Osteochondrodysplasia is a heterogeneous group of disorders appearing short limbed dwarfism. Because many of these entities are lethal and hereditary, an accurate diagnosis is mandatory. The purpose of this study is to define the clinicopathologic features and radiologic findings of osteochondrodysplasia. We reviewed 29 autopsy cases of congenital short limbed dwarfism, consisting of thanatophoric dysplasia (TD) (12 cases), osteogenesis imperfecta (OI) (12 cases), asphyxiating thoracic dysplasia (ATD) (3 cases), short-rib-polydactyly syndrome (SRPS) (1 case) and hypochondrogenesis (1 case). The gestational age ranged from 16 to 41 weeks. Of 6 fetuses that were born alive, 3 were ATD, 2 were TD and 1 was hypochondrogenesis. TD was frequently complicated by hydramnios. Of 8 cases studied chromosomally, only 1 showed chromosomal abnormality -46XY, inv 9. Intrauterine growth retardation was frequently associated with OI. Pulmonary hypoplasia was present in 23 cases (79%), including all cases of ATD, SRPS and hypochondrogenesis, 11 in TD and 7 in OI. Other associated anomalies were present in 17 cases (59%).
Autopsy* ; Chromosome Aberrations ; Diagnosis ; Dwarfism ; Extremities ; Fetal Growth Retardation ; Fetus ; Gestational Age ; Osteochondrodysplasias* ; Osteogenesis Imperfecta ; Polyhydramnios ; Thanatophoric Dysplasia

BACKGROUND: Hemodilution may increase cerebral blood flow (CBF) but the mechanism(s) remain controversal. Autoregulation is easily modified or disturbed by several conditions. The aim of this study was to evaluate the effects of isovolemic hemodilution on the autoregulation of cerebral blood flow in a rabbit model. METHODS: Stepwise hemodilution was accomplished by incrementally removing whole blood from the animals in amounts of 8-12 ml and replacing this with an equal volume of 6% hetastarch in saline. This procedure was continued until the target content values of approximately Hct -18% were achieved. To evaluate the influence of pressure changes on CBF, mean arterial pressure (MAP) was increased from a baseline pressure (approximately 78 mmHg) to 145 mmHg by infusing methoxamine, and cerebral blood flow was measured at each MAP level using the hydrogen clearence method after MAP had been stabilized for 15 min. RESULTS: Stepwise hespen replacement caused a sudden drop of Hct from 37.4% to 18.5% and a simultaneously a significant increase in local CBF of 161% in the hemodilution group. Hemodilution significantly reduced CaO2 in the hemodilution group (9.45 +/- 1.7 ml O2/dl) versus the control group (18.34 +/- 1.3 ml O2/dl). However, despite these decrease in CaO2, calculated cerebral oxygen delivery (DO2) was as well maintained in the hemodilution group (22.47 +/- 7.28 ml O2/100 gm/min) as in the control group (24.14 +/- 8.67 ml O2/100 gm/min). MAP increases from 78 mmHg to 145 mmHg produced a significant increase in CBF from 122.4 +/- 32.8 ml/100 gm/min to 170.9 +/- 23.7 ml/100 gm/min in control group (39.6%) and from 218.4 +/- 75.6 ml/100 gm/min to 268.4 +/- 106.5 ml/100 gm/min in the hemodilution group (44.6%) (P<0.001). These CBF increases were not significantly different in the two groups. CONCLUSIONS: The present study demonstrates that in the normal brain the decrease in CaO2 caused by hemodilution is well compensated for by an increase CBF, and that oxygen transport to the brain is also well maintained during at a Hct value of 20%. Although the present study did not show the tight CBF control within the MAP range from 78 mmHg to 145 mmHg, hemodilution did not alter the response of the cerebral circulation to increased MAP.
Animals ; Arterial Pressure ; Brain ; Hemodilution* ; Homeostasis* ; Hydrogen ; Hydroxyethyl Starch Derivatives ; Methoxamine ; Oxygen

Pneumothorax and pneumomediastinum can occur spontaneously, secondary to trauma, or from dissection of air from the neck or retroperitoneal space. The most common cause of traumatic pneumomediastinum is a rupture of the esophagus, which can occur during an episode of severe vomiting or, less frequently, following esophageal instrumention. We experienced a case of pneumothorax and pneumomediastinum, developed after esophageal perforation by stylet during difficult endotracheal intubation even though an esophagogram did not reveal the perforation site.
Esophageal Perforation* ; Esophagus ; Intubation, Intratracheal* ; Mediastinal Emphysema* ; Neck ; Pneumothorax* ; Retroperitoneal Space ; Rupture ; Vomiting

Awake craniotomy is indicated for tumor resection involving eloquent cortex. It allows the operator to perform appropriate cortical mapping during surgery and facilitate maximum tumor resection while minimizing neurologic deficit. Therefore anesthesia should provide adequate analgesia and sedation but also importantly a full consciousness and cooperation for neurologic testing. This case reports the use of target-controlled infusion (TCI) and monitoring of sedation and anesthetic depth through bispectral index (BIS), providing good control of sedation and analgesia to meet frequent changes throughout the different levels of the procedure while maintaining good condition for intraoperative brain mapping. We propose that TCI of propofol and remifentanil in combination may be a useful alternative for awake craniotomy requiring intraoperative brain mapping surgery.
Analgesia ; Anesthesia ; Brain Mapping ; Conscious Sedation ; Consciousness ; Craniotomy ; Neurologic Manifestations ; Piperidines ; Propofol

We report a case of the accidental intravenous administration of a large dose of magnesium sulfate during cesarean section. A 41-year-old woman, at 33 weeks gestation, with pregnancy-aggravated hypertension, headache and generalized edema presented in acute labor and showed fetal bradycardia on a nonstress test. Laboratory tests demonstrated an increased level of magnesium (5.4 mg/dl). A cesarean section was performed under general anesthesia with O2-N2O-enflurane and vecuronium. After delivery 2,000 mg of magnesium sulfate was mixed with the lactated Ringer's solution 1,000 ml and 550 ml administered to the patient. After noticing the accidental infusion of the magnesium sulfate, we replaced the lactated Ringer's solution with normal saline 1,000 ml and performed arterial blood gas analysis, checked serum electrolyte, including Mg2+ and Ca2+, and had monitored depth of muscle relaxation and vital signs. The level of magnesium had increased to 8.9 mg/dl after the accidental magnesium infusion. For about one and half hours after emergence from general anesthesia, she complained of dyspnea and paraparesis of extremities. To treat the hypermagnesemia, 3% calcium gluconate 1,000 mg and furocemide were given intravenously to antagonize magnesium and to increase the urine output. The depth of neuromuscular block was frequently monitored using a nerve stimulator. After conservative treatment, she recovered from the effect of the hypermagnesemia and was discharged on the fifth postoperative day. Anesthesiologists must to keep in mind the preoperative patients' pathophysiologic conditions, check co-administered drugs and the contents of intravenously connected solutions.
Administration, Intravenous* ; Adult ; Anesthesia, General ; Blood Gas Analysis ; Bradycardia ; Calcium Gluconate ; Cesarean Section* ; Dyspnea ; Edema ; Extremities ; Female ; Headache ; Humans ; Hypertension ; Magnesium Sulfate* ; Magnesium* ; Muscle Relaxation ; Neuromuscular Blockade ; Paraparesis ; Pre-Eclampsia ; Pregnancy ; Vecuronium Bromide ; Vital Signs

BACKGROUND: It is difficult to manage the symptoms of neuropathic pain, especially alloynia. The mechanism of the induction and maintenance of mechanical allodynia has been extensively researched for several decades. N-methyl-D-aspartate (NMDA) receptor antagonists are known to reduce mechanical allodynia. Recently, the role of prostaglandins in spinal nociceptive processing has been the focus of attention. Therefore, the present study was designed to investigate the effect of a combination of ketamine, a non-competitive NMDA antagonist, and of ketorolac, non-selective cyclooxygenase (COX) inhibitor on mechanical allodynia. METHODS: Male SD rats were prepared by tightly ligating the left L5 and L6 spinal nerves. All rats developed mechanical allodynia 7 days after surgery. N group (control, n = 6) received 5 ml of 0.9% normal saline intraperitoneally. K group (n = 6) received ketamine 1 mg/kg. T group received ketorolac 30 mg/kg, and KT group received ketamine 1 mg/kg and ketorolac 30 mg/kg simultaneously. Paw withdrawal thresholds to von Frey hairs were measured before and at 15 min, 30 min, 60 min and 120 min after drug administration. RESULTS: Normal saline and ketamine 1 mg/kg did not increase the paw withdrawal threshold from baseline. Ketorolac 30 mg/kg increased the paw withdrawal threshold only at 120 min after intraperitoneal injection. However, the co-administration of ketamine 1 mg/kg and ketorolac 30 mg/kg increased the paw withdrawal threshold significantly from baseline for 120 min. CONCLUSIONS: Intraperitoneal injection of ketamine and ketorolac attenuated the mechanical allodynia developed by spinal nerve ligation. Therefore, we suggest that combination of ketamine and ketorolac might be useful for the management of neuropathic pain.
Animals ; Hair ; Humans ; Hyperalgesia* ; Injections, Intraperitoneal* ; Ketamine* ; Ketorolac* ; Ligation* ; Male ; N-Methylaspartate ; Neuralgia ; Prostaglandin-Endoperoxide Synthases ; Prostaglandins ; Rats* ; Spinal Nerves*

BACKGROUND: Neuropathic pain is resistant to conventional treatments, and may seriously affect the quality of life. Insufficient has been done on drug combination for the treatment of neuropathic pain. So we undertook to determine the effect of ketamine and steroid on mechanical hyperalgesia in rats with spinal nerve ligation. METHODS: Rats were administerend L5 and L6 spinal nerve ligation to cause mechanical hyperalgesia. Control group (n = 6) were administered normal saline 5 ml intraperitoneally, the ketamine group (n = 6) ketamine 1mg/kg, and the steroid group (n = 6) ketamine 1 mg/kg and methylprednisolone 10 mg/kg. Mechanical hyperalgesia was assessed using a von Frey filament before injection, and then 15, 30, 60, 120, and 180 min after injection. RESULTS: Ketamine 1 mg/kg significantly attenuated mechanical hyperalgesia for 60 min. The combination of ketamine 1 mg/kg and methylprednisolone 10 mg/kg also significantly attenuated mechanical hyperalgesia. But the combination group did not exert a superior attenuating effect than ketamine alone. CONCLUSIONS: The combination of ketamine and metylprednisolone did not exert a superior antinociceptive effect than ketamine alone in rats with spinal nerve ligation.
Animals ; Hyperalgesia* ; Ketamine ; Ligation* ; Methylprednisolone ; Models, Animal* ; Neuralgia* ; Quality of Life ; Rats* ; Spinal Nerves

PURPOSE: We investigated the results of the third generation alumina on alumina bearing total hip arthroplasty in patients under the age of forty. MATERIALS AND METHODS: Fifty patients (57 hips) under the age of forty, who had uncemented total hip arthroplasty using third generation alumina bearing, between April 2000 and March 2006 were observed. The group consisted of 24 men and 26 women. The average age at the operation was 30.3 years (18-39 years). The average follow up period was 8.0 years (5-11 years). The most common cause for surgery was rheumatoid arthritis. We assessed the clinical and radiological results and postoperative complications. RESULTS: The mean Harris hip score at the last follow-up was 96 points on average. There was no inguinal pain. However, there were two hips of which the patient was experiencing thigh pain. One hip with squeaking was observed. We could observe the stable bony fixation of implants in all hips. There were no aseptic loosening and no osteolysis around the implants. Postoperative complications included one hip with nonunion of trochanteric osteotomy and one hip with dislocation. There were no ceramic fractures, no postoperative infections and no revisions. CONCLUSION: We observed the favorable clinical and radiographic outcomes of the third generation alumina on alumina total hip arthroplasty in patients under the age of forty. However, in the case of squeaking, a longer term follow-up is needed.
Aluminum Oxide ; Arthritis, Rheumatoid ; Arthroplasty ; Arthroplasty, Replacement, Hip ; Ceramics ; Dislocations ; Female ; Femur ; Follow-Up Studies ; Hip ; Humans ; Male ; Osteolysis ; Osteotomy ; Postoperative Complications ; Thigh ; Ursidae

BACKGROUND: Under normal conditions, cerebral blood flow is regulated as cerebral perfusion pressure changes (autoregulation). Inflammatory conditions like bacterial meiningitis result in a loss of cerebral autoregulation several hours after exposure to bacterial endotoxin. Endotoxin appears to produce effects via the production of reactive oxygen species, such as the superoxide anion. METHODS: Cerebral blood flow (CBF) was measured by hydrogen clearance in anesthetized rats 30 minutes after intravenous endotoxin 5 mg/kg or normal saline (control). Mean arterial pressure was reduced from 100 mmHg to 80 mmHg and 60 mmHg by hemorragic hypotension, and cerebral blood flow was measured at each pressure. RESULTS: In the control group, CBF did not change when arterial pressure was reduced to 80 mmHg (113.9 ml vs 111.9 ml), but declined significantly at 60 mmHg (113.9 ml vs 88.4 ml). In the group treated with endotoxin, both a reduction of mean arterial pressure to 80 mmHg (129.8 ml vs 101.8 ml) and 60 mmHg (129.8 ml vs 78.4 ml) caused a significant reduction in CBF, indicating that autoregualtion had been abolished. CONCLUSIONS: The results of this study indicate that within minutes of endotoxin exposure, CBF markedly increased, and that the autoregualtion of CBF was inhibited.
Animals ; Arterial Pressure ; Homeostasis* ; Hydrogen ; Hypotension ; Meningitis ; Perfusion ; Rats ; Reactive Oxygen Species ; Superoxides

No abstract available.
Cri-du-Chat Syndrome* ; Humans