A European multicenter outcome study on the different perioperative airway management policies following midface surgery in syndromic craniosynostosis:a proposal for a Standard Operating Procedure

BACKGROUND: Perioperative airway management following midface advancements in children with Apert and Crouzon/Pfeiffer syndrome can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications.METHODS: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc / le Fort III procedures.RESULTS: Ultimately, 275 patients (129 monobloc and 146 Le Fort III) were included; 62 received immediate extubation and 162 delayed extubation; 42 had long-term tracheostomies and nine perioperative short-term tracheostomies. Short-term tracheostomies were in most centers reserved for selected cases. Patients with delayed extubation remained intubated for three days (IQR 2 - 5). The rate of no or only oxygen support after extubation was comparable between patients with immediate and delayed extubation, 58/62 (94%) and 137/162 (85%) patients, respectively. However, patients with immediate extubation developed less postoperative pneumonia than those with delayed, 0/62 (0%) versus 24/161 (15%) (P = 0.001), respectively. Immediate extubation also appeared safe in moderate/severe OSA since 19/20 (95%) required either no or only oxygen support after extubation. The odds of developing intubation-related complications increased by 21% with every extra day of intubation.CONCLUSIONS: Immediate extubation following midface advancements was found to be a safe option, as it was not associated with respiratory insufficiency but did lead to fewer complications. Immediate extubation should be considered routine management in patients with no/mild OSA and should be the aim in moderate/severe OSA after careful assessment.</p

Multifocal intraductal tubulopapillary neoplasm of the pancreas with total pancreatectomy: report of a case and review of literature.

Intraductal neoplasms of the pancreas are classified as intraductal tubulopapillary neoplasms (ITPNs) and intraductal papillary mucinous neoplasm (IPMNs) in the current WHO classification. ITPN is a rare tumor and there are only a few cases of ITPN reported in the literature. We present the case of an otherwise healthy 42-year-old male, who presented with upper abdominal pain. He was subsequently diagnosed with multifocal ITPN and underwent total pancreatectomy. The pathological report showed invasive growth. The postoperative course was uneventful and the patient received 6 months of adjuvant chemotherapy with gemcitabine-capecitabine. The patient is still alive 19 months after the procedure with no signs of recurrence. Literature review revealed only 30 individual cases of ITPN in the pancreas including our reported case. Mean age was 61 years (16 males/14 females; ratio 1.14:1). Mean tumor size was 3 cm. Immunohistochemical staining was positive for CK-7 in 100% of the patients, CK-19 in 95% and for MUC-1 in 88%. Trypsin was negative in all cases. β-catenin was negative in 94% and MUC-2 was negative in 96% of the cases. BRAF, KRAS, TP53 and PIK3CA mutations were infrequently seen. Invasive growth was present in 54% of the cases. Tumor size and Ki-67 index showed a statistically significant association with invasive growth. Survival rate could not be determined, due to short follow-up, and further research is needed to establish prognostic factors for disease recurrence and survival

FACE-Q craniofacial module: Part 2 Psychometric properties of newly developed scales for children and young adults with facial conditions

Background: The FACE-Q Craniofacial Module is a patient-reported outcome measure designed for patients aged 8 to 29 years with conditions associated with a facial difference. In part 1, we describe the psychometric findings for the original CLEFT-Q scales tested in patients with cleft and noncleft facial conditions. The aim of this study was to examine psychometric performance of new FACE-Q Craniofacial Module scales. Methods: Data were collected between December 2016 and December 2019 from patients aged 8 to 29 years with conditions associated with a visible or functional facial difference. Rasch measurement theory (RMT) analysis was used to examine psychometric properties of each scale. Scores were transformed from 0 (worst) to 100 (best) for tests of construct validity. Results: 1495 participants were recruited with a broad range of conditions (e.g., birthmarks, facial paralysis, craniosynostosis, craniofacial microsomia, etc.) RMT analysis resulted in the refinement of 7 appearance scales (Birthmark, Cheeks, Chin, Eyes, Forehead, Head Shape, Smile), two function scales (Breathing, Facial), and an Appearance Distress scale. Person separation index and Cronbach alpha values met criteria. Three checklists were also formed (Eye Function, and Eye and Face Adverse Effects). Significantly lower scores on eight of nine scales were reported by participants whose appearance or functional difference was rated as a major rather than minor or no difference. Higher appearance distress correlated with lower appearance scale scores. Conclusion: The FACE-Q Craniofacial Module scales can be used to collect and compare patient reported outcomes data in children and young adults with a facial condition