Journal of Postgraduate Medicine
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Year : 1982  |  Volume : 28  |  Issue : 2  |  Page : 109-11  

Multiple cysts in the liver and infantile polycystic disease of kidneys (a case report).

RH Udani, AG Desai, AC Joshi, UL Wagholikar 
 

Correspondence Address:
R H Udani





How to cite this article:
Udani R H, Desai A G, Joshi A C, Wagholikar U L. Multiple cysts in the liver and infantile polycystic disease of kidneys (a case report). J Postgrad Med 1982;28:109-11


How to cite this URL:
Udani R H, Desai A G, Joshi A C, Wagholikar U L. Multiple cysts in the liver and infantile polycystic disease of kidneys (a case report). J Postgrad Med [serial online] 1982 [cited 2022 Dec 7 ];28:109-11
Available from: https://www.jpgmonline.com/text.asp?1982/28/2/109/5583


Full Text



 INTRODUCTION



Multiple congenital hepatic cysts are uncommon in children but sometimes are found associated with congenital cystic disease of kidneys and other organs. Here we report such a case.

 CASE REPORT



A 21 year old male child, 3rd of the four living children of non-consanguinous healthy parents was admitted for breathlessness, fever, cough and loss of appetite for 3 days. He had repeated attacks of upper respiratory tract infections since 6 months of age and was treated adequately everytime. This child had an attack of measles 30 days prior to the present illness. He was a full term, normally born child with normal physical and mental mile stones. He had received primary immunisations.

On admission, he was conscious, irritable. anemic, malnourished (height of the 10th percentile and weight below the 3rd percentile of Harward), dehydrated and had signs of congestive heart failure with massive hepatomegaly of 8 cm below the costal margins, consistency being firm, with sharp border and smooth surfaces and splenomegaly of 1.5 cm. The results of the initial investigations showed haemoglobin to be 8 gm%; W. B. C. 9,700/cu. mm. with P50 L48 E2 and E.S.R. of 52 mm at the end of one hour. Blood urea was 43 mg%, and the liver function tests were normal. X-ray chest showed evidence of vascular congestion consistent with congestive cardiomegaly but E.C.G. could not be done. He was diagnosed as protein energy malnutrition of grade III with anemia and bronchopneumonia with congestive cardiac failure. The possibilities like cirrhosis of liver, disseminated tuberculosis and hepatoma were kept in mind. The child was given decongestive line of treatment with antibiotics, sodium bicarbonate and I/v maintenance fluids, but he went into peripheral circulatory failure, despite blood pressure of 100/70 mm Hg and died after 26 hours of admission.

Post mortem examination revealed enlarged, firm liver [Fig. 1 ]. On cut sections, there were 8-10 cysts, 2-7 cm in diameter, filled with clean, light yellow coloured fluid and lined by a smooth, tough, white wall. The rest of the liver appeared normal without any evidence of extra-hepatic biliary obstruction. Histology revealed multiple cysts of various sizes [Fig. 3], The biggest cyst was lined by dense fibrocollagenous tissue. The smaller cysts revealed a single layer of columnar lining suggestive of being biliary in origin. Liver parenchyma and sinusoids were unremarkable.

Kidneys were enlarged and nodular with X multiple cysts of varying sizes from a dot to pin head in the cortex and medulla containing clear light fluid without any evidence of lower urinary tract infection [Fig. 2] . Histology revealed multiple cysts in the cortex and medulla, lined by a single layer of flattened epithelium with mature mesenchymal tissue in between some of the tubules with a few foci of pyelonephritis [Fig. 4].

There was left ventricular hypertrophy. Lungs, pancreas, spleen and brain were normal.

 DISCUSSION



About 30-50% of patients of polycystic disease of liver have polycystic kidney[12], [13] and some have cysts in the pancreas, spleen, ovary and lungs.[10], [13] In Rall and Odel's series,[13] cardiac hypertrophy was noted in 52.3% cases.

Congenital polycystic disease of liver is rarely diagnosed clinically because patients are either asymptomatic or sometimes have vague symptomatology like abdominal distention, or mass in the abdomen or pain in the abdomen; however, the possibility of polycystic disease should be considered in a patient with an enlarged liver with little or no impairment of hepatic functions.[9] Diagnosis is usually made accidentally on laparotomy and when suspected, radio-isotope scanning, coeliac axis arteriography, liver biopsy and ultrasonogram may help to establish the diagnosis.

Incidence of liver cyst varies from 14 to 53 per 10,000 abdominal explorations[4], [5], [14] Although symptomatic liver cysts have been reported in all age groups from full-term fetus to the elderly, the majority of cases occur in the middle years of life.

Hepatic cysts are classified in various ways by various authors.[7], [8], [14]

An interesting feature observed in our case was "infantile polycystic kidneys" without any symptomatology related to obvious kidney disease. Infantile polycystic kidneys occur twice as often in females as in males and this has been considered to have a single autosomal recessive mode of transmission. Blythe and Ockenden[1] have classified this entity into four subgroups; perinatal, neonatal, infantile and juvenile according to the age of clinical presentation. Lieberman et al11 studied 14 children extensively and have reported that the prognosis in the surgical group was good. The use of respiratory aids and prompt treatment of pulmonary infection helps to improve the prognosis in infancy. Several of these 14 children studied by Lieberman et al11 have lived for many years; the oldest child was 181/2 years.

Treatment in this condition remains purely symptomatic as with any other chronic hepatic and/or renal disease.[3] Aspiration of large cysts as advocated by Jones et al[9] is a controversial method of treatment. Symptomatic or large cysts should be treated surgically.[2], [6], [16] The preferred operation is dissection of the cyst. If this is not feasible, then simple drainage or marsupialisation often causes regression of the cyst.[15]

 ACKNOWLEDGEMENTS



We wish to thank Dr. D. K. Dastur, Professor of Neuropathology, J.J. Hospital, Bombay, for getting microphotographs.

References

1Blythe, H. M. and Ockenden, B. G.: Polycystic disease of kidneys and liver presenting in childhood. J. Med. Genet., 8: 257-261, 1971.
2Clerk, D. D., Marks, C., Bernhard, V. M. and Bunkfeldt, F. M.: Solitary hepatic cysts. Surgery, 61: 687-693, 1967.
3Coffey, R. J. and Fitzmaurice, M. T. A.: Non-parasitic hepatocystic disease; A report of 9 cases. Amer. Surg., 25: 301-305, 1959.
4Eliason, E. L. and Smith, D. C.: Solitary non-parastic cysts of the liver; case report. Clinics, 3: 607-621, 1944.
5Feldman, M.: Polycystic disease of the liver. Amer. J. Gastro-enterol., 29: 83-86, 1958.
6Grime, R. T., Moore, T., Nicholson, A. and Whitehead, R.: Cystic hematomas and polycystic diseases of the liver. Brit. J. Surg., 47: 307-313, 1959.
7Henson, S. W. Jr., Gray, H. K. and Dockerty, M. B.: Benign turmours of the liver. Solitary cysts. Surg. Gynaec. & Obstet., 103: 607-612, 1956.
8Jone: Quoted by Nelson, W. E., Vaughan. V. C. and McKay, R. J.: "Text Book of Paediatrics". 9th Edition, W. B. Saunders & Co., Philadelphia, 1969, p. 850..
9Jones, W. L., Mountain, J. C. and Warren, K. W.: Symptomatic nonparasitic cysts of liver. Brit. J. Surg., 61: 118-123, 1974.
10Kissane, J. M. and Smith, M. G.: "Pathology of Infancy and Childhood". The C. V. Mosby Company, Saint Louis, 1967, p. 530-536.
11Lieberman, E., Salinas-Madrigal, L., Gwinn, J. L., Brennan, L. P., Fine, R. N. and Landing, B., H.: Infantile polycystic disease of kidney and liver. Clinical, pathological and radiological correlations and comparison with congenital hepatic fibrosis. Medicine, 50: 277-318, 1971.
12Oppenheimer, G. D.: Polycystic disease of kidney. Ann. Surg., 100: 1136-1158, 1984.
13Rail, J. E. and Odel, H. M.: Congenital polycystic disease of the kidney. Review of literature and data on 207 cases. Amer. J. Med. Sci., 218: 399-407, 1949.
14Sanfelippo, P. M., Bearhs, O. H. and Weiland, L. H.: Cystic disease of liver Ann. Surg., 179: 922-925, 1974.
15Vaughan, V. C., McKay, R. J. and Nelson, W. E.: "Nelsons Text Book of Paediatrics". 10th Edition, W. B. Saunders & Company, Philadelphia, 1975, p. 899.
16Warren, K. W. and Polk, R. C.: Benign cysts of the liver and biliary tract. Surg. Clin. North Amer., 38: 707-728, 1958.

 
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