Search

Palmoplantar keratoderma(PPK) refers to a group of heterogeneous disorders defined by thickening of skin on the palms and soles. Due to diversity of symptoms and involved organs, the term PPK does not describe each individual patient precisely. The syndrome is quite rare and unknown to the anesthesiologists, while serious associations such as difficult airway or cardiac disease are significant challenges during perioperative management. The literature lacks enough data on anesthetic considerations in these patients, implicating case reports as the only available sources. We revised the accessible information through description of our patient.

Background: The purpose of this randomized, single-center study was to prospectively investigate the impact of anesthetic techniques for craniotomy on the release of cytokines (interleukin (IL)-1, IL-6, tumor necrosis factor factor-alpha (TNF-α)) and to determine whether intravenous anesthesia compared to inhalational anesthesia attenuates the inflammatory response.Methods and Materials: The study enrolled 60 patients undergoing craniotomy, allocated into two equal groups to receive either Isoflurane (n=30) and Propofol (n=30). Non-invasive hemodynamic monitoring was used. Serum levels of IL-1, IL-6 and TNF-α were evaluated before and at the end of surgery and anesthesia.Results: Although there was a significant rise in serum level of inflammatory cytokine but compared with patients anaesthetized with Isoflurane, patients who received Propofol had significant lower levels of IL-1, IL-6 and TNF-a after surgery (p

Up until now, hydroxyethyl starch (HES) solutions have mainly been indicated as "volume expanders". Recent unfavorable reports of these solutions such as probability of kidney injury or inducing coagulopathy have relatively ejected these solutions in many centers, particularly in European countries. Despite several confirming studies, multiple anecdotal letters or reviews has challenged total disappearance of these colloids from medicine practice. Also, some novel observations emphasized the distinguished ability of HES to preserve the microvascular integrity even at the presence of an inflammatory process. Despite extreme statements has been published demanding immediate suspension of these fluids from the market, it seems logical to revise the most recent studies which may declare new pathways to approach HES family of solutions.

Churg-Strauss syndrome (CSS) or allergic granulomatosis is a necrotizing vasculitis with different presentations. In this case highlights the anesthetic management of a known case of CSS who underwent posterior spinal fixation surgery. Our patient fulfilled the ACR diagnosis criteria because of showing asthma, paranasal sinusitis, positive skin biopsy and eosinophilia.Before anesthesia induction, laryngeal nerves block by lidocaine was conducted. Fiber-optic bronchoscopy was preceded by inserting a No. 7.5 spiral cuffed endotracheal tube.

Osteogenesis Imperfecta (OI) is a genetic disorder with skeletal, vascular and soft tissue involvement with different clinical presentations. Since these patients need different surgical procedure in their lifetime, anesthesiologists are involved with their perioperative care and should be familiar with pathogenesis and anesthetic considerations of OI. Here we represent a case of OI which was candidate for Video Assisted Thoracic Surgery (VATS) and review the literature.

Introduction: Klippel feil syndrome (KFS) is a rare entity which is characterized by failure of normal segmentation of cervical vertebrae resulting in short neck with restricted movement and cervical instability. This anomaly increases the risk of neurological damage during airway instrumentation like laryngoscopy and positioning for surgery.Case Rports: We report three patients, a 42 year old male patient with KFS who scheduled for craniocervical fusion under general anesthesia and a 6 year old girl candidate for cleft palate repair and a 26 year old woman candidate for craniocervical fusion.Conclusion: All the cases were successfully managed using fiberoptic bronchoscope in first two cases and gum elastic bougie (GEB) in the last case. All things together, Awake fiberoptic intubation could be the safest technique for airway management.

Crouzon syndrome is a rare autosomal dominant premature cranyosynostosis, caused by fibroblast growth factor receptor 2 gene mutation on chromosome 10. The predominant skull and facial malformations with potential compromise airway make the crouzon syndrome a demanding issue for anesthesiologists and surgeons, required dynamic team work. In this report we describe a child, a known case of Crouzon syndrome who was a candidate for optic nerve decompression through endoscopic surgery. The anesthetic considerations and management are presented.Key words: Crouzon Syndrome, FGFR2 gene, Difficult Intubation, Anesthesia

Nieman-Pick disease type C is a rare, autosomal recessive, neurometabolic disorder associated with the accumulation of unesterified cholesterol in lysosomes and late endosomes. Because of multiple organ involvement and wide range of clinical manifestations, these patients will demand multiple diagnostic and therapeutic procedures requiring anesthesia. Sincepathogenesis of this disease is still unknown, further investigations on cellular and molecular basis of NPC is needed. In this report we present a known case of NPC1 requiring anesthesia for Percutaneous Endoscopic Gastrostomy and a brief review about molecular basis and recent advances in this field.

Wolfram syndrome was first described by physician D J Wolfram and Wagener in 1938. This autosomal recessive syndrome is also referred to as DIDMOAD syndrome which stands for Diabetes Insipidus, Insulin Dependent Diabetes Mellitus, Optic Atrophy and Deafness