CONGENITAL TUBERCULOSIS OF THE EAR AND PAROTID GLAND

Abstract

The number of cases of tuberculosis in women of childbearing age is increasing. The risk of tuberculosis in pregnancy leads to increased risk of congenital tuberculosis. Congenital transmission of tuberculous infection from mother to infant is very rare; fewer than 300 cases have been reported in the world literature and only 29 cases have been reported since the last major review in 1980.1, 2 Here we present an interesting case of congenital tuberculosis with ear and parotid gland involvement and a cystic lesion in the liver. Case report. A 2630-g girl was born vaginally at 34 weeks of gestation. When she was 60 days old she was hospitalized because of right ear discharge and nontender swelling of the right parotid region for 20 days. The mother, a 28-year-old woman, had complaints of fever and chest pain which lasted for 2 weeks at 28 weeks of gestation. The two siblings had no medical problems. Physical examination revealed purulent discharge through a perforation of the right eardrum. There was a hard, approximately 3- to 4-cm nontender swelling in the right parotid gland. She had three enlarged cervical lymph nodes 2 by 2 cm each in the right upper cervical region. There was no hepatic and splenic enlargement or respiratory distress. Ultrasound scan of the right parotid gland demonstrated enlargement of the gland with multiple hypoechoic and anechoic images; by abdominal ultrasound a hypoechoic area 7.5 mm in diameter was seen within the upper right lobe of the liver to the posterolateral diaphragmatic surface. Because there was no improvement after 10 days of antibiotic treatment, a lymph node biopsy was done. Histologic examination demonstrated multiple granulomas, most associated with multinucleated giant cells (Langhans' type), caseous necrosis and epithelioid histiocytes. Stains for acid-fast bacilli with Ziehl-Neelsen staining were positive from the lymph node biopsy and from ear discharge materials. Bacillus of Calmette-Guérin had not been given at birth and a tuberculin skin test (5 tuberculin units) was negative. Chest roentgenogram was normal, sedimentation rate was 70 mm/h and a test for HIV antibody was negative. Mycobacterium tuberculosis hominis was isolated from the lymph node suppurative material which was susceptible in vitro to isoniazid, streptomycin, pyrazinamide, rifampin and ethambutol. PCR testing of the suppurative material of the lymph node was positive for M. tuberculosis. A chest roentgenogram of the mother revealed right pleural effusion and right paratracheal lymphadenopathy. Her tuberculin skin test reaction was 20 mm. Endometrial biopsy showed caseous granulomatous inflammation and Langhans' giant cells. The patient was treated with isoniazid, rifampin and pyrazinamide. After 15, 22 and 30 days of the treatment, parotid enlargement, ear discharge and cervical enlargement, respectively, disappeared. The hypoechoic area in the liver disappeared by the 45th day of the treatment. Pyrazinamide was discontinued at the end of the second month; the other drugs were given for 9 months. The patient is being followed up regularly for 15 months and remains healthy. Discussion.M. tuberculosis can be transmitted to the fetus by hematogenous spread from the infected placenta through the umbilical vein or by aspiration or ingestion of amniotic fluid contaminated by placental or genital infection.3 Criteria for making the diagnosis were proposed in 1935 by Beitzke.4 These criteria have become difficult to apply in current practice; therefore in 1995 Cantwell et al.1 proposed revised diagnostic criteria for congenital tuberculosis, by which tuberculous infection of placenta or maternal tract must have been demonstrated. In our patient's mother endometrial tuberculosis was confirmed by biopsy. The most common signs of congenital tuberculosis are respiratory distress, fever, hepatomegaly and splenomegaly.1-3 Congenital tuberculosis with otitis has been reported before.1-3, 4 Our case is similar to two cases of Naranbhal et al.5 Ear discharge without respiratory distress, fever and hepatosplenomegaly was the first clinical sign in their cases. The ear is an unusual site for a primary infection. It may be speculated that transmission occurred when infected amniotic fluid lodged in the fetal auditory canal or via the Eustachian tube either in utero or during birth.5 Tuberculosis of the parotid gland is a rare disease especially in childhood and has not been previously reported in congenital tuberculosis. Two pathologic types occur: a more common focal form resulting from lymph node infection; and a rarer diffuse form resulting from parenchymatous involvement.6 In our case according to the physical examination and ultrasound scan, it was thought to be the focal form. We did not perform incisional biopsy because of the risk of fistula formation with resulting prolonged drainage.7 If the suspicion of tuberculosis is high, a therapeutic trial of antituberculosis chemotherapy may be diagnostic.8 In our case infection in the parotid gland resolved completely with treatment. In our patient abdominal ultrasound showed a hypoechoic area in the liver. This might have been a coincidental simple liver cyst or a manifestation of tuberculosis. Tuberculosis can affect the liver in two ways: as a diffuse involvement in association with miliary or pulmonary tuberculosis; or as a local process in which the hepatic involvement is the principal manifestation of the disease.9 The demonstration of a primary hepatic complex in the Beitzke criteria requires an open surgical procedure or autopsy to confirm liver and regional lymph node involvement.4 We could not perform open surgical or ultrasound-guided biopsy because of the subdiaphragmatic location. The cystic mass disappeared after 2 months of therapy. Similar hepatic tuberculous lesions have been reported.10 The disappearance of the cystic mass with antituberculosis therapy was taken as evidence of a tuberculosis-related lesion. Nesrin Şenbil, M.D. Figen Şahin, M.D. M. Kazim Çağlar, M.D. Ayşegül Oksal, M.D. Filiz Yilmaz, M.D. Fevzi Tuna, M.D. Departments of Pediatrics (NS, FS, MKC, FT), Pathology (AO) and Radiology (FY); Dr. Sami Ulus Children's Hospital; Ankara, Turkey