Adult patients with spina bifida cystica: genetic counselling, pregnancy and delivery

With more aggressive surgical management, patients born with spina bifida may now reach adulthood and achieve pregnancies. Any female patient with spina bifida is strongly recommended to have preconceptional genetic counselling. The risk for parents with spina bifida of having affected offspring (approx. 4%) is considerably increased compared with the general population (0.1-0.3%). This risk may be lowered when periconceptional folic acid supplements are given. In pregnancy, special care is needed in the management of urological, obstetric, neurological and anaesthetic problems. Urological complications like neurogenic bladder, incontinence, chronic infection, increased chance of developing bladder carcinoma and impaired renal function are common in the spina bifida patient. In case of urinary diversion, obstruction may complicate the pregnancy. The incidence of premature labour is increased. Clinical assessment of the pelvis is necessary because of a possibly contracted pelvis. If the head engages normally, vaginal delivery should be allowed if possible. Caesarean section should be performed for obstetric reasons only. Cerebrospinal fluid shunts may give neurological problems during pregnancy. In most cases reported, symptoms improved spontaneously after delivery. In case of a shunt, vaginal delivery is preferable, pushing during second stage not contra-indicated, and in case of caesarean section, prophylactic antibiotics and thorough irrigation of the peritoneal cavity are indicated.