Congenital tuberculosis with facial nerve palsy

Congenital tuberculosis is unusual, and central nervous system involvement is even more rare. We report a case of congenital tuberculosis presenting with facial palsy and purulent otorrhea. Congenital transmission of tuberculous infection from mother to infant is very rare. This is the first reported case of congenital tuberculosis in which facial nerve palsy was a prominent symptom. The patient also had ear, mastoid, pulmonary and central nervous system involvement. Case report. The patient’s mother was a 31-year-old, gravida 15 para 7 woman with a history of prostitution and cocaine and alcohol abuse. She had not received prenatal care, and her amniotic membrane ruptured at 32 weeks of gestation. Nine days later a 2085-g girl was delivered by cesarean section after a brief labor complicated by suspected maternal chorioamnionitis. The neonatal course was complicated by mild respiratory distress syndrome treated with oxygen supplementation. A radiograph of the chest was unremarkable. The patient received intravenous ampicillin sodium and cefotaxime sodium for 1 week for a clinical suspicion of sepsis, despite a negative blood culture. Her condition improved, and she was discharged to a foster home at 1 week of age. Physical contact between the biologic mother and the patient was limited to only a few minutes of being held immediately after birth. The patient presented to The Children’s Hospital at Westchester Medical Center at nine weeks of age with a 6-week history of left ear drainage, left eyelid weakness, drooling and poor feeding and 2 weeks of left sided facial drooping and enlarged lymph nodes around the ears and neck. On the day of admission there was an acute onset of gagging on saliva and turning blue. There was no history of fever. At the time of admission the vital signs were: temperature, 97.8°F; heart rate, 144/min; respiratory rate, 38/min; blood pressure, 75/35 mm Hg; weight, 3.21 kg; length, 44 cm; and head circumference, 37 cm (each of the anthropometric measurements was between the 50th and 75th percentile for her degree of prematurity). The infant was nontoxic appearing with erythematous tympanic membranes bilaterally and a perforated left tympanic membrane with purulent otorrhea. She had bilateral anterior and posterior cervical lymphadenopathy (2 by 1 cm), dysphagia, decreased gag reflex, hepatomegaly (3 cm below the costal margin) and left facial nerve palsy. Initial radiograph of the chest showed diffuse bilateral interstitial markings with areas of consolidation in the lingular and right middle lobes. The baby was cyanotic and had an apneic episode in the emergency room lasting ∼30 s. Oxygen therapy via a nasal cannula was initiated. Blood, urine, cerebrospinal fluid and ear drainage were cultured. White blood cell count was 19 000/dl with 35% neutrophils, 51% lymphocytes and 11% monocytes on differential. Cerebrospinal fluid was clear with 3 white blood cells/dl (48% segmented neutrophils, 4% monocytes, 48% lymphocytes), 3350 red blood cells/dl, glucose 66 mg/dl and protein 80 mg/dl. Gram stain of the ear drainage revealed Gram-negative bacilli. Computed tomograph scan of the head without contrast was normal. Antibiotic therapy was started with erythromycin estolate, trimethoprim-sulfamethoxazole and ceftazidime. During the first 3 days of hospitalization, there was slight improvement of the lymphadenopathy and ear drainage but persistent feeding difficulty, progressive lethargy and respiratory distress. Serial radiographs of the chest showed worsening interstitial densities in the right middle lobe, right upper lobe and lingula. A Mantoux test with 5 tuberculin units of purified protein derivative was positive with 12 mm of induration after 2 days. Smears of the ear discharge, cerebrospinal fluid and gastric aspirates were negative for acid-fast bacilli (AFB). Treatment was started with isoniazid, rifampin, ethambutol and pyrazinamide. Other antibiotics were stopped after 6 days of treatment. Computed tomograph scan of the temporal bone with contrast showed enhancement consistent with osteomyelitis of the left petromastoid region with associated left cervical lymph node calcification and inflammatory changes of the second portion of the left facial nerve canal. Biopsy showed caseating granulomas and the presence of AFB in a left cervical lymph node and left mastoid bone. Hepatic ultrasound examination showed prominent periportal triads interpreted as either a normal variant or acute hepatitis. Liver function tests were normal. There was no evidence of human immunodeficiency virus infection by polymerase chain reaction. By the third hospital week cultures of cerebrospinal fluid, left mastoid tissue biopsy, gastric aspirates and left middle ear fluid grew Mycobacterium tuberculosis. A second lumbar puncture performed at that time yielded clear cerebrospinal fluid with 2 white blood cells/dl (17% segmented neutrophils, 14% monocytes, 59% lymphocytes), 450 red blood cells/dl, glucose 57 mg/dl, protein 321 mg/dl. Prednisone was started in a dosage of 2 mg/kg/day which was reduced to 1 mg/kg/day after 1 week. Culture of the second cerebrospinal fluid sample also grew M. tuberculosis. One week after initiation of antituberculous medications, the patient began to improve clinically, and the facial weakness gradually resolved. She became more alert and active and started to gain weight. A third lumbar puncture 4 weeks after initiation of anti-tuberculous therapy yielded cerebrospinal fluid with 0 white blood cell/dl, 40 red blood cells/dl, glucose 53 mg/dl and protein 44 mg/dl. Culture of the third cerebrospinal fluid sample did not grow M. tuberculosis after 8 weeks of incubation. The patient was sent to her foster home 5 weeks after admission to continue treatment with isoniazid, rifampin, pyrazinamide and weaning doses of prednisone. Discharge weight was 4.12 kg, a 28% increase over the admission weight. The patient’s mother was admitted to a local hospital for treatment of nephrolithiasis 1 month after the baby had been born. Three days into her hospitalization, the mother was diagnosed to have pneumonia and Klebsiella pneumoniae bacteremia. She was treated with intravenous antibiotics and discharged receiving ciprofloxacin. When the baby’s diagnosis of tuberculosis was established, the mother was also admitted for evaluation. She reportedly had had a negative Mantoux test early in her pregnancy. During this admission her Mantoux test showed 20 by 22 mm of induration, and her chest radiograph was unremarkable without evidence of infiltrates, effusion, calcification or adenopathy. Cultures of three sputum specimens did not grow M. tuberculosis after 8 weeks of incubation. An endometrial biopsy was obtained; no AFB were seen by microscopy, but cultures of the specimen grew M. tuberculosis. Pathology report showed necrotizing granulomatous endometritis. The M. tuberculosis isolates from patient and mother had the same antimicrobial susceptibility profile. Restriction fragment length polymorphism performed by Wadsworth Laboratory, New York State Department of Health, showed both isolates to be identical. The baby’s contacts were evaluated for tuberculosis. Each contact in her foster home and all health care workers in the neonatal intensive care unit had a nonreactive Mantoux test. Discussion. Congenital tuberculosis with central nervous system involvement is very rare. Since 1950 there has been only one reported case in the United States 1 and a few other cases reported internationally. 2 The pathogenesis of congenital tuberculosis begins with transmission of M. tuberculosis 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 Bacilli in the pharynx migrate into the Eustachian tube and middle ear, causing otitis media. Bacilli may also be aspirated into the lungs causing pulmonary disease. Despite the fact that our patient presented at 9 weeks of age, the history strongly suggests that the disease process began ∼3 weeks after birth. We conclude that our patient had congenital tuberculosis based on the following: (1) the patient had minimal contact with the mother after delivery; (2) the mother had no respiratory symptoms and had a normal chest radiograph; (3) all of the patient’s contacts including relevant hospital staff and household members were healthy and had nonreactive Mantoux tests; (4) the patient had caseating granulomatous lesions in the ear canal, lymph node and mastoid bone; (5) the mother had endometrial caseating granulomas; and (6) the organisms cultured from mother and infant had the same restriction fragment length polymorphism pattern. These factors virtually exclude the possibility of postnatal transmission and demonstrate in utero transmission. Our patient presented with unilateral facial nerve palsy, which has been seen in cases of adult tuberculosis 4 but has not been described among clinical findings of congenital tuberculosis. In adults once facial palsy occurs, recovery of function is often poor, particularly if diagnosis and treatment are delayed. 5 Facial palsy may also occur because of inflammation of the facial nerve from a bony dehiscence within the middle ear from an episode of acute otitis media. 6 The palsy is usually unilateral and resolves with medical therapy for acute otitis media. Myringotomy, facial nerve decompression and mastoidectomy may become necessary if paralysis does not improve with medical intervention alone. 6 Facial nerve palsy in our patient was thought to be secondary to tuberculous otitis media and not caused by tuberculous meningitis because the inflammatory process involved the left petromastoid region and left facial nerve canal. Also the patient had tuberculous central nervous system infection without cerebrospinal fluid pleocytosis.