Case presentation
A 35-year-old Japanese woman with KS was referred to our department with a desire to achieve pregnancy. She married a 47-year-old healthy Japanese male 9 months before her first visit. She had been found to have situs inversus at 5 years old. She was diagnosed with KS at 27 years old, and her clinical and imaging findings revealed chronic sinusitis, bronchiectasis and situs inversus on electron microscopic examination of bronchial cilia. She had heterozygous mutations inDNAH5 at 33 years old [7]. She was offered an indication for lung transplantation at 34 years old. In her family history, her sister had KS.
Infertility examinations were performed, including basal body temperature charts, hysterosalpingography, semen analysis, antisperm antibodies and assays of serum thyroxine, prolactin and follicle-stimulating hormone, and all the data were within normal limits. Considering her offer of lung transplantation and the advanced age of her husband, IVF-ET was planned. She had conceived by the first cycle of IVF at 36 years old and attended antenatal care visits in our department. During the pregnancy, she was examined by a respiratory specialist at our hospital every month.
She noticed frequent exacerbation of cough with copious purulent sputum at 33 weeks of gestation. At 35 weeks and 1 day of gestation, she was admitted to our hospital because of exacerbation of cough and progressive dyspnea that made it difficult to maintain her supine position. On admission, a physical examination revealed a blood pressure of 94/60 mmHg, regular pulse rate of 94 beats per minute, respiratory rate of 24 breaths per minute, body temperature of 36.9 °C and SpO2 of 96% at room air. Auscultation of the chest revealed coarse crackles in both lungs, and the heartbeat was audible on the right side of the chest. The chest radiograph showed a cardiac shadow on the right side and bronchiectasis with fibrotic bands in the lower field on both sides, which was an unremarkable change from before pregnancy (Figure 1). Laboratory findings revealed a white cell count of 11,470/μL, hemoglobin of 11.1 g/dl, and C-reactive protein level of 1.84 mg/dL. The echocardiogram demonstrated normal cardiac function, and there was no evidence of systemic inflammatory disease. Ultrasound evaluation confirmed an appropriate date and viable fetus.
The timing and mode of delivery, as well as the potential pulmonary impact of continuing pregnancy, were discussed with combined input from the obstetrician, respiratory specialist and neonatologist. After a joint multidisciplinary team meeting, a decision was made to perform a preterm cesarean section under epidural and spinal anesthesia to avoid further exacerbation of the respiratory function due to diaphragmatic elevation by the gravid uterus. The cesarean section was performed at 35 weeks and 5 days of gestation under epidural and spinal anesthesia. The operating table was placed in a slight reverse Trendelenburg position (head up position at a 30° angle) during the operation (Figure 2). A healthy, female neonate weighing 2,633 g with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively, was delivered with no gross anomalies.
Following the operation, the patient was transferred to the intensive care unit (ICU), and supplemental oxygen was continued. In the ICU, chest physiotherapy by postural drainage was initiated to facilitate the drainage of bronchopulmonary secretions from the tracheobronchial tree. On the 1st postoperative day, supplemental oxygen was discontinued, and she was moved to the obstetrics ward. Her cough and dyspnea gradually improved, and she was discharged from our hospital on the 6th postoperative day. One month after delivery, the patient’s respiratory function was restored to prepregnancy values.