Craniosynostosis and Apert Syndrome

2018 ◽  
Author(s):  
Petra M. Meier ◽  
Thomas O. Erb
Keyword(s):  
Point Of Care ◽  
Multidisciplinary Treatment ◽  
Sleep Apnea Syndrome ◽  
Maxillofacial Surgery ◽  
Surgical Techniques ◽  
Growth Factor Receptor ◽  
Air Embolism ◽  
Apert Syndrome ◽  
Venous Air Embolism ◽  
Obstructive Sleep

Apert syndrome is a complex, progressive multisystem condition of the craniosynostosis spectrum originating from a fibroblast growth factor receptor disorder. Multidisciplinary treatment teams may include craniofacial surgery, neurosurgery, otolaryngology, ophthalmology, oro-maxillofacial surgery, and pediatric intensive care. Secondary to midface hypoplasia, children often present with a compromised airway and have a high incidence of sleep disorders. Anesthetic considerations include difficult airway assessment, the presence of obstructive sleep apnea syndrome, and increased intracranial pressure. Extensive remodeling procedures can be associated with massive hemorrhage (e.g., venous sinus bleeding) and venous air embolism. Transfusion-related complications include coagulopathy, metabolic derangements, and primarily noninfectious hazards such as transfusion-related acute lung injury and transfusion-related immunomodulation. Multimodal blood management should focus on a combination of appropriate surgical techniques and blood conservation, along with guidance from point-of-care testing (including coagulation).

scholarly journals Lateral-Expansion Pharyngoplasty: Combined Technique for the Treatment of Obstructive Sleep Apnea Syndrome

2019 ◽  
Vol 24 (01) ◽  
pp. e107-e111 ◽  
Author(s):  
José Antonio Pinto ◽  
Luciana Balester Mello de Godoy ◽  
Heloisa dos Santos Sobreira Nunes ◽  
Kelly Elia Abdo ◽  
Gabriella Spinola Jahic ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Obstructive Sleep Apnea Syndrome ◽  
Sleep Apnea Syndrome ◽  
Surgical Techniques ◽  
Upper Airway ◽  
Apnea Hypopnea Index ◽  
Lateral Expansion ◽  
Obstructive Sleep ◽  
Apnea Syndrome

Abstract Introduction Obstructive sleep apnea syndrome (OSAS) is a multifactorial disease characterized by episodes of partial or complete collapse during sleep of different regions of the upper airway. Surgery for OSAS evolved with the introduction of different techniques, considering new surgical concept of reconstruction of the upper airway. Objective To retrospectively evaluate the effectiveness of a new approach aimed at reducing pharyngeal collapse by combining two surgical techniques: lateral and expansion pharyngoplasty. Methods We reviewed the medical records of 38 patients with OSAS undergoing lateral/expansion pharyngoplasty from January 2012 to December 2016. The following data were collected: patient age, gender, and pre- and postoperative body mass index (BMI), Epworth sleepiness scale (ESS) scores, snoring visual analogue scale (VAS) scores, and polysomnography (PSG) results. Results The PSG results showed a significant reduction in the apnea/hypopnea index (AHI) from 22.4 ± 27.3 events/h preoperatively to 13.6 ± 17.9 events/h postoperatively (p = 0.009), with postoperative AHI reduction greater than 50% in 63.2% of the patients. There was also a significant reduction in the microarousal index (19.5 ± 22.6 vs 11.0 ± 13.4 events/h; p = 0.001) and in the minimum oxygen saturation (82.6 ± 10.3 vs 86.9 ± 11.1; p = 0.007). Conclusions Lateral-expansion pharyngoplasty represents a new surgical strategy for the treatment of OSAS in patients with palatal collapse by combining two different techniques: lateral and expansion pharyngoplasty. The two techniques, performed as a one-stage procedure, led to improvements in excessive daytime sleepiness, snoring, and PSG respiratory parameters by acting on lateral and retropalatal collapse, producing favorable results with good applicability in otolaryngology clinical practice.

scholarly journals Manipulation of Lateral Pharyngeal Wall Muscles in Sleep Surgery: A Review of the Literature

2020 ◽  
Vol 17 (15) ◽  
pp. 5315
Author(s):  
Giovanni Cammaroto ◽  
Luigi Marco Stringa ◽  
Giannicola Iannella ◽  
Giuseppe Meccariello ◽  
Henry Zhang ◽  
...  
Keyword(s):  
English Literature ◽  
Sleep Apnea Syndrome ◽  
Surgical Techniques ◽  
Upper Airway ◽  
Variable Degree ◽  
Lateral Pharyngeal Wall ◽  
Pharyngeal Wall ◽  
Obstructive Sleep ◽  
Upper Airway Collapsibility ◽  
No Gold Standard

Background: Obstructive sleep apnea syndrome (OSAS) occurs due to upper airway obstruction resulting from anatomical and functional abnormalities. Upper airway collapsibility, particularly those involving the lateral pharyngeal wall (LPW), is known to be one of the main factors contributing to the pathogenesis of OSAS, leading the authors of the present study to propose different strategies in order to stiffen the pharyngeal walls to try to restore normal airflow. Methods: An exhaustive review of the English literature on lateral pharyngeal wall surgery for the treatment of OSAS was performed using the PubMed electronic database. Results: The research was performed in April 2020 and yielded approximately 2000 articles. However, considering the inclusion criteria, only 17 studies were included in the present study. Conclusions: The analyzed surgical techniques propose different parts of LPW on which to focus and a variable degree of invasivity. Despite the very promising results, no gold standard for the treatment of pharyngeal wall collapsibility has been proposed. However, thanks to progressive technological innovations and increasingly precise data analysis, the role of LPW surgery seems to be crucial in the treatment of OSAS patients.

scholarly journals An Infant with Apert Syndrome and Tetralogy of Fallot for Craniosynostosis Correction: Anesthetic Challenges

2019 ◽  
Author(s):  
Karen R. Lionel ◽  
Satish K. Sundararajan ◽  
Ranjith K. Moorthy ◽  
Ramamani Mariappan
Keyword(s):  
Tetralogy Of Fallot ◽  
Autosomal Dominant ◽  
Air Embolism ◽  
Massive Bleeding ◽  
Apert Syndrome ◽  
Craniofacial Anomalies ◽  
Autosomal Dominant Disorder ◽  
Venous Air Embolism ◽  
Congenital Cardiac Anomalies ◽  
Hands And Feet

AbstractApert syndrome (AS) is an autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and symmetrical syndactyly of hands and feet. Ten percent children with AS can have associated congenital cardiac anomalies. Association of complex cyanotic heart disease with craniosynostosis is very rare. So far, only one case has been reported in the literature. The craniosynostosis corrective surgery is associated with the risk of massive bleeding or venous air embolism, which can cause paradoxical air embolism and precipitate cyanotic spell, which makes the anesthesia more challenging. In this report, we present the anesthetic challenges of an 8-month-old infant with AS and tetralogy of Fallot for craniosynostosis correction.

Obstructive Sleep Apnea Syndrome: Pathophysiology, Upper Airway Evaluation and Surgical Treatment

1993 ◽  
Vol 72 (1) ◽  
pp. 67-76 ◽  
Author(s):  
Shiro Fujita
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Obstructive Sleep Apnea Syndrome ◽  
Sleep Apnea Syndrome ◽  
Maxillofacial Surgery ◽  
Upper Airway ◽  
Medical Problems ◽  
Obstructive Sleep ◽  
Cardiopulmonary Complications ◽  
Apnea Syndrome

This paper describes the pathophysiology of the upper airway and the methods of evaluation which include mainly the Mueller maneuver, and cephalometrics. The management of Obstructive Sleep Apnea Syndrome (OSAS) is determined by the level of daytime dysfunction, the degree of cardiopulmonary complications, age, co-existing medical problems, life style, and the extent of the upper airway obstruction and abnormality. Continuous positive airway pressure (C-PAP), uvulopalatopharyngoplasty (UPPP), and permanent tracheostomy are procedures indicated depending on the severity of the case. Midline laser glossectomy was introduced as a new surgical procedure approach to the treatment of OSAS, as an alternative to maxillofacial surgery. When combined with UPPP, the successful results are improved.

SYSTEMATIC REVIEW AND META-ANALYSIS OF THE PALATAL SURGERIES IN THE TREATMENT OF OBSTRUCTIVE SLEEP APNEA

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Amr Nabil Rabie ◽  
Ossama Mady ◽  
Ahmed Negm El-Shazly ◽  
Alaa Abouzeid
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Meta Analysis ◽  
Sleep Apnea Syndrome ◽  
Surgical Techniques ◽  
P Value ◽  
Obstructive Sleep ◽  
Expansion Sphincter Pharyngoplasty ◽  
Sphincter Pharyngoplasty ◽  
Meta Analyses

Abstract Background The first description of a patient with obstructive sleep apnea was in ‘The Posthumous Papers of the Pickwick Club’ published by English writer Charles Dickens in 18361. The first introduction of the terms ‘sleep apnea syndrome’ and ‘obstructive sleep apnea syndrome’ was by Guilleminault et al in 1967. Subsequently, Guilleminault coined the term ‘upper airway resistance syndrome’ in 19932. Objectives This paper aims to examine published articles between January 2008 and January 2019 on the different palatal surgeries performed on OSA patients and the treatment outcomes, which in turn will be used to determine two things. The first being the effectiveness of various palatal surgical techniques in treating OSAS and the second (if applicable) the most effective of the palatal surgical techniques in treating OSAS. Patients and Methods The current review followed the guidelines of preferred reporting items for systematic reviews and meta-analysis statement 2009 (PRISMA)5. The detailed steps of methods were described elsewhere as well as PRISMA checklist. The quality of relevant studies was assessed using NIH quality assessment tool for observational cohort and cross -sectional studies as well as NIH tool for quality assessment for case series studies. Results Meta-analyses of relevant studies showed that the surgical technique that achieved the best reduction on AHI was the lateral pharyngoplasty followed by the Anterior Palatoplasty, with a significant mean reduction of [(SMD= -0.848, 95% CI (-1.209 – -0.487), p-value<0.001) and (SMD= -0.864, 95% CI (-1.234 – -0.494), p-value<0.001), respectively]. The technique responsible for the best improvement in ESS was the Relocation Pharyngoplasty, with a significant mean reduction of [SMD= -0.998, 95% CI (-1.253 – -0.743), p-value<0.001]. Minimal O2 saturation level improved most with the Expansion Sphincter Pharyngoplasty, with a significant mean reduction of [SMD= 1.011, 95% CI (0.581 – 1.440), p-value<0.001]. The surgical procedure that result in the best post-operative VAS was Z-Palatoplasty, with a significant mean reduction of [SMD= -1. 551, 95% CI (-2.049 – -1.052), p-value<0.001]. soft palate length change with a significant mean reduction of [SMD= -2.219, 95% CI (-2.730 – 1.708), p-value<0.001]. Finally, meta-analyses of relevant studies showed that expansion sphincter pharyngoplasty achieved the overall highest success rate [Event rate= 77%, 95% CI (65.4%– 85.5%), p-value<0.001] Conclusion The best procedure for treating OSA varies from patient to patient and there is no universal cure-all. Careful patient selection and pre-operative evaluation are mandatory.

scholarly journals Point-of-care Ultrasound Detection of Intraoperative Venous Air Embolism

2017 ◽  
Vol 127 (4) ◽  
pp. 711-711 ◽  
Author(s):  
Walid Alrayashi ◽  
Tonya Miller ◽  
Daniel Vo
Keyword(s):  
Point Of Care ◽  
Air Embolism ◽  
Point Of Care Ultrasound ◽  
Venous Air Embolism
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