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.