2007_07_01_archive

Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) with

lymphangiectasia

Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome).

With lymphangiectasia as a complication

Diagn Pathol. 2007 Jul

Vankalakunti M, Gupta K, Kakkar N, Das A.

ABSTRACT

BACKGROUND: Renal-Hepatic-Pancreatic dysplasia syndrome described by

Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal

dysplasia and hepatic dysgenesis.

CASE PRESENTATION: We describe two unrelated cases of

Renal-hepatic-pancreatic dysplasia syndrome in stillborn babies. The

characteristic microscopic features were present in both the cases.

The second case illustrates the unique association lymphangiectasia

with Renal-hepatic-pancreatic dysplasia syndrome. Both cases are

unrelated and there is no history of any consanguineous marriage.

CONCLUSION: These two cases are unrelated and are rare. In the

developmental research, the perinatal autopsy needs to be utilized as

a major tool and an Ad hoc committee formation is required to

formulate the approach towards syndromic diseases.

Diagnostic Pathology