Emergency and prolonged use of intravenous etomidate to control hypercortisolemia in a patient with Cushing's syndrome and peritonitis

We report the emergency and prolonged use of etomidate to control circulating cortisol levels in a patient with Cushing's syndrome secondary to ectopic ACTH production from a pancreatic islet cell tumor. Duodenal perforation and peritonitis complicated an episode of salmonella septicemia, precluding the use of conventional oral medical adrenolytic therapy. Endogenous cortisol secretion was abolished by parenteral etomidate, allowing serum cortisol levels to be controlled with an iv infusion of hydrocortisone over an 8-week period in intensive care before definitive pancreatic surgery.     

Laparoscopic adrenalectomy in a patient with Cushing syndrome

We present a female child with phenotypical and clinical features of the axial mesodermal dysplasia complex. Typical manifestations of both the Goldenhar syndrome and the caudal regression syndrome are present in this complex. Only a few reports have described patients with this pattern of malformations localized in both the cranial and caudal regions. Our case represents a mild form of the complex and may contribute to a better delineation of this condition.     

Capsular tension ring in a patient with Weill-Marchesani syndrome

Weill-Marchesani patients with cataractous lenses may presnet a surgical challenge in the presence of zonular weakness and microsherophakia. A 52-year-old Weill-Marchesani patient developed zonular dehiscence during capsule contraction after cataract extraction in her right eye. Use of a poly(methyl methacrylate) capsular tension ring in the second eye facilitated lens removal and intraocular lens placement. Postoperative results suggest the capsular tension ring provides long-term zonular stabilization by maintaining an internal force against the capsule.     

Warfarin resistance in a patient with short bowel syndrome

Drug therapy in short bowel syndrome can be complicated by inadequate or incomplete absorption of drugs in the small intestine. Many case reports claim that warfarin absorption is not affected by the syndrome. We treated a patient with oral warfarin for recurring deep vein thrombosis; up to 20 mg/day was administered with no increase in the international normalized ratio. Drug-drug interactions that may prevent absorption, increase metabolism, or antagonize the effects of warfarin were ruled out. Intravenous lipid administration, which is anecdotally reported to precipitate warfarin resistance, may have contributed to the condition, but dosing was less frequent than in published reports. The most probable explanation of warfarin resistance is the reduced surface area for drug absorption secondary to surgical removal of the patient's duodenum and gastrojejunostomy.     

Intestinal lymphangiectasia in a patient with Zellweger cerebrohepatorenal syndrome

OBJECTIVE: To assess the natural history and response to treatment of shoulder disorders in a community elderly population. METHOD: This community study of shoulder pain in the elderly reviewed patients three years after an earlier prevalence study. RESULTS: One hundred and eight of the original 136 patients with an identifiable shoulder disorder were available for reexamination. Eighty (74%) had persisting signs on examination, with persistent symptoms leading to impairment of personal care (21%) and household tasks (27%), and pain on movement (34%). There was no difference between treated and non-treated groups in terms of outcome. CONCLUSIONS: The results of this study serve to confirm and highlight the chronicity of shoulder lesions in this age group and the consequent personal suffering and implications for health care.     

Renal cell carcinoma in a patient with Beckwith-Wiedemann syndrome

We report the case of a patient with Beckwith-Wiedemann syndrome (BWS) who developed renal cell carcinoma (RCC). At birth, this patient presented with macroglossia, diastasis recti, mild gigantism, hepatomegaly and hypoglycemia, and the diagnosis of BWS was made. At 22 months, an intrapelvic rhabdomyosarcoma was detected and resected. At 37 months, computed tomography (CT) demonstrated a small mass with high attenuation in the right kidney, which was surgically confirmed to be RCC.     

Disseminated strongyloidiasis in a patient with acquired immunodeficiency syndrome

Seven patients developed recurrent hemarthroses following total knee arthroplasty. The average interval between arthroplasty and the first bleed was more than 20 months (range, 1-30 months). All seven required open synovectomy an average of 21 months (6-31 months) after arthroplasty. Follow-up evaluation averaged 44 months (19-60 months) and all had an excellent result without further bleeds. A prolific synovitis was seen in all cases, with histologic features revealing a chronic synovitis with fibrosis and hemosiderin staining. Entrapment of the proliferative synovial tissue between the components is postulated to be the etiology for these recurrent bleeds which averaged four per patient prior to synovectomy.     

Anesthetic management of a patient with Bernard-Soulier syndrome

Bernard-Soulier syndrome (BSS) is a hereditary hemorrhagic disease characterized by prolonged bleeding time due to abnormal platelet aggregation and giant platelets. Transfusion of platelet-rich plasma is the only treatment available for the hemorrhagic episodes in patients with this disease. A 20-year-old female with BBS was scheduled for sagittal osteotomy of the mandibular rami under general anesthesia. Anesthesia was induced with fentanyl and diazepam, and was maintained with nitrous oxide, fentanyl, and 0.5% enflurane. No exacerbation of the bleeding tendency was observed during or after the surgery. We consider that the use of halothane should be avoided in patients with BBS because it may inhibit the aggregation of platelets and prolong the bleeding time.     

Peripartum management of a patient with Isaacs' syndrome

PURPOSE: To describe the peripartum management of a patient with Isaacs' syndrome with specific reference to the anaesthetic implications of the disease process. Associated medical problems included obesity, pregnancy induced hypertension and a difficult airway. CLINICAL FEATURES: This 30-yr-old gravida V para 0 woman presented to the anaesthesia consultation clinic at 37-wk gestation to discuss pain relief options for labour and delivery. She had a history of Isaacs' syndrome (a peripheral motor neuron disorder), congenital heart disease (ASD and VSD), treated Hashimotos thyroiditis, obesity and a family history of haemachromatosis. On the day of consultation, she was hypertensive and peripheral oedema was noted. Her urine showed trace protein. Four days later, she presented to the labour suite and her cervix was 9 cm dilated. An epidural anaesthetic was given without difficulty and she had an uneventful labour and delivery course. There were no subsequent neurological complications. CONCLUSION: Isaacs' syndrome is an extremely rare peripheral motor neuron disorder. This patient was successfully managed with epidural analgesia for labour and delivered a healthy child with no congenital anomalies.     

Anesthetic management of a patient with Cowden syndrome

Cowden syndrome is a rare syndrome of chromosome abnormalities presenting with polyposis of digestive tracts, characteristic skin eruption and neuromuscular disorders. A 56-year-old male patient with Cowden syndrome underwent upper abdominal surgery under general anesthesia followed by post-operative epidural analgesia with buprenorphine. Proposed total gastrectomy was not performed because of massive invasion of carcinoma in the abdominal cavity and gastrojejunostomy was done instead. The anesthesia was satisfactory with inhalation of nitrous oxide and enflurane with intravenous vecuronium. Neuromuscular monitoring with electric twitch-responses of the hand showed normal patterns throughout the anesthesia. The recovery from anesthesia and neuromuscular blockade was prompt. Intermittent epidural buprenorphine, twice a day (0.2 mg of buprenorphine in 9 ml of normal saline for one time) was started just after the recovery of anesthesia and continued for four days. Delirium occurred two days after beginning epidural buprenorphine and disappeared three days after its discontinuation. The patient died 52 days after the operation from obstructive jaundice and sepsis. The delirium, therefore, seems to have been caused by buprenorphine possibly due to its impaired metabolism by the liver. Although we did not experience any abnormal neuromuscular reactions to vecuronium or anesthetic agents, it is important to perform preoperative neuromuscular examinations and peri-operative monitoring in the anesthetic management of a patient with this syndrome.     

Epididymocutaneous fistula in a patient with the acquired immunodeficiency syndrome and Marfan's syndrome

Epididymocutaneous fistula is a rare entity. A recent case in a patient with the acquired immunodeficiency syndrome and Marfan's syndrome led to this review. The patient's immunocompromised status as well as his past medical history necessitated special considerations in the diagnosis and management of his epididymocutaneous fistula.     

Kleine-Levin syndrome in a female patient

The concept of bone conduction hearing is old. By the 16th century the conduction of sound by a rod or the staff of a spear was reported by a number of writers; however, these writers considered these phenomena as a curiosity rather than having practical value. In the 17th century, John Bulwer and George Sibscota, both interested in the deaf and their education, applied the bone conduction phenomenon as an aid to defective hearing. Soon, independent reports from Germany, France, and Italy also described bone conduction rod devices as aids to impaired hearing. In 1879, the Audiphone, a hearing fan that operated by bone conduction, was patented. The invention of the Audiphone triggered the development and sale of a number of similar devices that had considerable popularity until the invention of the carbon-electric hearing aid in the early 1900s.