Inspiratory stridor and dysphagia in two newborn infants caused by ectopic thymus tissue.

We report two cases of ectopic cervical thymus, a solid thymic lesion, and a thymus cyst causing inspiratory stridor and mild dysphagia in the neonatal period. Because of the rarity of thymic dystopia, the two masses were initially misdiagnosed as more common entities, namely, lymph node enlargement and lymphangioma, respectively. The correct diagnosis was made only after surgical excision and histopathological examination. This case report is completed by a short review of embryogenic development, diagnostic procedures with differential diagnoses, and therapeutic outcome of ectopic thymus.

[Von Hippel-Lindau disease. Interdisciplinary patient care]. / Von-Hippel-Lindau-Erkrankung. Interdisziplinäre Patientenversorgung.

Von Hippel-Lindau disease is an important hereditary tumor syndrome with a clear option for effective treatment if diagnosed in time. Interdisciplinary cooperation is the key to successful management. Major components of the disease are retinal capillary hemangioblastomas, hemangioblastomas of cerebellum, brain stem and spine, renal clear cell carcinomas, pheochromocytomas, multiple pancreatic cysts and islet cell carcinomas, tumors of the endolymphatic sac of the inner ear, and cystadenomas of the epididymis and broad ligament. A well structured screening program should be performed at yearly intervals.

Comparison of economic and environmental impacts between disposable and reusable instruments used for laparoscopic cholecystectomy.

BACKGROUND: The economic and environmental effects were compared between disposable and reusable instruments used for laparoscopic cholecystectomy. Special consideration was given to the processing of reusable instruments in the Miele G 7736 CD MCU washer disinfector and the resultant cost of sterilization. METHODS: The instruments frequently used in their disposable form were identified with the help of surgeons. Thus, of all the instruments used for laparoscopic cholecystectomy, the disposable and reusable versions of trocars, scissors, and Veress cannula were compared. RESULTS: For the case examined in this study, the performance of laparoscopic cholecystectomy with disposable instruments was 19 times more expensive that for reusable instruments. The higher cost of using disposable instruments is primarily attributable to the purchase price of the instruments. The processing of reusable instruments has little significance in terms of cost, whereas the cost for disposing of disposable instruments is the least significant factor. The number of laparoscopic cholecystectomies performed per year does not substantially influence cost. In the authors' opinion, assessment of the environmental consequences shows that reusable instruments are environmentally advantageous. CONCLUSIONS: Considering the upward pressure of costs in hospitals, disposable instruments should be used for laparoscopic cholecystectomy only if they offer clear advantages over reusable instruments.

Laparoscopically assisted colorectal resection in Hirschsprung's disease.

BACKGROUND: Minimally invasive surgery (MIS) has been adapted to virtually every kind of abdominal operation. Colorectal resections of any extent are possible. The reduction in local and systemic surgical trauma in MIS suggests this method could be valuable for rectal resection for Hirschsprung's disease. - METHOD: Diagnostic work-up is similar to that in open surgery. Three trocars are sufficient, and a fourth may be helpful. Dissection encompasses the entire aganglionic segment and is extended orally to normal bowel.. After complete dissection down to the pelvic floor the bowel is everted transanally, resected in due length and the coloanal anastomosis sutured from outside. Since 1996 four children out of nine (age 11 weeks - 18 years) have been operated laparoscopically at our institution. - RESULTS: There were no intraoperative complications. Recovery time is impressively rapid. Oral intake may be begun on the first postoperative day and physical activity is rapidly restored. There are however problems typical to this method: anastomotic leakage, stricture formation, and the necessity of continuing dilation. - CONCLUSIONS: The feasibility of laparoscopic colorectal resections of any extent is unquestionable. Benefits for the patient seem evident. Prospective randomized studies to provide a higher level of evidence for the benefit of laparoscopy as compared to open technique are difficult to perform due to the small number of patients.

Expression of CD44, ICAM-1 and N-CAM in colorectal cancer. Correlation with the tumor stage and the phenotypical characteristics of tumor-infiltrating lymphocytes.

The cellular adhesion molecules (CAMs) CD44s, CD44v6, CD44v10, ICAM-1 and N-CAM were immunohistologically detected in colorectal cancers using the APAAP method. The expression of CD44s and CD44v6 was associated with the presence of lymph node metastases in the examined tumors. The pattern of ICAM-1 expression was inversely related to that of CD44, i.e. lower numbers of ICAM-1 positive cells were observed in metastasizing tumors. An intense focal staining of N-CAM was observed in the majority of the metastasizing tumors. The expression of CD44v, ICAM-1 or N-CAM on tumor cells did not correlate with the density of the tumor-infiltrating lymphocytes (TIL) within the tumors. The flowcytometric analysis of TIL showed a significant accumulation of CD25+ and HLA-DR+ cells and a reduced number of CD45RA+ cells as compared to autologous peripheral blood lymphocytes (PBL) or intraepithelial lymphocytes of the colon mucosa (IEL). These phenotypic characteristics of TIL did not correlate with the CAMexpression on tumor cells.

Is MIB-1 proliferation index a predictor for response to neoadjuvant therapy in patients with esophageal cancer?

BACKGROUND: The overall survival rate for patients with an esophageal cancer remains poor. As a consequence, preoperative chemoradiation was introduced for patients with tumor stage T >1 M0 regardless of tumor histology or localization. However, factors predicting response to this therapy pretherapeutically are largely unknown. METHODS: Clinical results of preoperative chemoradiation were investigated. The rates of proliferation and apoptosis were determined in pretherapeutic tumor samples and correlated with tumor response and long-term survival after surgery. RESULTS: A complete tumor response due to chemoradiation (n = 42; cervically localized tumors excluded) was achieved in 11 patients (26%) after resection. Five-year survival rate was significantly improved in these patients compared with those who did not respond to chemoradiation (48% versus 5.5%; P = 0.003). Chemoradiation was performed without benefit in 43%. Perioperative hospital mortality rate was 14.3% in all patients. No correlation of apoptosis with response to chemoradiation or postoperative long-term survival was observed. However, there was a clear correlation between the proliferation rate as determined by MIB-1 immunohistology. Five-year survival rate of patients with a proliferation index (PI) >/=39% was 38% compared with 0% in tumors with a PI <39%. Tumors with a PI >/=39% responded to chemoradiation in 71.4%, but 100% of tumors with a PI <39% did not. Mean survival time of these patients was 33 months and 11 months, respectively (P = 0.015). CONCLUSIONS: The results indicate that the PI may be used for stratification of patients treatment prior surgery. However, these results need further validation in larger patient numbers in the search for factors indicating response pretherapeutically to preoperative chemoradiation in esophageal cancer.

Value of RARE-MRI sequences in the diagnosis of lymphangiomatosis in children.

Three patients suffering from extensive cavernous lymphangiomatosis are presented here. They were examined by MRI using RARE-MR hydrography (rapid acquisition with relaxation enhancement) as well as conventional spin-echo sequences. RARE sequences, which depict each fluid-filled lymphatic space, can be used for screening. RARE-sequences help to shorten investigation time, particularly in cases involving the skeleton. The imaging strategy can be changed according to the results of this sequence. It may be performed prior to spin-echo sequences and facilitates follow-up investigations. RARE sequences distinguish between lymphangiomatosis and hemangiomatosis, or a combination of the two.

Dynamic graciloplasty in children with fecal incontinence: a preliminary report.

BACKGROUND: Various therapeutic methods have been developed for anal incontinence. A reconstruction of the sphincter system with functional adaptation can be achieved by stimulation of the transposed gracilis muscle with an implanted stimulator. METHODS: The authors performed a dynamic graciloplasty in 4 boys, aged between 6 and 10 years. Causes for the incontinence were polytrauma with pelvic rupture, VACTERL syndrome with rectal urethral fistula, anal atresia with primary reconstruction and 6 consecutive operations, and coccygeal teratoma in a premature infant with surgical treatment of the recurrent tumour. All children had grade III incontinence. Postoperatively, the duration of stimulation was increased successively by telemetric programming of the pulse generator up to a continuous mode. RESULTS: One child is now almost continent, 2 show grade I incontinence, 1 is incontinent with frequent soiling (the programming has not been completed yet). CONCLUSION: An evaluation of this method for children will not be possible until more operations have been performed, under the conditions of a prospective study, which appears indicated in view of the preliminary but encouraging results presented.

Mucinous cystadenoma of the ovary in a 15-year-old girl.

Benign neoplasms of the ovary originating from epithelial tissue are common tumors in adult women. They are, however, rarely seen in children or adolescent girls. Here the authors present a case of an ovarian mucinous cystadenoma in a premenarchal girl. To our knowledge, there are only 5 other cases reported in the literature.

Hepatopathy in two infants with short-bowel syndrome and cytomegalovirus infection.

In children with short-bowel syndrome and the need for long-term parenteral nutrition, hepatic dysfunction is a multifactorial phenomenon that has not been completely understood. Alterations in gut motility lead to intraluminal stasis which is thought to be a major etiologic factor for bacterial overgrowth and subsequent cholestasis, especially when the ileocecal valve is absent. We report on two infants with short-bowel syndrome caused by gastroschisis and intestinal atresia. The intestinal lengths after resection were 18 and 55 cm. Long-term parenteral nutrition (PN) was obligatory due to intestinal shortness in the first patient and dilatation of the preatretic bowel segment with ineffective peristalsis in the second patient. Despite multiple trials of enteral nutrition and medical therapy for gut decontamination and stimulation of bowel motility, hepatopathy developed in both patients in a similar period of time and to about the same degree. At the age of 4 and 6 weeks, respectively, increasing bilirubin values were measured. Deterioration of liver function and thrombocytopenia at the age of 3 to 4 months led to the diagnosis of acute cytomegalovirus (CMV) infection. Treatment with ganciclovir followed. Both patients died of acute liver failure at the age of 7 and 9 months, respectively. Additional hepatic injury secondary to CMV infection might have contributed to the rapid deterioration of liver disease. Screening for further hepatotoxic factors, especially infectious etiologies, is therefore recommended in children with short-bowel syndrome. Liver transplantation should be considered early in cases of progressive hepatic dysfunction.

Ergonomics: requirements for adjusting the height of laparoscopic operating tables.

BACKGROUND AND OBJECTIVES: In the last few years many new instruments and devices have been developed and introduced into the operating room (OR). A debate has been ongoing about the optimal ergonomic posture for the operating staff. From practical experience, we have learned that the operating tables cannot be adjusted adequately to allow surgeons of different stature to maintain a comfortable posture. The goal of this study was to establish the most ergonomic table height for the particular physique of the surgeon and the different types of laparoscopic instrument handles that he or she uses. METHODS: In a simulated model, two probands of different stature (50th [BS 50] and 95th [BS 95] percentile) used laparoscopic instruments with four different handle designs (shank, pistol, axial, and rod). The instruments were inserted into a board in three different angles ([IA] = 20 degrees, 30 degrees, 40 degrees). Additionally the elbow angles (EA) of the volunteers were fixed to either 90 degrees or 120 degrees. For every variable (size of surgeon and his or her elbow angle, design of handle, insertion angle of the instrument) the height of the board, as a parameter for the level of the abdominal wall of a patient with pneumoperitioneum, was measured from the floor. RESULTS: All parameters had an effect on the optimal operating table height. The lowest required operating table level was 30 cm, the highest was 60.5 cm. In laparoscopic surgery-long shafted instruments and patients with pneumoperitoneum-the tabletops are too high for over 95% of all surgeons. As skin incision and wound suture are performed the conventional way, the operating tabletop must be adjustable up to the common height of 122 cm. The maximal difference between the optimal heights of the OR-table for one volunteer using two different handles with different insertion angles of the instruments (BS 95, EA 90 degrees, IA 20 degrees, rod handle to BS 50, EA 120 degrees, IA 40 degrees, axial handle) was about 27 cm. CONCLUSION: New operating tables with a much lower adjustability are necessary to fulfill ergonomic requirements. The use of differently designed handles can hinder the ergonomic posture of the surgeon, because each handle requires a different working height.

[A retaining dam against reflux. Laparoscopic fundoplication helps even in stubborn cases]. / Ein Staudamm gegen den Reflux. Die laparoskopische Fundoplicatio hilft auch in hartnäckigen Fällen.

The introduction of proton pump inhibitors (PPI) and laparoscopic fundoplication in the treatment of gastroesophageal reflux disease offered an opportunity for definitive healing. The indication for surgery is the failure of medical treatment, recurrence of symptoms following conservative treatment, severe side effects of the medication, and the patient's wish to stop taking drugs. The laparoscopic treatment has a low rate of complications. Apart from temporary dysphagia (30%), rapid satiety, increased flatulence and suppressed eructation are possible undesirable sequelae. Intra-operative bleeding and organ perforation (1%) are major feared occurrences. The rate of conversion to open surgery is 5.8%, and the mortality rate is 0.1%. Persistent dysphagia in 3.4% may be caused by a slipped cuff. A revision procedure is necessary in 0.7% of the patients. Patient satisfaction with the results of the operation ranges between 87 and 100%.

[Laparoscopic gastric banding in therapy of obesity. Which patients benefit?]. / Laparoskopisches Magenband zur Therapie der Fettsucht. Bei welchen Patienten macht das Sinn?

In view of concomitant illnesses and sequelae associated with significantly increased morbidity and mortality, obesity is considered a major pathological condition. The aim of treatment must be the lasting reduction of overweight. The prerequisite for this is a fundamental change in the patient's inappropriate eating habits, as also other lifestyle aspects. This requires comprehensive and long-term disciplinary management. Within this framework, surgical treatment, in particular the laparoscopic placement of an adjustable gastric band, is the most effective form of achieving weight reduction. The band delays passage of food, and causes early distension of the stomach wall, thus inducing a sensation of satiety. To ensure therapeutic success, nutritional and surgical counseling of the patient has a major role to play.