Endoscopic surgery for fibrous dysplasia of the sinonasal tract in pediatric patients

Fibrous dysplasia (FD) is a non-neoplastic, expansile lesion of unknown origin. In about one-fourth of cases this disorder affects the head and neck area, where the mandible and maxilla are the most frequently involved sites. Its localization to the ethmoid is a rare event. Since the disease slowly progresses, its management is delayed until significant clinical symptoms or non-well-tolerated aesthetic deformities are present. When required, surgery is the treatment of choice. Several external procedures have been used to manage the lesion, but recently, more conservative transnasal approaches have been proposed. We report the history of a 6-year-old boy with fibrous dysplasia of the ethmoid labyrinth that underwent successful transnasal endoscopic removal. Furthermore, an analysis of the literature is presented with particular emphasis on clinical picture, diagnosis, and treatment of this rare illness.     

Management of septic shock and acquired respiratory distress syndrome in pediatric cancer patients

Septic shock occurs in 6 % of paediatric cancer patients with neutropenia and fever. The mortality of the septic shock is 40 % in BMT patients and 5 % in others. One third of paediatric ARDS cases affect immunocompromised individuals with a total mortality of 45 % and 80 % after BMT. Septic shock is caused by gram-negative bacteria in more than 75 %. ARDS is due to pneumonia in more than 50 %, sepsis in about 25 %. This article provides the recommendations of the Infectious Diseases Working Party of the German Society for Pediatric Infectious Diseases (DGPI) and the German Society for Pediatric Hematology/Oncology (GPOH) for treatment of septic shock and ARDS. Therapy of septic shock includes early antibiotic therapy and volume expansion (> or = 40 ml/kg initially). Refractory shock requires vasopressors (noradrenaline), followed by a judicious circulatory management. Hydrocortisone is indicated in patients with high probability of adrenal insufficiency. Mainstay of ARDS therapy is ventilation with sufficient end-expiratory pressure (PEEP) to prevent loss of functional residual capacity and with limited tidal volumes (< or = 6 ml/kg) and limited inspiratory pressure (< 35 cm H(2)O) respectively, to minimize ventilator induced lung injury. Volume therapy consists of maintenance of sufficient preload to counteract the impaired venous return, induced by positive pressure ventilation. Diuretics and eventually veno-venous haemofiltration are used to reduce free lung water. Surfactant application may be considered in severe cases. Steroids are indicated in pneumocystis carinii pneumonia and in engraftment pneumonitis.     

Significance of CA 125 serum level in discrimination between benign and malignant masses in the pelvis

Aim Our aim was to confirm that preoperative CA 125 serum level can be useful for discrimination between benign and malignant masses in the pelvis.Methods Preoperative CA 125 serum level was analyzed retrospectively in 121 patients who had surgery because of a malignant ovarian tumor and in 91 patients with benign masses in the pelvis. The cutoff serum level CA 125 between benign and malignant masses in the pelvis was 35 and 65 IU/ml.Results Of those patients with a malignant ovarian tumor, 65.3% had menopause whereas only 31.5% of those with a benign tumor did so. The average age of the patients with a malignant tumor was 54.2 years and of those with a benign tumor 46.8 years. The preoperative CA 125 serum level was higher than 35 IU/ml in 80.2% and higher than 65 IU/ml in 72.7% of all analyzed patients with a malignant tumor, whereas it was 23.9% and 9.8% respectively in patients with a benign mass. In early stage ovarian cancer disease (borderline stage, I/II) the preoperative CA 125 serum level was higher than 35 IU/ml in 67.8% and in 52.5% higher than 65 IU/ml. In advanced stages (III/IV), it was higher than 35 and 65 IU/ml in 96.1%. After therapy the CA 125 serum level dropped below 35 IU/ml in 70.8% and after three chemotherapy courses in 78.1%. A CA 125 level less than 35 IU/ml was achieved by therapy in 84.2% patients with an early stage disease (I/II) and in 62.1% in advanced stages (III/IV). The calculated sensitivity was 80.2% and negative 74.5% (CA 125 higher than 35 IU/ml) and 72.7%, 90.2%, 90.7%, 71.6% respectively (CA 125 higher than 65 IU/ml).Conclusion Preoperative determination of CA 125 is a very useful method to discriminate between benign and malignant masses in the pelvis.     

Interplast in India. Review of 14 years

Interplast teams conducted two-week (or less) camps in India over a 14 year period. The majority of the teams were mixed and consisted of German and Dutch plastic surgeons and nurses. In five different villages and cities, 1015 patients were operated: 41% concerned cleft lip and palate and 32% post-burn contractures. The work is rewarding and is considered by some the best holiday imaginable. The teams intend to continue their project in the future.     

Structural and functional abnormalities in the islets isolated from type 2 diabetic subjects

Type 2 diabetic subjects manifest both disordered insulin action and abnormalities in their pancreatic islet cells. Whether the latter represents a primary defect or is a consequence of the former is unknown. To examine the beta-cell mass and function of islets from type 2 diabetic patients directly, we isolated islets from pancreata of type 2 diabetic cadaveric donors (n = 14) and compared them with islets from normal donors (n = 14) matched for age, BMI, and cold ischemia time. The total recovered islet mass from type 2 diabetic pancreata was significantly less than that from nondiabetic control subjects (256,260 islet equivalents [2,588 IEq/g pancreas] versus 597,569 islet equivalents [6,037 IEq/g pancreas]). Type 2 diabetic islets were also noted to be smaller on average, and histologically, islets from diabetic patients contained a higher proportion of glucagon-producing alpha-cells. In vitro study of islet function from diabetic patients revealed an abnormal glucose-stimulated insulin release response in perifusion assays. In addition, in comparison with normal islets, an equivalent number of type 2 diabetic islets failed to reverse hyperglycemia when transplanted to immunodeficient diabetic mice. These results provide direct evidence for abnormalities in the islets of type 2 diabetic patients that may contribute to the pathogenesis of the disease.     

Breast cancer and the role of breast size as a contributory factor

Several studies have been published on the incidence of breast cancer following augmentation mammaplasty, with very different conclusions. A lower incidence of breast cancer than expected was found in a study conducted by Deapen and Brody in women who had undergone augmentation surgery, and an anticarcinogenic effect of silicone implants has been suggested. Before accepting this conclusion it is important to study the relationship between breast size and the risk of developing breast cancer. It can be assumed that on average, women who have their breasts enlarged have smaller breasts, originally, than other women. Therefore, it seemed possible that breast size might be the predominant factor, and not the silicone implant. To test the hypothesis that women with breast cancer have statistically larger breasts than women who are not found to have breast cancer, a questionnaire was sent to each of 232 women who had undergone surgery for breast cancer; 146 of these questionnaires were returned. Body mass index (BMI) and breast size were compared against the corresponding values in a population-based control group. The results showed no statistical difference between the two groups in BMI or breast size. These findings seem to support the hypothesis that silicone breast implants have some kind of anticarcinogenic effect.     

Parapharyngeal lymph node metastasis: an unusual presentation of papillary thyroid carcinoma

BACKGROUND: Parapharyngeal space nodal metastases are usually secondary to malignancies of the pharynx and sinonasal tract, although localization of lymphomas is also possible. Parapharyngeal metastases arising from thyroid papillary carcinoma are instead an exceedingly rare event, with only 10 cases reported up to now in the literature. METHODS: We describe two cases of parapharyngeal metastasis from thyroid papillary carcinoma in a man and a woman, aged 40 and 52 years, respectively. RESULTS: Both patients had a lesion that clinically appeared to be located in the parapharyngeal space; they underwent CT and MRI, which detected a cystic mass in the poststyloid compartment. In the first patient, fine-needle aspiration cytology failed in identifying the histologic nature of the lesion, which was excised through a transcervical approach. A diagnosis of metastatic thyroid papillary carcinoma was rendered and therefore the patient underwent total thyroidectomy. In the second patient, a total thyroidectomy, previously scheduled for multinodular goiter, was performed along with the removal of the parapharyngeal mass. Definitive histologic findings revealed that the two parapharyngeal masses were cystic metastases from a thyroid papillary carcinoma. Both patients received postoperative 131I treatment. Twenty-four months after surgery, the first patient is free of disease, whereas the second one has clear signs of abnormal 131I uptake in the lungs. CONCLUSIONS: The differential diagnosis of a parapharyngeal poststyloid mass should also include metastasis from thyroid papillary carcinoma. When the lesion displays a cystic appearance on imaging, it is advisable to rule out a thyroid primary by ultrasonographic examination. The occurrence of a metastasis in such unusual site, even though rarely reported, does not seem to significantly affect the prognosis of the disease.     

Notfalltherapie der akuten Fremdkörperaspiration beim Kind

Zusammenfassung Die akute Fremdkörperaspiration stellt einen relativ häufigen und lebensbedrohlichen respiratorischen Notfall im Kleinkindesalter dar. Nach Alarmierung eines Rettungsteams wegen akuter Erstickungssymptome besteht bei ca. 40% der Patienten diese Symptomatik noch beim Eintreffen vor Ort weiter. Die Diagnosestellung erfordert die explizite Anamnesefrage nach einer möglichen Aspiration. Insgesamt am häufigsten ist die Aspiration von Nahrungsbestandteilen, insbesondere Nüssen. Lebensbedrohliche Situationen ergeben sich bei laryngealer oder trachealer Lage des Fremdkörpers. Solange der Patient eine Eigenatmung aufweist bzw. bei Bewusstsein ist, sollten keine Entfernungsversuche vor Ort durchgeführt werden. Ist der Patient jedoch asphyktisch und bewusstlos, kann durch verschiedene Manöver (Thoraxkompression, jenseits des Säuglingsalters evtl. auch Schlag auf den Rücken, Heimlich-Handgriff) versucht werden, den Fremdkörper zu entfernen. Bei Erfolglosigkeit lässt sich bei der Intubation ein pharyngealer Fremdkörper direkt entfernen oder bei trachealer Lage durch Beatmung ggf. mit manueller Thoraxkompression in der Exspiration oft doch noch eine Ventilation erreichen oder der Fremdkörper nach distal schieben. In allen Fällen sollte jedoch der Patient so rasch als möglich unter notärztlicher Begleitung in eine Klinik gebracht werden, wo ein erfahrenes Team zur bronchoskopischen Entfernung des Fremdkörpers zur Verfügung steht.     

Short-term and long-term results of speech improvement after surgery for velopharyngeal insufficiency with pharyngeal flaps in patients younger and older than 6 years old: 10-year experience

Velopharyngeal insufficiency (VPI) is a well-known cause for hypernasality. To overcome this problem, the authors use a static surgical technique: a cranially or caudally based flap. In 93 patients, the results of this technique on speech (hypernasality, nasal air escape, articulation) and velopharyngeal function were evaluated over a period of at least 1 year. In 53 patients, pharyngoplasty flaps were based caudally. In 40 patients, pharyngoplasty flaps were based cranially. The patients were age 2.5 to 24.5 years, with a mean of 5.5 years (SD: 4 years and 2 months). Improvement was found in almost all patients. The patients who underwent surgery when they were younger than age 6 significantly improved better then the patients who were treated when they were older than age 6. There were no differences in outcome between cranially based and caudally based flaps. There were also no differences between patients with plain VPI and patients with VPI (e.g., Pierre Robin sequence and Shprintzen).