Monochorionic Twin Pregnancy in a Patient with Type 3 Osteogenesis Imperfecta: A multi-disciplinary challenge

A Case Report 

Background:

Osteogenesis imperfecta (OI), also known as brittle bone disease, is
a rare connective tissue disorder that causes collagen deficiency
and multiple fractures. Patients with severe OI usually have severe
short stature and kyphoscoliosis, restrictive lung disease, and are
often wheelchair bound. Pregnancies in such patients have multiple
challenges and considerations which must be carefully managed in
a multi-disciplinary matter.

 

Case Summary:

A 27-year-old woman with severe type III osteogenesis imperfecta
spontaneously conceived a monochorionic, diamniotic twin
pregnancy. At delivery, she was 87cm tall and weighed 20kg. Due
to the severity of her condition, her pregnancy required close
follow-up involving a multidisciplinary team, including high-risk
obstetricians, anaesthesiologists, pulmonologists and respiratory
therapists. Her cardiorespiratory function showed signs of
compromise early on in pregnancy even from the first trimester.
She was initiated on non-invasive ventilation at 23 weeks.
Eventually, in view of her increasing respiratory distress, the twins
were delivered via classical Caesarean section at 26 weeks’
gestation under general anaesthesia. Intra-operatively there was
uterine atony and blood loss requiring transfusion. She remained
clinically stable and was discharged well on post-operative day 11.