Susac Syndrome: Clinical characteristics, diagnostic findings and treatment in 19 cases.

Susac's Syndrome (SS), which was first described in 1979, is a rare and presumably autoimmune disorder characterized by encephalopathy, hearing loss, and visual disturbance resulting from branch retinal artery occlusion (BRAO). This study reports 19 SS patients' clinical characteristics, MRI features, CSF analysis, treatment strategies and outcomes. At initial presentation, only three of 19 patients demonstrated the complete clinical triad. Clinic presentation varied from isolated hemiparesis to the full triad (encephalopathy, hearing loss and visual disturbances). Corpus callosum (CC) involvement was noted in the MRI of 18 patients (97%) and BRAO was detected in 17 (95%) patients. All patients were treated with intravenous methylprednisolone after the initial assessment. This case series is presented to emphasize the differences in clinical presentation of SS and the importance of MRI and FFA in diagnosis.

Negative appendectomy experience in children.

BACKGROUND: The aim of this study was to discuss the findings of our patients who had negative appendectomy. PATIENTS AND METHODS: Hospital records of negative appendectomy for abdominal pain patients were evaluated retrospectively. RESULTS: Negative exploration for acute appendicitis (AP) was done in 149 patients. The most frequent complaints were abdominal pain, vomiting and fever. The commonly established diagnoses after negative exploration were gastroenteritis and urinary infections. However, the frequent pathologies observed during the operation were mesenteric lymphadenitis and Meckel's diverticulum. All patients with systemic disease such as Henoch-Schonlein's purpura operated for AP had it diagnosed during the postoperative course. Interestingly, two different types of worms were found in the lumen of the appendices. CONCLUSION: Despite new techniques, 100% correct diagnosis of AP is still a challenging problem. Furthermore, appendicitis is a deadly disease if not treated properly. Therefore, it is best to perform exploration without undue delay in cases with suspicious AP.

Neuropsychiatric manifestations in multiple sclerosis: correlation of fatigue and depression with disease progression.

Fatigue is one of the most common disabling symptoms in multiple sclerosis (MS) with significant impact on daily life. The aim of this study is to explore the association among MS fatigue, clinical disability and depression. Fifty-seven patients were assessed by fatigue severity scale (FSS), expanded disability status scale (EDSS) and Beck depression inventory (BDI). Mean FSS score was 4.1 +/- 1.6. Based on FSS scores, patients were divided into three groups: Patients with FSS score >5 (n=10, 32%) were evaluated to present with fatigue symptoms, patients with borderline fatigue (n=29, 50%) had an FSS score between 4 and 5 and patients with no fatigue (n=18, 18%) had an FSS<4. When the patients were compared according to the presence of fatigue symptoms, patients with fatigue had significantly higher EDSS scores (p=0.03). BDI evaluation revealed that 33 (57%) patients had a score > or =11. MS patients with fatigue showed significantly higher BDI scores when compared to patients without fatigue (p=0.0002). A significant relationship among fatigue, disease disability and depression was observed in our study, implying the complex interplay of fatigue and depression with disability.

Electrophysiological and clinical correlates of corpus callosum atrophy in patients with multiple sclerosis.

Multiple sclerosis (MS) is an idiopathic inflammatory demyelinating disorder of the central nervous system (CNS) characterized by demyelination and axonal degeneration. Corpus callosum (CC) is commonly involved during the disease process leading to atrophy (93%). Currently, there are no established markers of disease progression and the interplay of processes leading to brain atrophy in MS remains unknown. The primary aim of this study was to assess the frequency of CC atrophy in MS patients. Furthermore, the relationship between expanded disability status scale (EDSS) and transcranial magnetic stimulation (TMS) evoked motor potentials (MEP) were assessed to capture disease effects by independent parameters. Seventy-nine MS patients and 50 controls were included and their CC volumes were assessed. Out of 79 patients, 31 patients (39·2%) had CC atrophy. The distribution of EDSS scores among the group with CC atrophy [13 (32%) patients with EDSS 0-2; 11 (58%) patients with EDSS 2-4; 19 (24%) patients with EDSS ≥4] was not statistically significant (p>0·05). MEP latency was abnormal in 34 (43%) patients, 67 (85%) patients had abnormal MEP amplitude and CMCT was abnormal in 32 (41%) patients. The relation between EDSS and MEP was statistically significant among the patient population including the subgroup of patients with CC atrophy (p<0.05). Our results lacked to provide an association between disability and CC atrophy, but there was a correlation between CC atrophy and TMS evoked motor potentials. Early evolution of axonal degeneration and brain atrophy should be considered in terms of follow-up measures to provide long-term efficiency impacting disability, progression and brain atrophy.

Correlation between disability and transcranial magnetic stimulation abnormalities in patients with multiple sclerosis.

Multiple sclerosis (MS) is an idiopathic inflammatory demyelinating disorder of the central nervous system. Clinical evaluation, MRI, cerebrospinal fluid testing and evoked potentials (EP) are among the available methods utilized for disease diagnosis and monitoring. To date, no surrogate markers have been established to assess disease evolution and progression. The aim of this study is to assess motor evoked potentials (MEP) of MS patients by transcranial magnetic stimulation (TMS) and investigate the possible correlations between TMS abnormalities and disability in the patient group, which includes a subgroup with no apparent pyramidal tract dysfunction. A total of 131 clinically definite MS patients were included in the study. Motor responses to TMS stimulation were recorded. Absent values, decreases in amplitude, prolongation of latency and central motor conduction time (CMCT) were considered as abnormal. A total of 109 (83%) patients displayed abnormal MEP amplitude, 68 (52%) displayed MEP latency, and 64 (49%) displayed CMCT abnormalities. Abnormal CMCT, latency and amplitude results were correlated with Expanded Disability Status Scale scores (p<0.001). Our results indicate that TMS-EP in MS patients is correlated with disability, and that these findings may support the role of EPs in predicting disability even in subclinical presentations.

Ankyloglossia and effects on breast-feeding, speech problems and mechanical/social issues in children.

BACKGROUND: This aim of this study was to define the characteristics of the patients who underwent surgery for ankyloglossia. PATIENTS AND METHODS: The patients (n = 127) with ankyloglossia underwent surgery between 1987 and 2005. The patients were evaluated for age, gender, complaints, grade, and operative procedures. This study did not cover postoperative evaluation of the procedure. RESULTS: The ages of the children ranged from 20 days to 7 years, and 84% of them were under 1 year of age. Seventy-two percent were boys; 28% were girls. The most common complaint of the parents of infants under one year of age was breast-feeding (35/84). When the tongue movements of the patients were examined, 57 patients (of whom 18 were over one year of age) had limited tongue mobility. The mean frenulum length of the patients was grade 1 in 72 patients and grade 2 in 55 patients. Ankyloglossia was corrected by frenotomy. Three patients had bleeding from their frenotomy site which resolved with local pressure. General anaesthesia was preferred for 77 patients, and there was a need for suturing in 20 patients. CONCLUSION: The correction of ankyloglossia at an early age reduces the risk of latent complications. In addition, the early correction will mitigate the feeding- and speech-related concerns of parents and doctors alike.

Association of tumor necrosis factor, interleukin-6 and cyclooxygenase pathway with lipopolysaccharide-induced intussusception.

INTRODUCTION: Many factors and mechanisms have been proposed as causes for intussusception (IN); however, the etiology remains unclear. Inflammatory mediators such as tumor necrosis factor (TNF) and interleukin-6 (IL-6), which are elevated during infectious diseases, can significantly affect gastrointestinal motility. Motility changes caused by these agents might contribute to the development of IN. The aim of this experimental study was to determine the preventive effects of indomethacin on lipopolysaccharide (LPS)-induced IN in mice and to investigate the role of TNF and IL-6 on intussusception. MATERIALS AND METHODS: Seventy-eight mice were divided into five groups. In the Control group (n=6), no procedure was done. In the Sham group (n=6), 1 ml saline, in the Indomethacin group (n=6), 10 mg/kg of indomethacin, in the LPS group (n=30), 12 mg/kg of LPS was administered intraperitoneally (IP). In the Treatment group (n=30), 10 mg/kg of indomethacin was administered IP following 12 mg/kg of LPS. All animals were laparotomized 6 hours following IP injections. The existence of IN was noted and blood specimens were obtained. TNFalpha and IL-6 plasma level measurements were performed by standard ELISA for mice. The results were compared using the Mann-Whitney U test and one-way ANOVA test. A value of p<0.05 was considered significant. RESULTS: Five mice (1 in the control, 2 in the LPS, 2 in the Treatment group) were excluded from the study. IN was observed in 6 (20%) mice in the LPS group, whereas it was not found in any mice in the Treatment group. Mean TNFalpha and IL-6 levels were statistically higher in the LPS group (394.72+/-403.79; 195.18+/-218.37 pg/ml, respectively) compared to all other groups, including the Treatment group (p<0.05 for each comparison). Within the LPS group of mice, the levels were higher in animals with IN compared to the mice without IN. CONCLUSION: Increased TNFalpha and IL-6 levels induced by LPS correlated well with the occurrence of IN, and a decrease in these levels via cyclooxygenase (COX) inhibition by indomethacin prevented IN from forming in this experimental model.

Cefoperazone induced gastro-intestinal haemorrhage. A case report.

Cefoperazone is a parenteral third generation cephalosporin which is active against many Gram positive and Gram negative organisms. Cefaperazone, like other cephalosporins which contain methyltiotetrazole side chain, can cause hypoprotrombinaemia and bleeding problems. Here we report a nine-year old child with Meckel's diverticulum who had cefoperazone induced massive gastrointestinal bleeding on the fifth day following the operation.

Effect of melatonin on healing of colonic anastomosis in a rat model of peritonitis.

BACKGROUND: The aim of this study is to determine the effect of melatonin on intestinal anastomosis in the presence of peritonitis. MATERIAL AND METHODS: 32 Wistar albino rats were randomized into four groups (n = 8): A (sham), B (control), C (melatonin 5 mg/kg), and D (melatonin 10 mg/kg). In group A, only cecal dissection was carried out. In the other groups, cecal ligation and puncture (CLP) followed cecal dissection in order to induce bacterial peritonitis. 24 h after the previous operation, cecal resection and ileocolic anastomosis were performed in the rats of all groups. In group C (5 mg/kg) and group D (10 mg/kg), melatonin was injected for 5 consecutive days starting after CLP. At the 48th hour of the CLP procedure, blood was drawn via the tail vein for tumor necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6) analysis, and on the 4th day of the experiment relaparotomy was carried out for bursting pressure (BP) measurements. The intestinal tissue containing the anastomotic line was then snap-frozen in liquid nitrogen and stored at -80 degrees C for determination of tissue levels of malondialdehyde (MDA) and glutathione (GSH). RESULTS: The tissue MDA level, blood TNF-alpha and IL-6 levels of group B were significantly higher than in the other groups, whereas the BP results and GSH levels of group B were found to be significantly lower than in the other groups. The results of groups C and D are statistically different from those of group B. When we compared the results of groups C and D, we found significantly higher results in terms of BP and GSH levels in group D and also significantly lower results in terms of MDA, blood TNF-alpha and IL-6 levels in group D. CONCLUSION: The findings of this experiment suggest that melatonin has a dose-independent positive effect on wound healing of colonic anastomosis.

A child with a giant omental cyst.

A case of giant omental cyst diagnosed preoperatively is described. A 4-year old girl presenting with abdominal distension and misdiagnosed as having ascites, was subsequently proved to have a giant omental cyst. This is a rather difficult diagnosis to reach and results in wrong treatment methods. A high index of suspicion is therefore required early in the course of the disorder to decrease the complication rate. This report describes the diagnostic modalities and our approach to the patient who was cured by total excision of the cyst and free of disease in the fourth year of follow-up.

Sequential cytokine levels in the diagnosis of appendicitis.

OBJECTIVE: To determine the diagnostic value of sequential white blood cell count (WBC), tumor necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6) in patients with abdominal pain. MATERIAL AND METHODS: Tests were determined at hospital admission and 6 h later in 105 children. Patients who were non-operatively followed and had a normal histopathology were classified as the non-appendicitis (NA) group. Patients with symptoms consistent with appendicitis were classified as the appendicitis group (A). Data were analyzed as positive/negative predictive value, sensitivity, specificity and accuracy rate (AR). The global power of the variables in discriminating the patients between the two groups and advanced/simple appendicitis was assessed from the area under the receiver operating characteristic (ROC). RESULTS: Initial measurements showed WBC to be a valuable diagnostic tool in acute appendicitis (AR = 74 %), whereas IL-6 and TNF-alpha were found not to be valuable. The second measurements revealed higher values, and IL-6 reached its highest AR (89 %). When initial values were evaluated in combination, the highest AR of 73 % was observed with TNF-alpha + WBC. The highest AR (90 %) was seen with IL-6 + TNF-alpha in the second measurements. ROC analysis showed WBC to be the most valuable parameter of the three. The area under the curve (AUC) was 0.750 for the initial measurement and 0.779 for the repeat measurement of WBC (p = 0.001). The most useful diagnostic parameter in discriminating between the simple and the advanced cases was IL-6 as assessed with the ROC curve (p<0.01). CONCLUSIONS: WBC elevation in patients with suspected acute appendicitis is an important parameter supporting the diagnosis at initial admission, whereas IL-6 is a more valuable tool in diagnosing advanced appendicitis.

Surgical breast lesions in adolescent patients and a review of the literature.

BACKGROUND/PURPOSE: Breast disease in adolescence is uncommon, with most presenting lesions being benign. Breast lesions in adolescent patients are discussed in this article with a review of world literature. PATIENTS AND METHODS: The clinical records of 30 patients operated upon for breast lesions in our hospital during 18 years were reviewed. RESULTS: There were 25 female and five male patients, ranging in age from 16 years. Twenty-five female patients were operated on for breast mass and/or discharge, and five male adolescents were operated on for gynaecomastia. The most frequent (n = 27) complaint was palpation of mass in the breast. The most common histologies were fibro-adenoma (n = 14) and gynaecomastia (n = 5). The average duration of pre-operative symptoms was 2.9 months for fibro-adenoma, 1.6 years for gynaecomastia (extremes 2 days to 1 year). Nipple discharge was observed in three patients. Average diameters of palpable masses were 2.9 cm for fibro-adenomas and 5 cm for gynaecomastia. Lesions were excised surgically in all patients. Masses diagnosed as fibro-adenomas in the pre-operative period were reported upon pathological examination to be precancerous lesions such as cystosarcoma phylloiedes, juvenile fibro-adenoma, solitary intraductal papilloma, tubular adenoma and juvenile papilloma. One postoperative wound infection and one recurrence of fibro-adenoma was seen. CONCLUSION: The most frequently encountered breast masses were fibro-adenomas. These lesions are mostly benign in nature and can be treated conservatively but the possibility of precancerous lesion should be considered during follow-up.

Unusual localization of hydatid cyst.

We report a case of a single large echinococcal cyst that originated from the retroperitoneal space. Diagnosis was confirmed with serologic test and radiological examinations. Especially in the endemic areas hydatid cyst should be considered when evaluating cystic masses and it can be treated by extraperitoneal operation.

Treatment of Morgagni hernias by transabdominal approach / Tratamiento de las hernias de Morgagni mediante el abordaje transabdominal

BACKGROUND: Recently, the thoracic approach has been suggested in the surgical treatment of Morgagni hernias with some reported advantages over abdominal surgery. This manuscript reports the authors' experience with childhood Morgagni hernias repaired via laparotomy. SUBJECTS AND METHODS: Records of five cases of Morgagni hernias were evaluated with respect to age, presentation, operative data, complications, and outcome. RESULTS: The average age of four male and one female patients was 34 months (range 6 months to 8 years). Predominant presenting symptoms were related to the respiratory system. The diagnoses were made by posterior-anterior and lateral chest X-rays and confirmed by barium enema contrast radiographs. Associated anomalies were detected in three cases. All cases were managed by abdominal approach through upper midline incisions reducing the herniated viscera (transverse colon in all and including omentum in one patient) and excising the hernia sac which was present in all patients. The postoperative period was uneventful in this series and no recurrence was detected in an average of four years of follow-up. CONCLUSION: The transabdominal approach is appropriate in the surgical correction of paediatric Morgagni hernias.

Antecedentes: Recientemente, se ha sugerido el empleo del abordaje torácico en el tratamiento de las hernias de Morgagni, reportándose algunas ventajas del mismo sobre la cirugía abdominal. Este trabajo reporta la experiencia de los autores en relación con hernias de Morgagni en la infancia, reparadas mediante laparotomía. Sujetos y métodos: Las historias clínicas de cinco casos de hernias de Morgagni operadas en nuestro departamento, fueron evaluadas con respecto a edad, presentación, datos operatorios, complicaciones, y resultado. Resultados: La edad promedio de cuatro pacientes varones y una hembra fue de 34 meses (rango de 6 meses a 8 años). Los síntomas presentados estuvieron relacionados de forma predominante con el sistema respiratorio. Los diagnósticos se hicieron mediante rayos X posterior-anterior y lateral de tórax, y confirmados luego mediante estudio radiográfico de contraste con enema de bario. Se detectaron anomalías asociadas en tres casos. Todos los casos fueron manejados mediante abordaje abdominal a través de incisiones de la línea media superior, reduciendo así la víscera herniada (colon transversal en todos, incluyendo el omento en un paciente) y practicando la excisión del saco de la hernia, presente en todos los pacientes. El período post-operatorio no presento incidentes en esta serie, y no se detectó recurrencia durante el seguimiento, que duró un promedio de cuatro años. Conclusión: El abordaje transabdominal es apropiado en la corrección quirúrgica de las hernias pediátricas de Morgagni.

Omentum prevents intestinal adhesions to mesh graft in abdominal infections and serosal defects.

BACKGROUND: Many studies have evaluated the use of grafts in the reconstruction of abdominal wall defects. In this study, the effects resulting from the presence or absence of the omentum were evaluated in the setting of infection or serosal defects in the formation of adhesions in abdominal closures using mesh grafts. METHODS: For this study, 60 Wistar albino rats were divided into six groups. A circular 3.79-cm(2) fascioperitoneal defect was created. After group-specific procedures, defects were reconstructed using polypropylene mesh grafts. In group C (control group), only a mesh graft recontruction was performed, whereas group O (O for omentectomy) underwent an omentectomy plus mesh closure. In group SD (serosal defect group), the cecum was abrased with a brush before mesh closure. Group SDO underwent cecal abrasion plus an omentectomy. In group I (infection group), the intraabdominal space was filled with 1 ml of solution containing 100,000 colony-forming units (CFUs) of Escherichia coli per milliliter. Group IO received the same same amount of E. coli solution plus an omentectomy before mesh closure. After 28 days, the groups were evaluated by intraabdominal and blood cultures, grading of intraabdominal adhesions, graft-organ adhesions, proportion of adhesions to graft size, and histopathologic studies. The results were statistically evaluated using one-way variant analysis and Scheffe's and Fisher's definite chi-square tests. RESULTS: For the groups in which the greater omentum was preserved, intestinal adhesions to the graft surface were less frequently observed, especially in cases with intraabdominal infections and serosal defects (p < 0.05). CONCLUSIONS: Preservation of the greater omentum reduces the formation of intestinal adhesions, especially in cases with underlying infections and serosal defects in abdominal closures using mesh grafts. This could be beneficial in related clinical situations in lowering the rate of intestinal fistulas, erosions, and obstructions that can be attributed to the formation of adhesions.

Inhibin B levels in peripheral vein do not correlate with inhibin B levels in the spermatic vein in adolescents with varicocele.

This study was conducted to evaluate the inhibin B with FSH and LH levels on spermatogenesis in varicocele patients. The study group consisted of 10 adolescent with left idiopathic varicocele of grade II and III. Blood specimens were obtained from dilated spermatic vein and peripheral vein simultaneously. Peripheral samples were also collected from 7 healthy children as controls. Inhibin B was measured with ELISA inhibin B kits. FSH and LH were analyzed by radioimmunoassay techniques. The results were analyzed using Mann-Whitney U and Spearman's rank tests. A value of p < 0.05 was considered significant. Peripheral FSH, LH and inhibin B levels were the same in the study and control group (p > 0.05). Mean inbibin B levels of spermatic vein were significantly higher than the control and peripheral blood of the study groups (p < 0.05). FSH, LH, and inhibin B levels correlated poorly each other (p > 0.05). Preoperative serum inhibin B concentration could not reliably predict a response to varicocelectomy, but the increase in inhibin B levels after treatment might suggest an improvement in testicular function.

Treatment of Morgagni hernias by transabdominal approach.

BACKGROUND: Recently, the thoracic approach has been suggested in the surgical treatment of Morgagni hernias with some reported advantages over abdominal surgery. This manuscript reports the authors' experience with childhood Morgagni hernias repaired via laparotomy. SUBJECTS AND METHODS: Records of five cases of Morgagni hernias were evaluated with respect to age, presentation, operative data, complications, and outcome. RESULTS: The average age of four male and one female patients was 34 months (range 6 months to 8 years). Predominant presenting symptoms were related to the respiratory system. The diagnoses were made by posterior-anterior and lateral chest X-rays and confirmed by barium enema contrast radiographs. Associated anomalies were detected in three cases. All cases were managed by abdominal approach through upper midline incisions reducing the herniated viscera (transverse colon in all and including omentum in one patient) and excising the hernia sac which was present in all patients. The postoperative period was uneventful in this series and no recurrence was detected in an average of four years of follow-up. CONCLUSION: The transabdominal approach is appropriate in the surgical correction of paediatric Morgagni hernias.

Postoperative intussusception in children.

Postoperative intussusception (POI) is an uncommon cause of postoperative mechanical bowel obstruction in children. Four cases of POI during a period of 15 years (1987-2001) were analysed retrospectively. Symptoms developed after a median period of 2.5 days following the operation. All cases were succesfully treated with operative manual reduction. POI occurs after a wide variety of surgical procedures and is often difficult to diagnose because the symptoms are often obscure. As a conclusion, we state that reaching a diagnose requires a high index of suspicion.

Massive ovarian oedema in adolescents.

Massive ovarian oedema is an unusual clinical entity occurring in teenage girls. It is a rare tumour-like condition of the ovary characterized by marked enlargement of one or both ovaries due to marked accumulation of oedema fluid in the ovarian stroma. Massive ovarian oedema may result from partial or intermittent torsion of the mesovary, interfering with venous and lymphatic drainage, but not with arterial blood flow. The patients most commonly present with lower abdominal pain and enlargement. This entity can clinically mimic a neoplasia. We report three cases with massive ovarian oedema.

Pancreas pseudocyst associated with L-asparaginase treatment: a case report.

A major complication of L-asparaginase used in the treatment of paediatric malignancies in children is pancreatitis (2%-16%). However, only seven paediatric cases of pancreatic pseudocyst caused by the utilization of the agent have been reported in literature. We present the case of a 5-year old girl who had abdominal pain and epigastric dullness after the third course of BMF-95 protocol with a diagnosis of ALL. A pancreatic pseudocyst of 10 x 10 cm size was found by abdominal tomography. The cyst was treated by percutaneous external drainage, total parenteral nutrition (TPN), administration of octreotide and antibiotherapy for one month. Percutaneous external drainage has proven to be an effective, noninvasive method in this special case with a systemic disorder and the high risk of mortality should a surgical intervention have been performed.