Failed preoperative vacuum bell therapy does not affect outcomes following minimally invasive repair of pectus excavatum.

PURPOSE: It is unknown if failed preoperative vacuum bell (VB) treatment in patients undergoing minimally invasive repair of pectus excavatum (MIRPE), delays repair and/or affects postoperative outcomes. METHODS: A retrospective data analysis including all consecutive patients treated at one single institution undergoing MIRPE was performed between 2000 and 2016. Patients were stratified into preoperative VB therapy versus no previous VB therapy. RESULTS: In total, 127 patients were included. Twenty-seven (21.3%) patients had preoperative VB treatment for 17 months (median, IQR 8-34). All 27 patients stopped VB treatment due to the lack of treatment effect. Eight (47.1%) of 17 assessed VB patients showed signs of skin irritation or hematoma. VB treatment had no effect on length of hospital stay (p = 0.385), postoperative complications (p = 1.0), bar dislocations (p = 1.0), and duration of bar treatment (p = 0.174). Time spent in intensive care unit was shorter in patients with VB therapy (p = 0.007). Long-term perception of treatment including rating of primary operation (p = 0.113), pain during primary operation (p = 0.838), own perspective of look of chest (p = 0.545), satisfaction with the procedure (p = 0.409), and intention of doing surgery again (p = 1.0) were not different between groups. CONCLUSIONS: Failed preoperative VB therapy had no or minimal effect on short-term outcomes and long-term perceptions following MIRPE.

[Motility disorders of the esophagus]. / Motilitätsstörungen des Ösophagus.

Motility disorders of the esophagus comprise a heterogeneous spectrum of diseases. Primary malformations of the esophagus are now amenable to improved surgical and gastroenterological therapies; however, they often lead to persistent long-term esophageal dysmotility. Achalasia originates from impaired relaxation of the gastroesophageal sphincter apparatus. Systemic diseases may give rise to secondary disorders of esophageal motility. A number of visceral neuromuscular disorders show an esophageal manifestation but aganglionosis rarely extends into the esophagus. The growing group of myopathies includes metabolic and mitochondrial disorders with increasing levels of genetic characterization and incipient emergence of therapeutic strategies. Esophagitis with an infectious etiology causes severe dysmotility particularly in immunocompromised patients. Immunologically mediated inflammatory processes involving the esophagus are increasingly better understood. Finally, rare tumors and tumor-like lesions may impair esophageal motor function.

Presymptomatic thyroidectomy in multiple endocrine neoplasia 2a.

AIMS: To evaluate the value of prophylactic total thyroidectomy in multiple endocrine neoplasia 2a (MEN 2a), based on results of genetic testing, in a presymptomatic early stage of the disease. METHODS: Fourteen presymptomatic patients genetically diagnosed and surgically treated at our centre. We analysed age, gender, location of the RET mutation, calcitonin tests, surgery, histologic findings, TNM classification, and postoperative follow-up. RESULTS: The 14 patients belonged to two families with MTC (MEN 2a). Median age was 16 years. The RET mutation was located in codon 618 and 634. Basal calcitonin (CT) levels were normal in all patients. Twelve had pathologic peak CT measurements. Total thyroidectomy was performed in all and associated central neck dissection in 12 patients. Pathohistologic assessment showed C-cell hyperplasia in all specimens and 11 MTCs; the median size of the tumours was 0.2 cm; two patient had lymph-node metastases. According to TNM, three had stage 0, nine had stage I, one had stage II, and one had stage III disease. Postsurgery basal and peak CT values were normal in all but one patients, indicating a biochemical curative rate of 95%. Calcitonin determination did not distinguish between MTC and C-cell hyperplasia. CONCLUSION: Prophylactic thyroidectomy based on genetic testing allows identification and treatment of patients at an early stage of the disease. Pathologic peak CT values are markers for the presence of microscopic MTC and should be considered in selecting operative procedures for these patients.

Peritonitis in childhood: clinical relevance of cytokines in the peritoneal exudate.

BACKGROUND: The aim of the study was to assess pro-inflammatory cytokine (tumor necrosis factor alpha [TNF-alpha], interleukin 6 [IL-6]) and anti-inflammatory cytokine (interleukin 10 [IL-10]) responses in children with peritonitis secondary to hollow viscus perforation, and to evaluate the influence of peritoneal microbial status on cytokine expression and morbidity. METHODS: The peritoneal fluid of 13 children with perforated appendicitis was examined with qualitative analysis of bacteria, and measurement of cytokine levels, which were compared to cytokine plasma levels, over a five-day period following operation. RESULTS: All fluid specimens showed permanently elevated levels of TNF-alpha and IL-10. IL-6 tended to decrease to normal levels by the 5th postoperative day. Peritoneal exudate levels of TNF-alpha and IL-10 were more than 100 - 1000 times greater than those in plasma. The most common bacterial species isolated in the peritoneal fluid was Escherichia coli. Despite persisting high cytokine concentrations and bacterial load of the peritoneal cavity for 5 days postoperatively, the children recovered uneventfully and the systemic signs of infection disappeared rapidly. CONCLUSION: Neither the bacterial nor the pro-inflammatory cytokine load of the peritoneal cavity proved to be associated with the clinical course. We hypothesize that in peritonitis in childhood a significant and clinically relevant cytokine-mediated inflammatory response is compartmentalized in the peritoneal cavity. Therefore adjuvant surgical measures in addition to appendectomy and intraoperative debridement seem not to be necessary, at least for peritonitis due to perforated appendicitis in children.

Acute epididymitis in children: a 4-year retrospective study.

OBJECTIVE: To evaluate the incidence of acute epididymitis (AE) compared to testicular torsion (TT) as a form of acute scrotum in children, to describe clinical aspects and to assess the value of laboratory tests and radiological investigations in AE. METHODS: Retrospective review of the medical records of 49 patients presenting with clinical aspects of AE over a 4-year period; evaluation of clinical features, laboratory tests (blood, urine), radiological examinations (duplex and real-time ultrasound, renal sonography, voiding cysturethrography [VCUG]) and urodynamics. RESULTS: 49 patients with an overall mean age of 9.8 years (range 0.2-15.3 years) presented with AE. In the same period, 31 subjects required surgery for TT. Local pain on palpation of the epididymis and spontaneous pain in the testis and/or epididymis were the most common clinical signs. In 2 patients, urine cultures revealed significant bacteriuria. Duplex and real-time ultrasound showed no false negative result. 41% of the patients revealed concomitant urological diseases, but only in 1 patient was treatment influenced by the uropathy. 16/21 older boys (76%) demonstrated normal uroflow patterns. CONCLUSION: AE seems to be more common than acute TT. Urinalysis and urine culture should be performed for all children with AE. High resolution ultrasound with an experienced investigator is able to exclude TT reliably so that routine surgical exploration is seldom necessary.

A case of virilised female with urethral duplication and valve formation.

We report the case of a female (chromosomal 46 XX) with a complex congenital urogenital malformation. Caesarian section was performed at the 30th gestational week due to hydrops fetalis and polyhydramnion. Clinical and radiological evaluation demonstrated a complex urogenital malformation with an enlarged penoclitoral organ, urethral duplication, and concomitant malformations. The girl presented with a female urethra with a vaginal orificium as well as a male urethra culminating in a microphallus. Additionally, a urethral valve formation just cranial to the bifurcation of the male and female urethra was also present. Due to concomitant diseases, a complicated clinical course and recurrent urinary tract infections, the final operative correction resulting in a female phenotype, was performed at the age of 2 (1/2) years as a single-stage procedure.

Minimally invasive repair of pectus excavatum (MIRPE)--the Basel experience.

PURPOSE: Minimally invasive repair of pectus excavatum (MIRPE) was first reported in 1998 by D. Nuss. This technique has gained wide acceptance during the last 4-5 years. In the meantime, some modifications of the technique have been introduced by different authors. Our retrospective study reports our own experience over the last 36 months and modifications introduced due to a number of complications. METHODS: From 3/2000 to 3/2003, 22 patients underwent MIRPE. Patients median age was 15.5 years (10.7 to 20.3 years). Standardised preoperative evaluation included 3D computerised tomography (CT) scan, pulmonary function tests, cardiac evaluation with electrocardiogram and echocardiography, and photo documentation. Indications for operation included at least two of the following: Haller CT index > 3.2, restrictive lung disease, cardiac compression, progression of the deformity and severe psychological alterations. RESULTS: In 22 patients (2 girls, 20 boys) undergoing MIRPE procedure, a single bar was used in 21 patients and two bars in one boy. Lateral stabilisers were fixed with non resorbable sutures on both sides. Overall, postoperative complications occurred in six patients (27.3%). In two patients (9.1%) a redo-procedure was necessary due to bar displacement. An additional median skin incision was performed in two patients to elevate the sternum. Pneumothorax or hematothorax in two patients resulted in routine use of a chest tube on both sides. Long-term favourable results were noted in all patients. CONCLUSIONS: The MIRPE procedure is an effective method with elegant cosmetic results. Modifications of the original method help to decrease the complication rate and to accelerate acquirement of expertise.

Peritonitis in childhood: aspects of pathogenesis and therapy.

The pathogenesis of peritonitis due to hollow-viscus perforation is currently accepted as being mainly based on the local and systemic release of pro- and anti-inflammatory mediators triggered by the presence of bacteria and bacterial products in the abdominal cavity. Therefore, treatment consists in focal restoration, intraoperative debridement and lavage, and postoperative measures such as drainage, continuous peritoneal lavage, or scheduled reoperation aiming at the removal of infectious agents from the peritoneal cavity to prevent persisting peritonitis and sepsis. In order to evaluate the pathophysiologic relevance of the bacterial and endotoxin load of the peritoneal exudate, we examined the peritoneal fluid of 20 children with perforated appendicitis for qualitative and quantitative analysis of bacteria, antibiotic concentrations, and endotoxin content. The time period ranged in 12-h intervals from intraoperatively to day 5. Eighteen of 20 fluid specimens (90%) showed endotoxin levels above 1.5 endotoxin units EU/ml (standard <0.1 EU/ml). The most common bacterial species isolated was Escherichia coli, mostly in high concentrations. Despite persisting high endotoxin concentrations and bacterial loads in the peritoneal cavity during the 5 postoperative days, the children recovered uneventfully and the systemic signs of infection disappeared rapidly. In conclusion, neither the bacterial nor the endotoxin load of the peritoneal cavity proved to be associated with the clinical course. Therefore, we hypothesize that during peritonitis compartmentalization of the focus of infection prevents further systemic reactions and ultimately leads to removal of the infectious agents by endogenous mechanisms. At least in peritonitis due to perforated appendicitis in children, adjuvant surgical measures in addition to appendectomy and intraoperative debridement are not necessary.