scholarly journals Anesthesia management of pediatric dentistry patients with cardiofaciocutaneous syndrome: a case report

Author(s):  
Gian Luigi Gonnella ◽  
Pietro Paolo Giuri ◽  
Bruno Antonio Zanfini ◽  
Matteo Biancone ◽  
Luciano Frassanito ◽  
...  
2020 ◽  
Vol 24 (1) ◽  
pp. 105-107
Author(s):  
Sedighe Shahhosseini ◽  
Reza Aminnejad ◽  
Amir Shafa ◽  
Mehrdad Memarzade

Carvajal syndrome is a rare genetic disorder. Patients reporting for surgery pose some difficulties in anesthesia management. In this case report we present the case of a 12-year-old boy, who was a known case of Carvajal syndrome, referred for surgical resection of perianal condyloma. Close monitoring of hemodynamic status is the mainstay of anesthetic considerations in such patients. As in any other challenging scenario, it should be kept in mind that ‘there is no safest anesthetic agent, nor the safest anesthetic technique; there is only the safest anesthesiologist’. Citation: Shahhosseini S, Aminnejad R, Shafa A, Memarzadeh M. Anesthesia in Carvajal syndrome; the first case report. Anaesth pain intensive care 2020;24(1):___ DOI: https://doi.org/10.35975/apic.v24i1.


2019 ◽  
Vol 22 (2) ◽  
pp. 281-288
Author(s):  
Flávia Silva Pires ◽  
Viviane Andrade Cancio Paula ◽  
Khawana Faker ◽  
Natalia Di Lanaro ◽  
Raissa Christiane O de Carvalho ◽  
...  

Objective: The objective of this paper was to describe the oral conditions of two children accompanied by their mothers who reported to the Department of Pediatric Dentistry of Fluminense Federal University with Williams-Beuren syndrome (WBS). Case report: The 9-year-old female patient had a family and medical history significant for placental abruption, caesarean section, delayed psychomotor development, learning disabilities, tendency to selfdistract and congenital heart disease. In contrast, the 7-year-old male patient had a normal birth and no gestational intercurrences. Discussion: Clinically, the female presented with mixed dentition, crowding in the maxillary and mandibular arches, prolonged retention of deciduous teeth, anterior and posterior cross-bite and Angle Class I malocclusion, while the male had mixed dentition and retarded psychomotor development. Due to the patients having congenital heart disease, a prophylactic antibiotic regimen was prescribed prior to the dental procedures in both of them. Conclusion: These patients had been followed up for 2 years and this case report underscores the importance of early dental evaluation and counselling for parents of WBS patients.KeywordsWilliams Beuren syndrome; Oral manifestations; Child.


2018 ◽  
Vol 1 (1) ◽  
pp. 01-02
Author(s):  
Vardhaman Naide ◽  
Shenuka Singh

Introduction: Ingestion of a rubber dam clamp is a very rare condition that occurs due to careless manipulation of a rubber dam insertion especially in pediatric dentistry. Case report: The present paper reports a case in which a rubber dam clamp was accidentally swallowed by child undergoing pulpotomy in his upper right deciduous second molar, which entered esophagus and got stuck supraclavicular. Discussion: Ingestion of foreign materials may cause serious complications. Owing to the shape and sharpness of the instrument, there are chances of perforation. Once the instrument is lost in the oropharynx, it is very important to determine whether the instrument has entered the digestive tract or respiratory tract. Conclusion: Dentist should be able to manage an emergency situation, in which patient accidentally swallow a dental instrument.


2019 ◽  
Vol 8 (4) ◽  
pp. 49-53
Author(s):  
V. V. Umnov ◽  
N. V. Nikitina ◽  
A. M. Khodorovskaya ◽  
O. V. Barlova

The cardiofaciocutaneous syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation. The syndrome is caused by molecular disturbances in the RAS/MAPK pathway. We report on the girl, 9 year-old, with the cardiofaciocutaneous syndrome presenting with typical craniofacial appearance, heart defects, ectodermal abnormalities, neglected orthopedic pathology, developmental delay and spasticity, which rare in this syndrome.


2017 ◽  
Vol 22 (4) ◽  
pp. 202-203
Author(s):  
Abdullah Kahraman ◽  
Cahide Kahraman

2017 ◽  
Vol 41 (1) ◽  
pp. 66-69 ◽  
Author(s):  
Yousr N Mowafy ◽  
Nadia A Wahba ◽  
Aly A Sharaf

Background: Joubert syndrome is a very rare condition with dismal prognosis. It is characterized by several abnormalities including molar tooth sign on MRI. When coupled with mega cisterna magna- a feature of the Dandy Walker syndrome- it is categorized as Joubert plus syndrome. Case report: A 16 month old male child with Joubert syndrome was referred to the Pediatric Dentistry Department Clinic, Faculty of Dentistry Alexandria University, complaining of severe tongue and lower lip injury due to self-mutilation. He required multiple teeth extractions under general anesthesia to prevent further tongue and lip mutilation. Conclusion: Joubert plus syndrome is a very rare occurring condition. Because self-mutilation is sometimes fatal, a treatment plan tailored to each patient's need is mandatory. A multidisciplinary approach is recommended.


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