, hemog lobi n S level was 13.6%, a nd hem oglobin C 18.5%. C hest radiograph. clcctrocardiograrn. sLrep lOzyme :lssa y. C -rcactivc protein. uri nalysis, levels of alkali n e phosphntase. SGOT and SGPT. a nd bra in sca n were n ormal. He was started o n da ily oral peni cilli n un,d continued oil hypertransfusion . Repeat evalu ation sho wed an increuse in his IQ 10 91 with ov era ll im pro vem en t of left hemisphere fu nctio n. Over the next four months, his chorea reso lved . DISCUSSION On set of chorea after 17 months of hypertransfusion for a cerebrovascular accident is unusual , since prophylactic transfusions have been shown to improve the clinical status of such p atients." While rheumatic fever has been previously docu-
00 22-3476/78/ 12 106 1+01$00.1010 (CJ 1978 The C. V. Mosby Co.
REFERENCES
I. Port noy EA, a nd Hcrion IC: Neurol og ical manifestatio ns in sickle cell disease. Ann Intern Med 76:643, 1972. Greer M , and Schotland D : Abnorma l hemoglobin as a ca use of neurologic disease. Neu rology 12: 114, 1962. 3. Lusher JM . Hnghighat H, and Khalifa AS: A prophylactic transfusion program for children with sickle cell anemia complicated by CNS infarc tio n. Am I Hernatol 1:265. 1976, 4. Lisker SA, et al: The coexi stence of acute rheumatic fever and sickle cell-hemoglobin C d isease, Circulation 31: 108. 2.
1965.
Neisseria lactamica meningitis Neisseria lactamica is a lactose-ferment ing strain of N eisseria whi ch has been reco vered fro m tracheal aspirates, tonsils, sputum. lung tissue , amnio tic fluid. vagina , a nd nasopharynx. " t It has frequen tly been misidenti fied as N. meningitldis or N, subflava a nd has been considered to be of no clinical sign ificance.' The isolation of th is organism from the cerebr osp inal fluid or blood in associa tion wit h clinical illness has previous ly been repo r ted in two ch ildren." We report a case of N. lactamica, men ingitis in a 6-month-old infan t. M iddle-ear colonization is hypothesized as the foc us of th is pa tient's meningitis. CASE REPORT This 6-month-old white girl was othe rwise well when she developed signs of conjunctivitis . About 24 hours later she dev eloped fever, irritability, and bilateral otitis media. The CSF
0022-3476178/ 121061 +02$00.2010
