scholarly journals Anesthetic Management of a Patient with Harlequin Ichthyosis

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Klint J. Smart ◽  
Catherine A. Gruffi ◽  
Tara M. Doherty

Harlequin ichthyosis is a severe and often fatal form of congenital ichthyosis caused by defective lipid transport which results in a dysfunctional skin barrier. Patients who survive the neonatal period are predisposed to skin infections, sepsis, impaired thermoregulation, and dehydration. The unique skin characteristics can present significant anesthetic challenges. We highlight the relevant anesthetic considerations in a 3-year-old presenting for syndactyly release of the right second and fourth digits. We describe the steps to ensure protection of the fragile skin barrier during establishment of intravenous access and airway management, therefore providing guidance for care of this vulnerable patient population.

Author(s):  
Andrew T. Waberski ◽  
Nina Deutsch

Transposition of the great arteries is a congenital cardiac abnormality that presents in the neonatal period, most commonly as cyanosis. While variations in anatomic features exist, dextro-transposition of the great arteries, the most common form, results in 2 separate circulatory systems in parallel, such that the right ventricle pumps deoxygenated blood to the systemic circulation, and the left ventricle sends oxygenated blood back to the pulmonary circulation. To ensure survival, early diagnosis and intervention to allow for adequate mixing of blood is necessary. The arterial switch operation is the definitive treatment, usually undertaken in the first few days of life. Known complications of surgery include ischemia, bleeding, hemodynamic compromise, and arrhythmias. Anesthetic management must take these factors into account.


2018 ◽  
Vol 22 (3) ◽  
pp. 285-293 ◽  
Author(s):  
Rishi Parikh ◽  
Michael Eisses ◽  
Gregory J. Latham ◽  
Denise C. Joffe ◽  
Faith J. Ross

Truncus arteriosus is a congenital cardiac lesion in which failure of embryonic truncal septation results in a single semilunar valve and single arterial trunk providing both pulmonary and systemic circulations. Most patients with this lesion are symptomatic in the neonatal period with cyanosis and/or congestive heart failure and undergo complete repair in the first weeks of life. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with truncus arteriosus.


2021 ◽  
pp. 1-3
Author(s):  
Claire Bertail-Galoin

Abstract A fistula between the pulmonary artery and the left atrium is a rare entity and its diagnosis is uncommon in the neonatal period. There are more reported surgical treatments in the literature than with a transcatheter closure. We report the case of a prenatal diagnosis of a large fistula between the right pulmonary artery and the left atrium with successful transcatheter closure with an Amplatzer duct occluder II 6/4 mm.


HPB Surgery ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Aliki Tympa ◽  
Kassiani Theodoraki ◽  
Athanassia Tsaroucha ◽  
Nikolaos Arkadopoulos ◽  
Ioannis Vassiliou ◽  
...  

Background. Hazards of liver surgery have been attenuated by the evolution in methods of hepatic vascular control and the anesthetic management. In this paper, the anesthetic considerations during hepatic vascular occlusion techniques were reviewed. Methods. A Medline literature search using the terms “anesthetic,” “anesthesia,” “liver,” “hepatectomy,” “inflow,” “outflow occlusion,” “Pringle,” “hemodynamic,” “air embolism,” “blood loss,” “transfusion,” “ischemia-reperfusion,” “preconditioning,” was performed. Results. Task-orientated anesthetic management, according to the performed method of hepatic vascular occlusion, ameliorates the surgical outcome and improves the morbidity and mortality rates, following liver surgery. Conclusions. Hepatic vascular occlusion techniques share common anesthetic considerations in terms of preoperative assessment, monitoring, induction, and maintenance of anesthesia. On the other hand, the hemodynamic management, the prevention of vascular air embolism, blood transfusion, and liver injury are plausible when the anesthetic plan is scheduled according to the method of hepatic vascular occlusion performed.


Antibiotics ◽  
2020 ◽  
Vol 9 (2) ◽  
pp. 56 ◽  
Author(s):  
R. Smith ◽  
J. Russo ◽  
J. Fiegel ◽  
N. Brogden

The epidermal skin barrier protects the body from a host of daily challenges, providing protection against mechanical insults and the absorption of chemicals and xenobiotics. In addition to the physical barrier, the epidermis also presents an innate defense against microbial overgrowth. This is achieved through the presence of a diverse collection of microorganisms on the skin (the “microbiota”) that maintain a delicate balance with the host and play a significant role in overall human health. When the skin is wounded, the local tissue with a compromised barrier can become colonized and ultimately infected if bacterial growth overcomes the host response. Wound infections present an immense burden in healthcare costs and decreased quality of life for patients, and treatment becomes increasingly important because of the negative impact that infection has on slowing the rate of wound healing. In this review, we discuss specific challenges of treating wound infections and the advances in drug delivery platforms and formulations that are under development to improve topical delivery of antimicrobial treatments.


2021 ◽  
Vol 11 (2(40)) ◽  
pp. 64-67
Author(s):  
I.V. Lastivka ◽  
A.G. Babintseva ◽  
V.V. Antsupova ◽  
А.І. Peryzhniak ◽  
І.V. Koshurba ◽  
...  

Hemifacial Microsomia (HFM) is a term used to identify facial deformities associated with the development ofthe first and second pairs of branchial arches, characterized by underdevelopment of one half of the face. One typeof hemifacial microsomia is oculo-auriculo-vertebral dysplasia or Goldenhar syndrome.The incidence of HFM is 1:3500-1:7000 of live births and occurs in 1 case per 1000 children with congenitaldeafness. The ratio of boys to girls is 3:2. The etiology and type of inheritance is studied insufficiently. There are threepossible pathogenetic models: vascular abnormalities and hemorrhages in the craniofacial region, damage of Meckel'scartilage, and abnormal cell development of the cranial nerve crest. Environmental factors, maternal internal factors,and genetic factors (OTX2, PLCD3, and MYT1 mutations) may also cause the development of hemifacial microsomia.The article demonstrates a clinical case of hemifacial microsomia in a newborn boy from a mother with Z-21 inthe form of deformation of the left auricle with atresia of the auditory canal and "false" ears on the right, combinedwith congenital anomaly of heart (atrial septal defect) and brain (hypoplasia of the corpus callosum).Emphasis is placed on the need of involving a multidisciplinary team of specialists in the management of thispatient both in the neonatal period and in the system of subsequent follow-up.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Kaitlin E. Woods ◽  
J. W. Awori Hayanga ◽  
Daniel Sloyer ◽  
Roy E. Henrickson ◽  
Lawrence M. Wei ◽  
...  

Dextrocardia involves embryologic malformations leading to a right hemithorax heart with rightward apex. Situs inversus encompasses all viscera in mirrored position. A 76-year-old male with dextrocardia with situs inversus presented for coronary artery bypass grafting due to a non-ST elevation myocardial infarction. Management was altered accordingly. Electrocardiography leads and defibrillator pads were reversed. A left internal jugular vein central venous catheter provided direct access to the right atrium. Transesophageal echocardiography confirmation of aortic and venous cannulation required turning the probe right for the right-sided aorta and left for liver visualization, respectively. Proactive surgical and anesthetic management was imperative for the successful and uneventful outcome for this patient.


2009 ◽  
Vol 4 (1) ◽  
pp. 85-90 ◽  
Author(s):  
Robert J. Bollo ◽  
Jonathan L. Berliner ◽  
Ingeborg Fischer ◽  
Daniel K. Miles ◽  
Elizabeth A. Thiele ◽  
...  

Subependymal giant cell tumors (SGCTs) are observed in 5–20% of patients with tuberous sclerosis complex (TSC) but account for ~ 25% of neurological morbidity. The authors report the case of a 7-year-old girl with TSC and multiple cortical tubers who presented with worsening seizures in the context of the rapid growth of a cystic, calcified, extraventricular SGCT in the right frontal lobe, initially thought to represent a cortical tuber. The tumor and surrounding tubers were excised, and clinical seizures resolved. This is the first report of an extraventricular SGCT in a child with TSC outside the neonatal period.


2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Mohammad Hadi Gharedaghi ◽  
Saman Ahmadi ◽  
Arjang Khorasani ◽  
Farzad Ebrahimi

Pericardial cysts are rare mediastinal cysts composed of a single fluid-filled mesothelial layer and can be congenital in origin or develop secondary to pericarditis, trauma, or infection. Although most pericardial cysts are asymptomatic, life-threatening complications can occasionally occur. We report on a 57-year-old man with an asymptomatic 9 cm pericardial cyst that was incidentally found as an abnormal cardiac silhouette on routine chest radiography. Further imaging confirmed the presence of a pericardial cyst that was compressing the right atrium. The patient underwent successful video-assisted thoracoscopic removal of the pericardial cyst under general anesthesia. The patient’s postoperative course was uneventful and he was discharged on postoperative day 1 in a stable condition. To our knowledge, this is the first report regarding the anesthetic management of a patient with a giant pericardial cyst undergoing thoracic surgery. Knowledge regarding the perioperative challenges associated with the removal of pericardial cysts can prevent complications and improve patient outcomes.


1991 ◽  
Vol 261 (4) ◽  
pp. L3-L7
Author(s):  
Cathy J. Beinlich ◽  
Kenneth M. Baker ◽  
Gloria J. White ◽  
Howard E. Morgan

The newborn heart is an excellent model in which to study cardiac growth because the neonatal period is a normal situation in which the left ventricle (LV) grows rapidly and the right ventricle grows slowly. Accelerated LV growth is in response to mechanical, neural, and endocrine changes at birth. Faster growth of the LV is accounted for by greater capacity for protein synthesis, as evidenced by greater RNA content. At 18 h of life, ribosomes are formed in preference to total heart protein, but at 48 h of life, faster rates of both ribosome formation and total protein synthesis are observed. In the LV of hearts from 2-day-old pigs, these rates are insensitive to the addition of glucagon, 1-methyl-3-isobutylxanthine, or a combination of norepinephrine and propranolol. These observations could result because of maximal growth stimulation already present in the LV of the newborn heart. To restrain LV growth in the neonatal period, we treated pigs with enalapril maleate, an angiotensin II-converting enzyme inhibitor. Enalapril blocked growth of the LV as well as the increase in RNA content. When hearts from enalapril-treated pigs were perfused in vitro, rates of protein synthesis and ribosome formation in the LV were lower. These studies suggest that angiotensin II is an important factor accounting for rapid growth of the neonatal heart in response to pressure overload at birth. adenosine 3',5'-cyclic monophosphate; angiotensin II; cardiac hypertrophy; glucagon; norepinephrine; protein synthesis; ribosome formation


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