RESUMEN
BACKGROUND: Fronto-facial monobloc advancement with internal distraction (FFMBA) is a central procedure in the management of FGFR-related craniosynostoses. Children undergoing FFMBA may present with resorption of the frontal bony flap in the months or years following surgery. Here, we aimed at identifying the clinical factors associated with resorption and its extent in patients with Crouzon and Pfeiffer syndromes. METHODS: We conducted a retrospective monocentric study of children with Crouzon and Pfeiffer syndromes who underwent FFMBA between May 2008 and October 2021, with available CT-scan data (1) early after surgery before the initiation of distraction, (2) at the end of distraction, (3) 2 years after surgery and optionally (4) 5 years or more after surgery. The following parameters were included: sex, age, mutation, number of previous craniofacial procedures, presence of tracheostomy or ventriculo-peritoneal (VP) shunt, FFMBA indication (raised ICP, OSAS, exorbitism, morphology), per-operative parameters (use of osteosynthesis, per-operative advancement, duration of surgery) and post-operative parameters (CSF leak, infection, distraction initiation, total advancement, persistence of a retro-frontal dead space). RESULTS: 63 patients were included, with a mean age of 2.91 years (SD=1.61) at the time of surgery, male/female sex ratio of 1.1, and a majority of Crouzon syndrome (84%). Two years after surgery (average: 622 days, SD=286.7), mean frontal bone flap resorption was 4.83 cm2 ([0-27.3], SD=5.35). Factors associated with more severe frontal bone resorption were the intraoperative use of absorbable sutures vs steel wires (9.83 vs. 3.99, p=0.04) and the persistence of a retro-frontal dead space at 2 years after surgery (13.12 vs. 3.63, p<0.001). CONCLUSION: The use of absorbable sutures for frontal bone fixation in FFMBA and the persistence of a retro-frontal dead space two years after surgery are risk factors for bone resorption. This report indicates that rigid osteosynthesis should be favored, and both per-operative and early post-operative distractor activation should be performed with care and follow-up of brain expansion.
RESUMEN
OBJECTIVE: Epidural hematoma (EDH) has rarely been studied specifically in infants. The objective of this study was to investigate the outcomes of patients aged < 18 months (infants) with EDH. METHODS: The authors conducted a single-center retrospective study of 48 infants aged less than 18 months who underwent an operation for a supratentorial EDH in the last decade. Clinical, radiological, and biological variables were used in a statistical analysis to identify factors predictive of radiological and clinical outcome. RESULTS: Forty-seven patients were included in the final analysis. Seventeen children (36%) had cerebral ischemia on postoperative imaging, either due to stroke (cerebral herniation) or by local compression. Factors associated with ischemia after multivariate logistic regression were the presence of an initial neurological deficit (76% vs 27%, p = 0.03), low platelet count (mean 192 vs 267 per mm3, p = 0.01), low fibrinogen level (mean 1.4 vs 2.2 g/L, p = 0.04) and long intubation time (mean 65.7 vs 10.1 hours, p = 0.03). Cerebral ischemia on MRI was predictive of a poor clinical outcome. CONCLUSIONS: Infants with EDH have a low mortality rate but a high risk of cerebral ischemia, along with long-term neurological sequelae.