Research syndromic craniosynostosis

Severe macroglossia after posterior fossa and craniofacial surgery in children.

Bouaoud J, Joly A, Picard A, Thierry B, Arnaud E, James S, Hennessy I, McGarvey B, Cairet P, Vecchione A, Vergnaud E, Duracher C, Khonsari RH.

Int J Oral Maxillofac Surg. 2018 Apr;47(4):428-436.

Massive swelling of the tongue may occur after craniofacial surgery. It can be a life-threatening condition if upper airway obstruction results. Three cases with severe swelling of the tongue following surgery are presented. The patients has a prolonged stay at the intensive care unit.


The Role of Bipartition Distraction in the Treatment of Apert Syndrome.

Glass GE, Ruff CF, Crombag GAJC, Verdoorn MHAS, Koudstaal M, Anguilla F, Hayward R, Britto JA, Jeelani O, Dunaway DJ.

Plast Reconstr Surg. 2018 Mar;141(3):747-750.

Ten patients with Apert syndrome underwent a bipartition procedure with distraction. Measurement before and after the surgery were taken to determine changes in facial shape. Results showed that this procedure corrects the midface, the shallowness of the orbits (exorbitism), and the too wide position of the eyes (hypertelorism). However, the disproportion in height of the midface and the abnormal shape of the orbits are not corrected. The face remains too wide at the level of the zygomas and facial asymmetry persists.


Sleep Architecture Linked to Airway Obstruction and Intracranial Hypertension in Children with Syndromic Craniosynostosis.

Spruijt B, Mathijssen IM, Bredero-Boelhouwer HH, Cherian PJ, Corel LJ, van Veelen ML, Hayward RD, Tasker RC, Joosten KF.

Plast Reconstr Surg. 2016 Dec;138(6):1019e-1029e.

In this study, 39 patients with syndromic craniosynostosis were evaluated: 19 did not have obstructive sleep apnea nor intracranial hypertension, 11 had obstructive sleep apnea, 6 had intracranial hypertension and three had both. The sleep pattern of patients with syndromic craniosynostosis only were identical to healthy children of the same age. Patients with moderate to severe obstructive sleep apnea had a disturbed sleep pattern, with for instance reduced time in deep sleep. Following surgery that improved breathing, the sleep pattern also improved.