Influence of age on clinical presentation, diagnosis delay and outcome in pre-school children with acute appendicitis.

BACKGROUND: Unusual clinical presentation of acute appendicitis in preschool children leads to misdiagnosis and complications. We aimed to analyze the influence of age on clinical presentation, laboratory findings and complications in preschool children with acute appendicitis. METHODS: From January 2012 until December 2017, 29 children younger than 6 years of age (median 50 months) with acute appendicitis were enrolled in this retrospective study. Patients were grouped according to their age: group 1: < 48 months (n = 13); group 2: > 48 months (n = 16), their clinical data, laboratory results and complications were compared. RESULTS: In group 1, duration of nausea and vomiting was longer, alteration of general state was more frequent and pain in the right fossa iliaca less frequent than in group 2 (p = 0.026, p = 0.000 and p = 0.029, respectively). Heart rate was higher in group 1 than in group 2 (p = 0.012). Leucocyte and polynuclear neutrophil counts were lower in group 1 than in group 2 (p = 0.028 and = 0.004, respectively) but C-reactive protein levels were not different between groups. In the whole cohort however, C-reactive protein at admission value correlated negatively with age (p = 0.025). Abdominal ultrasound allowed diagnosis in 19/29 patients (65.5%), without any difference between groups. Appendicular perforation was more frequent in group 1 than in group 2 (p = 0.003). Perforation was also related to longer hospital stay (p = 0.018). Peritonitis occurred in 21/29 (72%), post-operative ileus in 5/29 (17%) and sepsis in 4/29 (14%) patients without any difference between groups. In the whole cohort, hospital stay correlated negatively with age (p = 0.000). There was no mortality. CONCLUSIONS: Among preschool children, those younger than 48 months present with longer duration of pre-admission symptoms indicating longer infection course than in older children. Altered general state and higher degree of tachycardia in the younger reflect higher systemic repercussions of the illness. Less specific abdominal pain and dissociation of the inflammatory markers with lower leucocyte- and neutrophil counts and higher C-reactive protein levels in the younger may contribute to further diagnosis delay and higher rate of perforation in these patients.

Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities.

BACKGROUND: Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years. MATERIAL AND METHODS: Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences. RESULTS: Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03). CONCLUSIONS: SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.

Concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma.

A 10-year-old girl with a family history of Hodgkin's lymphoma presented with a 2 month history of cervical lymphadenopathy and weight loss. Biopsy indicated concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma. Such an association is rare, especially so in children, but is not an isolated phenomenon, thereby prompting the question of whether Langerhans cell histiocytosis is a reactive or a neoplastic process.

Thoracoscopy for Pediatric Thoracic Neurogenic Tumors-A European Multi-Center Study.

OBJECTIVES: To assess the efficacy of thoracoscopy and the outcome for children with thoracic neurogenic tumors. METHODS: We performed a retrospective review of 15 European centers between 2000 and 2020 with patients who underwent thoracoscopy for a neurogenic mediastinal tumor. We assessed preoperative data, complications, and outcomes. Results were expressed with the median and range values. RESULTS: We identified 119 patients with a median age of 4 years old (3 months-17 years). The diameter was 5.7 cm (1.1-15). INRG stage was L1 n = 46, L2 n = 56, MS n = 5, M n = 12. Of 69 patients with image-defined risk factors (IDRF), 29 had only (T9-T12) locations. Twenty-three out of 34 patients with preoperative chemotherapy had an 18 mm (7-24) decrease in diameter. Seven out of 31 patients lost their IDRF after chemotherapy. Fourteen had a conversion to thoracotomy. The length of the hospital stay was 4 days (0-46). The main complications included chylothorax (n = 7) and pneumothorax (n = 5). Long-term complications included Horner's syndrome (n = 5), back pain, and scoliosis (n = 5). Pathology was 53 neuroblastomas, 36 ganglioneuromas, and 30 ganglioneuroblastomas. Fourteen had a postoperative residue. With a median follow-up of 21 months (4-195), 9 patients had a recurrence, and 5 died of disease. Relapses were associated with tumor biology, histology, and the need for chemotherapy (p = 0.034, <0.001, and 0.015, respectively). Residues were associated with preoperative IDRF (excluding T9-T12 only) and the need for preoperative chemotherapy (p = 0.04 and 0.020). CONCLUSION: Our results show that thoracoscopy is safe, with good outcomes for thoracic neurogenic tumors in selected cases. Surgical outcomes are related to the IDRFs, whereas oncologic outcomes are related to tumor histology and biology.

Outcome of alimentary tract duplications operated on by minimally invasive surgery: a retrospective multicenter study by the GECI (Groupe d'Etude en Coeliochirurgie Infantile).

BACKGROUND: Alimentary tract duplications (ATD) are a rare cause of intestinal obstruction in childhood. There are many case reports but few series about laparoscopy or thoracoscopy for ATD. The aim of our study was to report the outcome of minimally invasive surgery (MIS) for ATD. METHODS: This was a retrospective multicenter study from the GECI (Groupe d'Etude en Coeliochirurgie Infantile). We reviewed the charts of 114 patients operated on by MIS for ATD from 1994 to 2009. RESULTS: Sixty-two patients (54 %) had a prenatal diagnosis. Forty-nine patients (43 %) were symptomatic before surgery: 33 of those patients (63 %) with postnatal diagnosis compared to 16 (25 %) with prenatal diagnosis (P < 0.01). In this last group, the median age at onset of symptoms was 16 days (range = 0-972). One hundred and two patients had laparoscopy (esophageal to rectal duplications) and 12 patients had thoracoscopy for esophageal duplications. The mean operative time was 90 min (range = 82-98). There were 32 (28 %) resection anastomoses, 55 (48 %) enucleations, and 27 (24 %) unroofings. The conversion rate was 32 %, and in a multivariate analysis, it was significantly higher, up to 41 % for patients weighing <10 kg (P < 0.01). Ten patients (8 %) had unintentional perioperative opening of the digestive tract during the dissection. Eight patients had nine postoperative complications, including six small bowel obstructions. The median length of hospital stay was 4 days (range = 1-21) without conversion and 6 days (range = 1-27) with conversion (P = 0.01). The median follow-up was 3 months (range = 1-120). Eighteen of the 27 patients who underwent partial surgery had an ultrasound examination during follow-up. Five (18 %) of them had macroscopic residue. CONCLUSION: This study showed that MIS for ATD is feasible with a low rate of complications. Patients with prenatal diagnosis should have prompt surgery to prevent symptoms, despite a high rate of conversion in small infants.

Sternal cleft: prenatal multimodality imaging.

Sternal clefts have been reported sporadically, but there are no reports describing complete investigations of the malformation. We describe a child with isolated inferior sternal cleft diagnosed at 33 weeks of gestation and thoroughly investigated by prenatal US, MRI and CT and preoperative US. Our report highlights the importance of accurate and in-depth investigation by multimodality imaging that allows detection of accompanying serious anomalies and, hence, forms the basis for informed parental counselling and for postnatal interdisciplinary care.

Necrotizing myositis case report and brief literature study.

Necrotizing myositis is an extremely rare soft tissue infection, mainly caused by Group A Streptococci. Although its presentation is nonspecific and seems harmless, it quickly leads to death in almost all cases. Therefore, diagnosis and treatment of necrotizing myositis are considered as medical emergencies. The 27 years old patient we report benefited from early diagnosis and care. Necrotic tissues were surgically removed 24 hours after the appearance of the first clinical signs. Intravenous antibiotherapy as well as immunoglobulin therapy were also given on the first day. Starting from this clinical case, we present a brief explanation of the pathogenesis, the key clinical features and appropriate tools for diagnosis. Then, adequate antibiotherapy, role of immunoglobulin therapy and interest of hyperbaric oxygenotherapy will be discussed.

Minimally invasive surgery for anorectal malformation in boys: a multicenter study.

INTRODUCTION: The laparoscopic pull-through for high imperforate anus in boys has recently been developed in France and Benelux. The purpose of this study was to evaluate technical difficulties and surgical complications of this approach. PATIENTS AND METHODS: This report was based on a retrospective study from January 2002 to December 2007,including 34 patients from 10 centers. The rectourinary fistula found in 33/34 cases was bulbar in 10, prostaticin 20, and cervical in 3. RESULTS: Patients were 3.7 months old (range, 1.6-10.4) at surgery. Fistula was cut without closure in 11 cases or divided and closed on the genitourinary side with a suture in 15, with clips in five and linear stapler in two.Median operative duration was 152 mm (range, 80-410). The main per-operative complication, occurring in five cases, was leakage of stools from the rectum after the fistula section, requiring in one case a second laparoscopic procedure for washing and for all five cases intravenous antibiotics. Median hospital stay was 6 days(range, 3-15). During the follow-up, patients experienced eight anal stenoses and three prolapses, requiring in three cases perineal redo surgery. CONCLUSION: The laparoscopic approach of high imperforate anus allowed an easy control of the fistula and helped to determine the accurate passage through the levator ani. Nevertheless, technical points have to be improved,such as coprostasis before fistula section to avoid stool leakage, rectum tacking to prevent prolapse,and calibration of muscle channel to avoid stenosis.

Laparoscopic Magenstrasse and Mill Gastroplasty (M&M): Midterm Results.

BACKGROUND: The Magenstrasse and Mill gastroplasty (M&M) is a gastric restrictive procedure without band or stomach resection. Short-term evaluation of the laparoscopic procedure showed low morbidity and satisfactory results on weight loss. Evidence of the validity of the technique in the longer term is scarce. METHODS: Data from patients who underwent M&M procedure from May 2012 to September 2015 were retrospectively reviewed. Preoperative clinical characteristics and data up to 4 years after operation were analyzed. RESULTS: A total of 132 patients were included in this study with a mean age of 46 ± 13.4 years. The mean body mass index (BMI) at the time of procedure was 43 ± 4.5 kg/m2. Mean percentage of excess weight loss (%EWL) was 67, 67, 58, and 57% at 1, 2, 3, and 4 years, respectively. The remission rate for diabetes was 36%. About half of the insulin-dependent patients could stop their insulin treatment. Hypertension was resolved in 33.8% of the patients after 4 years. Incidence of vitamin and mineral deficiency was low throughout the study period, less than or equal to 3% for vitamin B12 and 1% for ferritin. Incidence of gastroesophageal reflux did not exceed 15% during the study. Over 75% of the patients reported a good or very good quality of life following the surgery. CONCLUSION: These results confirm the validity of M&M as a bariatric procedure. The low incidence of vitamin deficiencies and gastroesophageal reflux might be the important asset of M&M over other existing techniques.

[Pseudopapillary tumor of the pancreas revealed by rupture of esophageal and gastric varices secondary to biliary cirrhosis due to bile duct compression]. / Tumeur pseudo-papillaire pancréatique révélée par la rupture de varices oeso-gastriques secondaire à une cirrhose biliaire par une compression cholédocienne.

Pseudo-papillary tumors of the pancreas are rare and usually occur in young women. We report a case with a very rare presentation (rupture of esogastric varices complicating biliary cirrhosis secondary to bile duct compression by a pancreatic tumor). After biological and radiological explorations, a duodenopancreatectomy was performed. Diagnosis was confirmed by conventional histology and immunohistochemistry. One year later, the patient remained asymptomatic.

Application of laser capture microdissection in genetic analysis of neuroblastoma and neuroblastoma precursor cells.

Recently developed quantitative and high-throughput technologies that allow automated and rapid screening of the whole genome, transcriptome and proteome have revolutionized the field of cancer genetics. At the same time, new challenges are met, e.g. the need for improved data analysis and standardization of tumor sample handling. Even if these issues are resolved, an 'old' problem in genetic tumor analysis remains, i.e. contamination of tumor samples by stromal and surrounding normal cells. To overcome this obstacle, laser capture microdissection (LCM) has been developed in order to procure the cells of interest from stained tissue sections with retention of morphology. In this review we describe the possible down-stream applications of LCM in the genetic analysis of neuroblastoma (NB). Special focus is given to MYCN copy number determination using real-time quantitative polymerase chain reaction (Q-PCR), analysis of 1p-, 3p- and 11q-deletions using loss of heterozygosity analysis and Q-PCR expression analysis of microdissected normal neuroblast cells and NB cells.

Congenital absence of the left pericardium and diaphragmatic defect in sibs.

Congenital absence of the left pericardium (allowing communication between pericardial and pleural cavities) is a rare developmental defect that results from faulty partitioning of the pleuropericardic cavity during the 5th week of development. It occurs sporadically in most instances, and may be associated with other malformations of the thoracic viscera. We report here two sibs born to consanguineous parents with absent left fibrous pericardium and developmental defects of the septum transversum: left posterolateral diaphragmatic hernia in one child, left diaphragmatic eventration in the other sib. This appears to be the first familial report of this rare association.

Cecostomy button for antegrade enemas: survey of 29 patients.

OBJECTIVE: This study evaluated the Trap-door button use (Cook Medical, Bloomington, IL) for antegrade enemas in children. METHODS: Since 2002, patients with fecal incontinence or encopresis and constipation underwent percutaneous cecostomy under laparoscopy using a button. Technical details are described. Age at surgery, operative time, hospital stay, diagnosis, indications for cecostomy, and duration of follow-up were recorded. A survey was proposed via a questionnaire that was sent to the patients. Patients wearing the button for less than 1 month were excluded from this evaluation. The survey concerned volume and frequency of enemas, difficulties encountered, benefits and disadvantages of this method, and assessment of the antegrade enemas in continence. RESULTS: Twenty-nine patients, 18 males and 11 females, aged 3 to 21 years (mean, 8.5 years) underwent laparoscopic Trap-door button placement. The indications for all the patients were intractable fecal incontinence in 24 cases and constipation with encopresis in 5 cases. Incontinence was because of myelomeningocele (n = 10), anorectal malformations (n = 11), caudal regression syndrome (n = 1), 22q11 syndrome (n= 1), and Hirschsprung disease with encephalopathy with convulsions (n = 1). Constipation with encopresis was because of sacrococcygeal teratoma (n = 1), cerebral palsy (n = 1), and acquired megarectum with psychiatric and social disorders (n = 3). A total of 26 cecostomy button placements and 3 sigmoidostomy button placements were successful with no intraoperative complication. The mean operative time was 25 minutes (10-40 minutes), and the hospital stay was 2.5 days (1-4 days). Twenty-two parents or patients answered the questionnaire. At the time of this survey, 2 patients had improved their fecal continence and had had the button removed. A mean of 4 weekly enemas was enough to improve fecal continence troubles (range, 1 daily to 1 for 2 weeks). The volume for enemas was 250 to 1000 mL (mean, 700 mL). The time required for the irrigation of the bowel by gravity took from 5 to 60 minutes (mean, 25 minutes) for 20 patients. Before surgery, 14 patients needed a diaper, day and night, and 6 needed sanitary protection. Soiling was a very significant inconvenience for all the patients. After surgery, only 5 patients needed a diaper (cerebral palsy, 22q11, cloacal malformation, myelomeningocele, bladder exstrophy) because of moderate results or urinary incontinence and continued soiling. Patients were asked to give an assessment (null = 0, bad = 1, fair = 2, good = 3, very good = 4). None of the patients felt there had been no changes or a bad result. There were 5 patients who felt they had an average result, 5 a good result, and 12 a very good result. The mean grade was 3.44 (17.2/20). A total of 3 patients had hypertrophic granulation tissue formation around the cecostomy button, and 12 had tiny leakage. CONCLUSION: Percutaneous placement of a cecostomy button under laparoscopic control is an easy and major complication-free procedure. The use of the Trap-door device by the patients or with the help of the parents for antegrade enemas is effective and satisfactory. It improves the quality of life and is reversible.