Surgical procedures
Gradual enlargement of the posterior cranial vault in raised intracranial pressure and syndromic craniosynostosis.
Posterior vault distraction osteogenesis (PVDO) gradually enlarges the posterior cranial vault. Osteotomies are made in the occipital region and distractors are implanted, then advanced by small daily increments over several weeks — similar to the principle of callus distraction in bone. The space gained relieves the brainstem and cerebellum and lowers intracranial pressure.
Often in the first or second year of life — frequently as the first procedure in severely affected syndromic forms. Timing depends on the ICP course, ophthalmological findings (papilloedema) and growth rate. Regular monitoring is essential.
Through an occipital approach, osteotomies are made in the back of the skull and one or more distractors are implanted. After a latency phase of a few days, active distraction begins: the distractor is advanced by about 1 mm per day until the target volume is reached (typically 2–4 weeks of distraction, then retention). A second procedure to remove the distractors is required. Duration of the first procedure: approx. 2–4 hours.
Comparative studies (incl. Breakey et al., Plast Reconstr Surg 2023, Crouzon and Apert patients) show that posterior cranial vault reconstruction (PCVR), posterior vault distraction (PVDO) and spring-assisted posterior vault expansion (SAPVE) achieve comparable volume gains. PVDO offers the advantage of achieving larger expansion distances gradually and without major blood loss — particularly relevant in very young children.
The primary aim is to lower intracranial pressure and prevent secondary brain damage. The posterior airway is also relieved. In Pfeiffer syndrome type 2/3, PVDO is often life-saving and forms the basis for all further craniofacial procedures.
The back of the skull is gradually expanded to give the brain more room and reduce intracranial pressure. The procedure is planned individually.
Often in syndromic craniosynostosis in early childhood. The decision depends on the findings and is made at the specialist centre.