Caroli's syndrome with autosomal recessive polycystic kidney disease.

Caroli's syndrome (CS) is a rare congenital disorder characterized by multiple segmental cystic or saccular dilatations of the intrahepatic bile ducts and congenital hepatic fibrosis. We report a 9-year-old boy who was diagnosed with CS and autosomal recessive poly-cystic kidney disease. On screening, his 5-month-old asymptomatic sister had multiple dilated biliary radicals with multiple bilateral renal cystic lesions. Both the patient and the affected sibling have been advised regular follow-up for monitoring the progression of the disease. In conclusion, patients with CS should be screened for renal cystic lesions and vice versa even if they are asymptomatic. Also, as the disease is inherited in an autosomal recessive manner, it is important to screen family members for early diagnosis and management.

Perforation of the hard palate due to tuberculosis.

Tuberculosis (TB) of the hard palate is rare and usually secondary to pulmonary TB. We present a case of a 7-year-old boy who presented with difficulty in swallowing solid foods, low-grade fever and loss of weight. Oral cavity examination showed perforation of the hard palate with an irregular, undermined margin and a necrotic base. Chest X-ray showed homogeneous opacity in the right upper zone. Fine-needle aspiration of the cervical lymph nodes showed granular caseous necrosis and granuloma composed of epitheliod cells and histiocytes. In view of the clinical presentation and evidence of pulmonary and lymph node TB, the palatal perforation was most likely due to TB. Patient was started on antituberculous therapy and is on regular follow-up.