Comparison of environmental risk factors for esophageal atresia, anorectal malformations, and the combined phenotype in 263 German families.

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and anorectal malformations (ARM) represent the severe ends of the fore- and hindgut malformation spectra. Previous research suggests that environmental factors are implicated in their etiology. These risk factors might indicate the influence of specific etiological mechanisms on distinct developmental processes (e.g. fore- vs. hindgut malformation). The present study compared environmental factors in patients with isolated EA/TEF, isolated ARM, and the combined phenotype during the periconceptional period and the first trimester of pregnancy in order to investigate the hypothesis that fore- and hindgut malformations involve differing environmental factors. Patients with isolated EA/TEF (n = 98), isolated ARM (n = 123), and the combined phenotype (n = 42) were included. Families were recruited within the context of two German multicenter studies of the genetic and environmental causes of EA/TEF (great consortium) and ARM (CURE-Net). Exposures of interest were ascertained using an epidemiological questionnaire. Chi-square, Fisher's exact, and Mann-Whitney U-tests were used to assess differences between the three phenotypes. Newborns with isolated EA/TEF and the combined phenotype had significantly lower birth weights than newborns with isolated ARM (P = 0.001 and P < 0.0001, respectively). Mothers of isolated EA/TEF consumed more alcohol periconceptional (80%) than mothers of isolated ARM or the combined phenotype (each 67%). Parental smoking (P = 0.003) and artificial reproductive techniques (P = 0.03) were associated with isolated ARM. Unexpectedly, maternal periconceptional multivitamin supplementation was most frequent among patients with the most severe form of disorder, i.e. the combined phenotype (19%). Significant differences in birth weight were apparent between the three phenotype groups. This might be attributable to the limited ability of EA/TEF fetuses to swallow amniotic fluid, thus depriving them of its nutritive properties. Furthermore, the present data suggest that fore- and hindgut malformations involve differing environmental factors. Maternal periconceptional multivitamin supplementation was highest among patients with the combined phenotype. This latter finding is contrary to expectation, and warrants further analysis in large prospective epidemiological studies.

Morphologic assessment of thoracic deformities for the preoperative evaluation of pectus excavatum by magnetic resonance imaging.

OBJECTIVE: To assess whether MRI is a suitable modality for the preoperative assessment and quantification of pectus excavatum. METHODS: A total of 69 patients (57 male, 12 female; median age 15 years, range 5-35 years) with pectus excavatum were evaluated preoperatively using standardized MRI sequences on 1.5- and 3-Tesla systems (T2-HASTE/inspiration and expiration, T1-VIBE, T2-TRUFI free-breathing, T2-BLADE). The MR sequences were analysed for quality semiquantitatively. The Haller index, correction index, sternal rotation angle and asymmetry index were assessed; correlations between these indices and changes in inspiration and expiration were evaluated. RESULTS: T2-HASTE was the best sequence to assess pectus excavatum morphology, with a higher quality at 3 T than at 1.5 T. All indices could be assessed in every patient. A total of 37 patients had a symmetric deformity, 32 patients an asymmetric deformity. The Haller index correlated significantly (p < 0.001) with the correction index, both becoming higher in expiration. The asymmetry index correlated with the sternal rotation angle (p < 0.001) and did not change significantly in expiration (p = 0.28). CONCLUSIONS: Thoracic MRI is suitable for the preoperative evaluation of patients with pectus excavatum. An exact morphologic assessment is possible without radiation exposure as well as the determination of several indices to quantify the deformities.

Laparoscopic inguinal hernia repair in premature babies weighing 3 kg or less.

PURPOSE: This retrospective study aims to evaluate the feasibility, safety and complication rate of laparoscopic inguinal hernia repair for small babies weighing 3 kg or less. METHODS: A retrospective analysis was performed on the surgical charts of 67 infants (47 boys and 20 girls) weighing 3 kg or less who underwent laparoscopic hernia repair in a 3-year period. A regular 5-mm scope was used for visualization, and 2 or 3-mm instruments were used for the closure of the inner inguinal ring using 3/0 non-absorbable suture. The median weight at surgery was 2,600 g (range 1,450-3,000 g). All except three were premature. RESULTS: Of the 67 infants, 15 (22.3 %) presented with an irreducible hernia. In three cases of irreducible hernias, we also performed a transumbilical appendectomy at the end of the hernia repair. Minor problems related with anesthesia were noted in four cases. Hernia recurrence was observed in three patients (4.4 %). No cases of testicular atrophy occurred. In 10 boys, we observed 12 cases of high testes, only 4 testes requiring subsequent orchiopexy. CONCLUSIONS: Laparoscopic inguinal hernia repair for babies weighing 3 kg or less is feasible, safe and perhaps even less technically demanding than open inguinal herniotomy.

[Appendicitis in childhood: correlation of clinical data with histopathological findings]. / Akute Appendizitis im Kindesalter: Korrelation klinischer und histopathologischer Befunde.

BACKGROUND: Quantifying the histopathological diagnoses of appendectomies in daily routine paidopathology results in a high percentage of appendices without histomorphological sign of acute inflammation. To identify clinical factors significantly associated with the morphological diagnosis, histopathological findings and clinical data--documented in patients' files--were examined. PATIENTS: All 856 children (age: 5 m-15 yrs) whose appendix had been resected within a 7-year-period were--depending on the histopathological diagnoses--allocated to the group "appendix without" resp. "appendix with signs of acute inflammation". METHOD: All files were examined concerning anamnestic data, clinical signs of acute appendicitis and laboratory parameters. The data were analysed by χ(2)-test and Wilcoxon-test concerning differences between the 2 groups with regard to the anamnestic and clinical facts and parameters. Using binary logistic regression, these clinical parameters were analyzed in correlation with the histopathological diagnoses. RESULTS: By consideration of the factors "leucocyte count", "vomiting" and "percussion tenderness" 75% of the children would have been allocated to the accurate postoperative pathomorphological diagnosis. CONCLUSIONS: There was a significant correlation of "leucocyte count", "vomiting" and "percussion tenderness" with histopathology in 75% of the children. As hence 25% were not allocated correctly this combination is no unequivocal combination for prediction or exclusion of an acute appendicitis. Hence, even with these statistically significant parameters a comparably high percentage of falsely-positive appendectomies has to be anticipated.

[Cues and pseudocues in surgical multiple choice questions from the German state examination]. / "Cues" und "pseudocues" in chirurgischen MC-Fragen des deutschen Staatsexamens.

AIM: Multiple choice questions (MCQs) are the most common written test item in medical examinations. Writing MCQs is difficult and cues can decrease test validity. The aim of this article is to describe the prevalence and pattern of cues in surgical MCQs in the central German medical examination questions from 2000-2011. METHOD: All surgical questions were entered into a database. The questions were reviewed for cues and pseudocues independently by three students of different academic background and one clinical physician. RESULTS: Initially, 1014 questions were included, 22 questions were not rated uniformly by the reviewers and 3 questions were excluded because no consensus could be reached. Overall 15.2 % of the questions analyzed contained some type of cue or pseudocue. Of the total questions 0.2 % contained type A cues (i.e. disruption of grammatical flow), 6.5 % contained type B cues (i.e. heterogeneous length or differentiation), 4.0 % contained type C cues (two or more answers described closely related topics, serving to focus attention), 0.6 % contained type D convergence cues (i.e. answers with the most items in common with distractors are correct), 0.7 % contained type E verbal association cues (i.e. connection in the use of words leading to the correct answer) and 1.0 % contained type F cues (i.e. answers with absolute terms). Pseudocues were found in 3.7 % of the questions. DISCUSSION: The proportion of questions that contained cues or pseudocues should lead to further efforts to avoid such factors compromising test reliability by specific attention in the process of question design and review. CONCLUSION: Cues are still an important consideration in designing MCQs and are present in considerable numbers in medical state examination questions. Pseudocues should be explicitly avoided so as not to compromise validity and reliability.

Laparoscopic repair of incarcerated inguinal hernia. A safe and effective procedure to adopt in children.

BACKGROUND: The purpose of our retrospective study was to describe the efficacy and the advantages of laparoscopic approach to treat incarcerated inguinal hernia (IIH) in pediatric patients. METHODS: In a 2-year period, 601 children underwent a laparoscopic inguinal hernia repair, 46 (7.6 %) of them presented an IIH. Our study will be focused on these 46 patients: 30 boys and 16 girls (age range 1 month-8 years). RESULTS: Twenty-one/46 hernias (45.6 %) were reduced preoperatively and then operated laparoscopically (RH), 25/46 (54.4 %) were irreducible and they were operated directly in laparoscopy (IRH). We have no conversions in our series. The length of surgery in RH group was in median 23 min and in IRH group was in median 30 min. Hospital stay was variable between 6 h and 3 days (median 36 h).With a minimum follow-up of 14 months, we had 2/46 recurrences (4.3 %). CONCLUSION: The laparoscopic approach to IIH appears easy to perform from the technical point of view. The 3 main advantages of laparoscopic approach are that all edematous tissue are surgically bypassed and the cord structures are not touched; the reduction is performed under direct visual control, and above all, an inspection of the incarcerated organ is performed at the end of procedure.

Stent-free tubularized incised plate (TIP) repair of distal and mid-shaft hypospadias irrespective of age.

PURPOSE: Aim of this study was to report our experiences with tubularized incised plate (TIP) repair without placement of a postoperative urethral stent in 41 cases. PATIENTS AND METHODS: Since October 2005, we have performed TIP repair for distal penile hypospadias without using a postoperative urethral stent. A urethral stent is used intraoperatively for the tubularization of the neourethra and is removed at the end of the procedure. Follow-up included clinical examination and a structured telephone interview on parental satisfaction. RESULTS: The group included 41 boys, 60% with coronal or distal hypospadias and 40% with mid-shaft hypospadias (aged 6 months to 16 years, median: 3 years). In 7 cases, the prepuce was also reconstructed. There were 2 cases with fistula and one case of meatal stenosis. No glans dehiscence, severe bleeding, or wound infection was observed. No urinary retention requiring catheterization was observed, irrespective of age. All but one patient was discharged the day after surgery. Follow-up ranged from 8 to 48 months (average: 22 months). Most parents (87.5%) were satisfied or very satisfied. CONCLUSION: Based on our preliminary experiences, patient comfort and safety, parental satisfaction and the rate of complications seem to be promising with this technique.

Microlaparoscopy for recurrent abdominal pain in children.

PURPOSE: This prospective study evaluated the diagnostic and therapeutic feasibility and safety of microlaparoscopy for the management of chronic abdominal pain in children. METHODS: The study included 45 children (13 boys and 32 girls; age range from 6 to 16 years, average 9.5 years) undergoing diagnostic microlaparoscopy for chronic abdominal pain. Microlaparoscopy (the exclusive use of 2 mm instrument sets and small diameter scopes, i. e. 1.7 mm, 1.9 mm and 2.4 mm) was performed after common organic diseases were ruled out by careful baseline investigations. RESULTS: No complications occurred which were related to the exclusive use of 2 mm instruments and small scopes. CT scans were avoided in all patients. No intraoperative pathological findings were found in 18 children. In another 18 children, the intraoperative findings indicated the need for further surgical intervention. At follow-up, 26 patients reported that they were totally pain-free; 10 children had partial resolution after surgery, and 8 children reported only minimal resolution. In 20 cases, the procedures were accomplished as an outpatient surgery. CONCLUSION: Microlaparoscopy seems to be a safe and effective diagnostic tool with a favorable diagnostic accuracy, minimal access trauma and superior cosmesis in children.

Laparoscopic inguinal herniorrhaphy in premature infants.

INTRODUCTION: We report the clinical, operative, and outcome results in 58 premature infants (with maximum weight of 5,000 g at time of surgery) undergoing laparoscopic herniorrhaphy. PATIENTS AND METHODS: This study was designed as a prospective, non-randomized single center feasibility study. The inclusion criteria were: symptomatic inguinal hernia, gestational age up to 37 weeks and maximum weight of 5,000 g at the time of surgery. Out of 58 premature infants (42 boys and 16 girls), 24 had bilateral, 20 had right-sided and 14 had left-sided hernias. 14 (24.1%) infants were operated on for an irreducible hernia. RESULTS: The median gestational age at birth was 33 weeks (range 23-37) and the median gestational age at operation was 41 weeks (range 33-52). The body weight at surgery ranged from 1,450 g to 5,000 g (median 3 900 g); 11 infants (19%) weighed less than 2 500 g. No intraoperative surgical complications occurred. Anesthesia complications were noted in 7 cases. At median follow-up of 25 months (range 6-51 months), there were 3 hernia recurrences in 2 infants (3.6%). In 5 boys, we observed high testes requiring subsequent orchiopexy. Regression analysis showed that the risk of undescended testes increased by 65.5% for every 1 kilo lower weight at surgery. CONCLUSION: Based on our early results, it seems that laparoscopic hernia repair in preterm infants and very low birth weight babies is a safe and feasible procedure and has some procedural benefits compared to the standard open technique.

CD rats as an animal model in the experimental study of laparoscopic hernia repair.

PURPOSE: The aim of this study was to evaluate the technical feasibility and efficacy of a sutureless laparoscopic hernia repair in a small animal model. The objective was to occlude the processus vaginalis with biocompatible fleece and/or fibrin glue as an alternative to suturing. METHODS: Sixty-three male CD rats were randomly assigned to one of three groups. In group A (n=21), the internal inguinal ring was filled with 0.5 ml fibrin glue. The second group (B, n=21) also received fibrin glue, and a biocompatible fleece was placed on top. The third group consisted of control animals (C, n=21). Eleven rats in each group underwent laparoscopic surgery. The remaining rats were operated using an open technique, and the paraductal lipomas were resected in addition to inguinal ring closure. RESULTS: Complete closure of the internal hernia ring was not achieved in any of the rats, neither in the laparoscopic group nor in the open group or the control group. The paraductal lipoma grew back to its normal size, although resection of the lipoma was performed during the first procedure. CONCLUSION: The physiology of paraductal lipomas in this animal appears to make it an inadequate model for the study of laparoscopic inguinal hernia repair.

Ovarian tumours: late extramedullary recurrence of acute leukaemia.

OBJECTIVE: Isolated extramedullary relapse, especially ovarian recurrence, of acute leukaemia is rare. Local therapy such as irradiation or extensive surgical resection of the mass is ineffective and unnecessary. MATERIALS AND METHODS: Over a 20-year period we observed two girls with ovarian relapse of acute lymphoblastic leukaemia (ALL) in over 300 treated children for ALL. Pre-B ALL was diagnosed in a girl at the age of three. Treatment was initiated according to the CoALL 82-protocol. At the age of 11, the girl presented with a huge abdominal mass. Chemotherapy and low-dose radiotherapy succeeded in shrinking the tumour mass, making it operable. A salpingo-oophorectomy was performed. In the second case, a 14-year-old girl in whom pre-B ALL was diagnosed was treated according to the protocol CoALL 06-97. After having achieved complete haematological remission in the bone marrow, she stayed in remission for 18 months. Subsequently, she developed a painless abdominal tumour. Laparoscopic lymph node staging was performed and biopsies were taken. Chemotherapy was initiated according to the BFM protocol for ALL recurrence. Extensive surgical resection of the leukaemic mass, as well as additional radiation was avoided. CONCLUSION: Because we experienced favourable results with laparoscopic biopsy in our patients, we are of the opinion that laparoscopy-assisted biopsies are well suited for the management of intra-abdominal tumours in systemic malignant disease.