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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

Background: There are a number of adjuvants to be used for local anesthetics in spinal block. The aim of this study was to demonstrate the possible effect of intrathecal midazolam compared with bupivacaine as adjuvants in spinal anesthesia with bupivacaine in chronic opium abuses. Methods: In a double blind, randomized clinical trial, 90 opium abuser patients undergoing lower limb orthopedic surgery were selected and randomly assigned into 3 groups (30 cases each). The patients received 15 mg plain bupivacaine, or 15 mg bupivacaine plus 25 mcg fentanyl or 15 mg bupivacaine plus 1 mg midazolam, intrathecally. Results: The duration of anesthesia was much longer in the bupivacaine-midazolam group than the bupivacainefentanyl group; both were longer than the plain bupivacaine group (P < 0.05). Conclusions: Subarachnoid injection of adjuvant midazolam or fentanyl with plain 0.5% bupivacaine in opium abusers in lower limb orthopedic surgery increases the duration of sensory block. Therefore midazolam is more effective than fentanyl in such cases. (Korean J Anesthesiol 2012; 63: 521-526)

Background Anesthesia today has strived to decrease labor pain in a tolerable and controllable fashion. Intrathecal midazolam has been introduced as an adjunct to analgesics. The study was planned to assess the efficacy, safety and duration of analgesia produced by intrathecal midazolam adjunct to sufentanil in decreasing labor pain. Methods In a randomized clinical trial 80 parturient included in the study. The two groups were matched for age, cervical dilation, gravid, gestational age, and other demographic characteristics. Combination of sufentanil and midazolam administered intrathecally to experimental group and compared to sufentanil group. Time to reach maximum block, and pain score was measured and recorded. Results Groups were matched for age and weight and other demographic characteristic. No significant adverse effect was seen in both groups including decrease in Apgar score. Duration of analgesia was 92.0 ± 12.7 in sufentanil group and 185.2 ± 15.2 minutes in midazolam and sufentanil group which was significantly different (P = 0.002). Numeric rating scale score was significantly lower in midazolam group compare to sufentanil group at 120 min (P = 0.01), 150 min (P = 0.0014), and 180 min (P = 0.001). Conclusions Intrathecal midazolam as an adjunct to opioid could significantly enhance analgesia in labor pain with no significant adverse effect. Intrathecal injection of midazolam is an appropriate alternative to parenteral or epidural analgesia in small hospital settings.