BRIEF REPORTS

Intrahepatic Biliary Dilatation We present a 10 year old boy who was diag- nosed to have Caroli's disease with with Aortoarteritis aortoarteritis involving abdominal aorta and both renal arteries. Case Report A ten year old Muslim boy presented S.K. Kabra with history of not growing well for 4-5 N. Soni years, recurrent high grade fever with chills A. Talati and rigors associated with pain abdomen for S. Patel 3 years and generalized convulsions off and R. Mehta on for one month. Up to the age of 5 years R.Shah he was completely alright. He received vari- R.R. Modi ous hematinics and multivitamin tonics from general practitioners for not growing well. His mother and father were first de- gree cousins. His two elder sibs died at age Intrahepatic biliary dilatation or Caroli's of 6 and 8 years due to some seizure disor- disease is a rare clinical entity of unknown der and the details of illness are not known. etiology. It consists of dilatation of The other two sibs were completely normal intrahepatic biliary ducts which communi- at age of 6 years and 4 years. His develop- cate with normal common duct system. mental mile-stones were normal. It may manifest at any age but usual age of Physical examination revealed a poorly presentation is late childhood or early adult built but well nourished boy with normal life. The clinical features of disease in- facial features. His weight was 25 kg and cludes recurrent abdominal pain associated height was 112 cm. His pulse rate was 110 with fever, hepatomegaly and mild per minute regular, good volume in all the secondary to recurrent cholingitis(l). It may extremities. He was hypertensive with a be associated with disorders such as blood pressure of 220/150 mm of Hg in left congenital hepatic fibrosis(2) and hepatic upper limb. BP in right upper limb was 224/ carcinoma(3). Occasionally it may be asso- 150 mm of Hg while BP in both lower limbs ciated with renal tubular ectasia and renal was 200/140 mm of Hg. Per abdominal cysts(4). To our knowledge it's association examination showed a hepatomegaly of 6 with aorto arteritis has not been reported. cm below right costal margin in From the Departments of Pediatrics, Gastro- midclavicular line with firm consistency intestinal Surgery and Radio Diagnosis, V.S. and smooth surface with a liver span of 16 General Hospital, Ahmedabad 380 006, cm. Spleen was one cm below costal mar- Gujarat. gin. There was no clinically evident jaun- Reprint requests: Dr. S.K. Kabra, Gastrointesti- dice or lesions. Rest of the systemic nal Surgery and Radio Diagnosis, V.S. examination was within normal limits. General Hospital, Ahmedabad 380 006, Gujarat. Investigations revealed a hemoglobin of Received for publication: October 14, 1991; 9 g/dl with normal total and differential Accepted: May 30, 1994 leucocytic count. Peripheral smear showed

92 INDIAN PEDIATRICS VOLUME 32-JANUARY 1995 microcytic hypochronic anemia. Sedimenta- performed under general anesthesia and tion rate was 18 mm at end of first hour. revealed dilated intrahepatic bile ducts Blood urea and serum creatinine were 190 which were communicating with a normal mg/dl and 3.6 mg/dl, respectively with a common bile" duct (Fig. 2). Head CT and glomerular filtration rate of 18 ml/minute/ X-ray film of chest were normal. X-ray 1.73 meter2. Liver function test including S. films of various long and short bilirubin, SGPT, serum alkaline phos- revealed widening and irregularity in phatase were normal. Serum VDRL test was metaphysis. Other parts of skeleton inclu- negative. Routine urine examination and ding vertebrae and skull were normal. urinary catecholamines were within normal He was diagnosed to be suffering from limits. Among radiological investigations, Caroli's disease with aortoarteritis, chronic ultrasound abdomen revealed bilateral con- renal failure and started on tracted kidneys with smooth outline, antihypertensives in form of hydraiazine hepatomegaly with dilated intrahepatic and nifedipine with supportive care for biliary radicles with normal common bile chronic renal failure. During follow up over duct. The same findings were confirmed by a period of 1.5 years he required frequent abdominal CT scan. Aortogram showed very hospitalizations for fever and pain abdomen small renal arteries and nephrogram was not and occasionally for convulsions. Along seen even in delayed films indicating very poorly functioning bilateral kidneys(Fig. 1). Endoscopic retrograde cholangiography was

BRIEF REPORTS with antihypertensives and antibiotics he clear. The suggested causes include infec- required blood transfusion thrice. His radial tive, tubercular, toxic, immunologic, pulse in left upper limb showed decrease in etc.(7,8). The course of illness depends on volume over a period of 1 year. His renal individual vessel involvement and the functions deteriorated progressively while severity of illness. In our patient there was liver functions remained, within normal marked narrowing of renal vessels at the limits. At age of 11.5 years he developed time when he presented to us. He expired intractable seizures following stoppage of due to end stage renal disease secondary to antihypertensive for 2 days and expired severe ischemia. The treatment for before he could be transferred to hospital. aortoarteritis is not well defined and includes antinflammatory agents, anti- Discussion tubercular drugs and surgical intervention. Caroli's disease is diagnosed by demon- Explanation for occurrence of two rare stration of dilated intrahepatic ducts with but well defined clinical conditions of normal CBD. Ultrasonography and CT scan unknown etiology in our patient is difficult. may demonstrate dilated intrahepatic ducts An autosomal dominantly inherited syn- but should be confirmed by ERCP(5). Pres- drome involving intrahepatic biliary ducts ence of abscess or stones which are compli- with vessels and bones has been reported by cation of the disease may be seen in Alagille et al.(9). This syndrome consist of ultrasonography or CT scan. In our patient intrahepatic with vascular there were dilated intrahepatic channels involvement in form of pulmonary stenosis with normal bile ducts. No abscess or stone peculiar facies and vertebral anomalies. could be demonstrated. The course of This usually manifests in early infancy as Caroli's disease is variable. The patient may cholestasis. Though the anatomic structures survive for long. The cause of death most of involved in this syndrome and our patients the times is hepatic abscess or Gram nega- are similar, i.e., intrahepatic biliary ducts, tive septicemia; in few cases hepatic failure blood vessels, and bones but the clinical may occur in advanced stage of disease. Our manifestations are entirely different. In patient responded very well to antibiotics view of consanguinity and deaths of two for each episode of cholingitis. His liver sibs the combined disease in our patient function tests were normal till the end. The may be regarded as variant of the syndrome treatment of Caroli's disease consists of described by Alagille et al. However, a hepatic lobectomy in localized illness, possibility of some toxic or immunological repeated laparotomies for drainage of factor involving all the structures together abscess or removal of stones(6). cannot be ruled out. The diagnosis of obstructive aorto- REFERENCES arteritis in our patient was based on demon- stration of narrowing of aorta and both renal 1. Murray Lyon IM, Shilkin KB, Laws JW, arteries in aortagram(7). In ultrasound both et al. Non obstructive dilatation of the intrahepatic biliary tree with cholangitis. the kidneys were contracted with smooth Q J Med 1972, 41: 477-484. outline suggesting chronic ischemia secon- dary to renal arterial obstruction. The 2. McGongle RJS, Mowat AP, Bewick M, etiology of obstructive aortitis is not very et al Congenital hepatic fibrosis and polycystic disease: Role of 94 INDIAN PEDIATRICS VOLUME 32-IANUARY 1995

portocaval shunting and transplantation in and Biliary System. Ed. Sherlock S, 8th 3 patients. QJ Med 1981, 50: 269-274. edn. Loncon, Blackwell Scientific Publi- cations, 1989, pp 639-654. 3. Dayton MT, Longmire WP Jr, Tompkin RK. Caroli's disease: A premalignant 7. Srivastava S, Srivastava RN, Tandon R. condition. Am J Surg 1983, 145: 41-43. Idiopathic obstructive aortoarteritis in children. Indian Pediatr 1986, 23: 403- 4. Davies CH, Stringer DA, Whyte H, et al. 410. Congenital hepatic fibrosis with saccular dilatation of intrahepatic and in- 8. Kinare SG, Iyer I, Murthy A, Dave RH, fantile polycystic kidneys. Pediatr Radiol Kelkar MD. Nonspecific aortoarteritis. 1986,16: 302-304. Indian J 1983, 35: 337-340.

5. Missavage AE, Sugawa C. Caroli's dis- 9. Algille D, Estrada A, Hadchauel M, ease: Role of endoscopic retrograde Gautier M, Odievre M, Dommergues JP. cholangiopancreatography. Am J Gastro- Syndromic paucity of interlobular bile enterol 1983, 78: 815-819. ducts (Alagille syndrome or arteriohepatic 6. Sherlock S. Cysts and congenital biliary displasia)-Review of 80 cases. J Pediatr abnormalities. In: Diseases of the Liver 1987, 110: 195-200.

Klippel Trenaunay Weber Though a few cases have been reported(2- Syndrome Associated with 4), but to the best of our knowledge, the association of an abdominal hamartoma Abdominal Hamartoma with with undescended testis and appendage in Undescended Testis this disorder has not been documented. Case Report A 2!/2-year-old boy born of non- consanguinous parents was admitted to our K. Biswas hospital in October, 1991 with history of A.K. Sarkar progressive enlargement of left lower limb, S. Sarkar buttocks, perineum; pink stains at different B. Mukhopadhyay sites of the left side of the body since birth S. Ganguly From the Departments of Pediatrics, Pediatric Surgery and Pathology, Institute of Post- graduate Medical Education and Research and S.S.K.M. Hospital, Calcutta. Klippel Trenaunay Weber Syndrome is Reprint requests: Dr. Amiya K. Sarkar, Flat a non-heritable disorder consisting of a A-l/7, Kalindi Housing Estate, Calcutta macular vascular nevus (port-wine nevus) in 700 089. combination with bony and soft tissue hy- Received for publication: March 31, 1994; pertrophy and venous varicosities(l). Accepted: June 2, 1994

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