Thyroglossal Duct Cyst Associated with Xanthogranulomatous Inflammation

Xanthogranulomatous inflammation (XGI) is an uncommon destructive chronic inflammatory process mainly occurring in the kidney and gallbladder, characterized by the accumulation of foamy histiocytes, multinucleated giant cells (Touton type), cholesterol clefts and chronic inflammatory cells. The head and neck region is an uncommon site for XGI. This type of inflammatory reaction has been defined in branchial cleft cyst, salivary gland tumors following fine-needle aspiration biopsies, Rathke’s cleft cyst in the pituitary gland, and colloid cyst in the 3rd ventricle. We present herein a unique case of ruptured thyroglossal duct cyst leading to XGI, characterized by an infiltrative subcutaneous central neck lesion, clinically mimicking a thyroid carcinoma. In addition, we also summarize current insights into the pathogenesis of XGI in the head and neck region.     

Rickets with Dorfman-Chanarin syndrome

BACKGROUND: Dorfman-Chanarin syndrome is a rare, autosomal recessive, inherited, lipid storage disease. It is characterized by nonbullous congenital ichthyosiform erythroderma, leukocyte vacuoles and variable involvement of the liver, muscles and central nervous system, due to errors of triacylglycerol metabolism. To date only 32 cases of this syndrome have been described worldwide. AIMS: To report the case of a boy with Dorfman-Chanarin syndrome with rickets. CASE REPORT: A boy of Turkish origin was born of a nonconsanguineous marriage after an uncomplicated perinatal period. On examination the patient had failure to thrive, diffuse erythroderma and ichthyosis and clinical features suggesting rickets. A light-microscopic examination of peripheral smear revealed vacuolated leukocytes typical of Jordans' anomaly. Lipid analysis showed an increase in triacylglycerol and very low density lipoprotein. A radiographic study of the wrist revealed rickets. CONCLUSION: The clinical progression in these patients ranges from mild to fatal. We believe that patients with ichthyosis should be evaluated bearing in mind the possibility of Dorfman-Chanarin syndrome. This may increase the number of reported Dorfman-Chanarin syndrome cases, and the pathogenesis and progression of the disease will become clearer.     

Neuromuscular effects of cisatracurium in morbidly obese patients

Obesity is associated with significant changes in body composition and function that may alter the pharmacodynamics and pharmacokinetics of various drugs. In this study, we investigated the neuromuscular effects of cisatracurium in morbidly obese as compared to control group of normal body weight patients. In the morbidly obese group (n = 20), corrected weight was used to calculate the drug doses. In the control group (n = 20), the dose was calculated on ideal body weight (IBW). 0.15 mg/kg(-1) cisatracurium was administered as the neuromuscular blocker. Neuromuscular effects were recorded at T0 (onset time), T1 (appearance of first stimulus of TOF), T25 (25% recovery of T1) and T25-75 (time of T25 to T75, recovery time). T0 was determined as 177 +/- 23 s and 168 +/- 19 s in the morbidly obese, and control group, respectively. T25 was determined as 46 +/- 7 min and 56 +/- 8 min, in the morbidly obese and control group, respectively (p < 0.05). T25-75 was determined as 11 +/- 5 min and 14 +/- 6 min in the morbidly obese and control group, respectively (p < 0.05). Intubation conditions were determined as good in 13, excellent in 7 patients in the morbidly obese group, and as good in 4 and excellent in 16 patients in the control group (p < 0.05). As different neuromuscular effects of cisatracurium were detected, we conclude that ne uromuscular agents must be monitored in the morbidly obese patients.     

Is radial shortening useful for Litchman stage 3B Kienbock’s disease?

Treatment of Litchman stage 3 Kienböck’s disease is still controversial. In this study our aim was to evaluate the effectiveness of radial shortening on stage 3B Kienböck’s disease in comparison with stage 3A cases. Radial shortening was performed for 23 patients who had stage 3A (group I, n = 13) and 3B (group II, n = 10) Kienböck’s disease between 1994 and 2004. The radial osteotomy was performed 4.5 cm proximal to the distal articular surface. The mean shortening was 2.6 mm (range 2 to 4.5). The average follow-up period was 85 months (range 26–147). Based on the modified Nakamura system, the mean clinical points were 14.3 in group I and 13.3 in group II. There was no statistical difference between both groups with regard to clinical points (P = 0.483). The extension-flexion arc showed significant improvement in both groups. Based on the results of this long-term follow-up study, we concluded that radial shortening osteotomy can be performed in the treatment of type 3B Kienböck’s disease as reliably as type 3A, despite the lack of evident radiological improvement.     

Neurological soft signs as the stroke risk in sickle cell disease

Sickle cell disease (SCD) is a common form of hemoglobinopathy and is highly prevalent worldwide. Silent cerebral infarction, which represents infarction without clinical signs, is a risk factor for clinical stroke in patients with SCD. It is well known that silent infarction predisposes patients with SCD to overt stroke. The aim of the present study is to investigate the effect of silent infarction on neurological soft signs (NSS), which demonstrate subtle impairments in sensory integration, motor coordination and the sequencing of complex motor acts and to evaluate whether NSS can be used in clinical practice to evaluate the patients at risk of stroke in SCD patients with silent infarction. Fifty-nine SCD patients without any documented history of cerebrovascular accident and 28 healthy controls were included in this study. All the patients with SCD were evaluated with cerebral magnetic resonance imaging. We found that the NSS scores were significantly higher in patients with silent cerebral infarction than those in patients without silent infarction and control subjects (p < 0.05). Importantly, there was no significant difference in the NSS scores between the patients without silent infarction and control subjects. These results indicate that high NSS scores represnt an important finding for diagnosis of silent infarction in SCD patients. As silent infarction increases the risk for stroke in patients with SCD, NSS can be used to provide additional information in diagnosis of the patients with possible stroke risk during the course of SCD.     

Meningitis due to Providencia stuartii

In this report, we present a case of postneurosurgical meningitis due to Providencia stuartii, which was treated successfully with meropenem therapy lasting 21 days.     

Sickle cell disease with regional silent cerebral infarction detected by SPECT

An 18-year-old boy was admitted to the hospital for a right leg ulcer, which appeared 1 year ago. After physical and hematologic examinations, he was referred for neurologic and cardiologic examination because his hemoglobin analysis and hematologic findings were interpreted as being consistent with sickle cell anemia. Although he had no neurologic symptoms, MRI and Tc-99m ethyl cysteinate dimer cerebral perfusion single photon emission computer tomography were performed to rule out a silent cerebral infarction. Changes secondary to an infarct were seen in the region of the caudate nucleus.     

The effect of dividing the sphincter of Oddi at endoscopic sphincterotomy on the filling and emptying aspects of function of the gallbladder

The effect of dividing the sphincter of Oddi at endoscopic sphincterotomy (EST) on the filling and emptying aspects of gallbladder function has not been definitely established in humans. This prospective study is designed to examine the effects of EST on gallbladder emptying. In 13 patients (8 men and 5 women; mean age, 60.54 years +/- 2.14; range, 45-75 years), postprandial gallbladder emptying was measured ultrasonographically before and after EST (within 1-4 days). The fasting volumes after 8 hours of fasting, at times 0 min and before the test meal was given, residual volume (the smallest postprandial volume), gallbladder ejection fraction (EF), and total ejection volume, at 10, 20, 30, 40, 50, 60, 70, 80, and 90 min after the test meal, were studied. The fasting gallbladder volume and the residual volume tended to decrease after EST, but the differences were not significant (40.21 +/- 10.79 mL and 35.48 +/- 11.21 mL, 17.79 +/- 4.83 mL, and 13.10 +/- 4.83 mL, respectively; p > 0.05). Maximum EF was found to be 19.72% at 40 min and increased to 28.62% at 70 min after EST. Although the difference was not statistically relevant, a trend of improvement was evident after EST. The ejected volumes after EST have depicted a trend toward increase, without reaching to any statistical significance (p > 0.05). Our results demonstrate at least no adverse effects of EST and further support some positive effects on gallbladder kinetics.     

Detachment of the Connecting Tube from the Port and Migration into Jejunal Wall

After laparoscopic adjustable gastric banding (LAGB), early recognition and treatment of uncommon complications are important. A 36-year-old man who had undergone LAGB presented at our clinic with weight gain of 14 kg during the prior 6 months. During investigation, detachment of the connecting tube from the port and migration of this tube into the jejunal wall were found. The detached port was replaced with a new port, and laparoscopically-assisted jejunorraphy was performed.