Magnetic resonance urography and laparoscopy in paediatric urology: a case series.

Paediatric urology often presents challenging scenarios. Magnetic resonance urography (MRU) and laparoscopy are increasingly used. We retrospectively studied children affected by a disease of the upper urinary tract who after MRU were elected for laparoscopic treatment. This pictorial essay draws on our experience; it illustrates some specific MRU findings and highlights the usefulness of MRU for the diagnosis of upper urinary tract pathology in children. It also offers some examples of the potential additional diagnostic information provided by laparoscopy as well as its therapeutic role.

Provisional unicentric experience with an electronic incident reporting form in pediatric anesthesia.

OBJECTIVE: To present and compare with literature our experience with an electronic anesthesia-related incident reporting form as a quality control measure at Gaslini Children's Hospital over a 19-month period. METHODS: All events that occurred between March 2009 and September 2010 were recorded. We adopted an electronic reporting form included in the online recording process of every anesthetic procedure. Events were divided into near misses and adverse events. Adverse events were further divided into incidents, minor events, and major events. Patients were divided into three age-groups: <1, between 1 and 3, and >3 years. RESULTS: A total of 12,850 anesthetics were performed. Eight (0.06%) near misses and 108 (0.8%) adverse events were reported. Adverse events occurred more frequently in infants. Of 108 events, 35 (32.4%), 61 (56.5%), and 12 (11.1%) were classified as incidents, minor, and major events, respectively. Of all the adverse events, 66 (61%) were respiratory, 27 (25%) organizational, six (5%) drug-related, four (4%) cardiocirculatory, and five (5%) miscellaneous. CONCLUSIONS: Infants were at the highest risk to experience adverse events. Although experimental electronic incident reporting proved to be feasible, there is reason to suspect that there was underreporting of near misses. Overreporting of near miss events may be enhanced by easier and more straightforward reporting forms as well as by better education for anesthetic providers about the importance of recognizing and reporting near misses.

Technical considerations in children undergoing laparoscopic ileal-J-pouch anorectal anastomosis for ulcerative colitis.

BACKGROUND: Sub-total colectomy and restorative proctocolectomy with j-pouch ileo-anorectal anastomosis is the treatment of choice in children with ulcerative colitis uncontrolled with medical therapy. OBJECTIVE: To present some technical considerations about children undergoing laparoscopic ileal-J-pouch anorectal anastomosis. SETTINGS AND PATIENTS: All patients with ulcerative colitis undergoing laparoscopic ileal-J-pouch anorectal anastomosis were evaluated from January 2006 to February 2011. INTERVENTION: The new technical innovations herein are (1) total laparoscopic approach, (2) a very short 3-cm J-pouch ileal reservoir created outside the stoma incision, (3) preservation of the entire anal canal and the Knight-Griffen double stapled anastomosis, less than 3 cm from the dentate line, (4) use of a Multiple Instrument Access Port system in the stoma skin incision to reduce the number of port site incisions and (5) proctectomy performed using only an electrosurgical vessels sealing device thus avoiding clips to close rectal pedicle. RESULTS: Seventeen laparoscopic ileo J-pouch low rectal anastomosis were performed by the same surgical staff. Three complications occurred postoperatively: one bowel obstruction, one ileostomy prolapse, and one anastomotic stricture. Satisfactory functional results were achieved in all, there was no significant perineal excoriation and quality of life was excellent. CONCLUSIONS: A Multiport Instrument Access Port placed in the stoma site allowed the use of more instruments through a single incision. The very short ileo J-pouch low rectal anastomosis has been shown to be a safe, feasible, and effective reconstructive procedure.

Near-miss events are really missed! Reflections on incident reporting in a department of pediatric surgery.

PURPOSE: The aim of this study was to evaluate the frequency of surgical and organizational events that occurred in the whole Department of Paediatric Surgery at Gaslini Children's Hospital through an incident-reporting system in order to identify the vulnerabilities of this system and improve it. MATERIALS AND METHODS: This is a 6-month prospective observational study (1st January-1st July 2010) of all events (including surgical and organizational events, and near misses) that occurred in our department of surgery (pediatric surgery, orthopedics and neurosurgery units). RESULTS: Over a 6-month study period, 3,635 children were admitted: 1,904 out of 3,635 (52.4%) children underwent a surgical procedure. A total number of 111 adverse events and 4 near misses were recorded in 100 patients. A total of 108 (97.3%) adverse events occurred following a surgical procedure. Of 111 adverse events, 34 (30.6%) required re-intervention. Eighteen of 100 patients (18%) required a re-admission, and 18 of 111 adverse events (16.2%) were classified as organizational. Infection represented the most common event. CONCLUSIONS: An electronic physician-reported event tracking system should be incorporated into all surgery departments to report more accurately adverse events and near misses. In this system, all definitions must be standardized and near misses should be considered as important as the other events, being a rich source of learning.

Intraoperative bronchoscopy for bronchial carcinoid parenchymal-sparing resection: a pediatric case report.

Bronchial carcinoid tumors are the most common primary pulmonary neoplasm in the pediatric population. The widely accepted treatment for carcinoid tumors is surgical, specifically aiming at being as much as conservative on lung parenchyma, while the entire tumor is resected. A brief case is described, highlighting the importance and advantages of a surgical and endoscopic combined approach.

Hirschsprung's disease: what about mortality?

PURPOSE: Although significantly decreased during last decades, mortality rate for Hirschsprung's disease still ranges between 1 and 10%. The authors reviewed the main features of patients with Hirschsprung's disease treated in our Institution who died in the period between 1993 and 2010 in order to detect possible risk factors or prevention strategies. METHODS: The notes of all patients with Hirschsprung's disease admitted to our Institution between January 1993 and January 2010 were reviewed. All families were interviewed and possible unknown deaths were recorded. We reported demographic data, length of aganglionosis, type and number of associated anomalies, age and type of onset, age and cause of death, chronological relationship between death and pull-through, and all other significant details. RESULTS: Eight out of a series of 313 patients with Hirschsprung's disease died during the study period (mortality rate = 2.56%). Six patients were male for a male to female ratio of 3:1. Eleven associated anomalies were detected in five patients, including four congenital anomalies of the kidney and urinary tract, four heart, two central nervous system, and one skeletal malformations. One patient had Down Syndrome and one Cat Eye Syndrome. Two patients were born preterm. All patients had neonatal onset with delayed meconium passage. Five patients had aganglionosis confined to rectosigmoid colon (classic forms), two had total colonic aganglionosis and one had total intestinal aganglionosis. Enterostomy was performed in five patients. Median age at death was 75 days (range 30 days-8 years). The cause of death was enterocolitis in four patients, heart failure in three, and intestinal failure in one. Six patients died before the pull-through. Two patients died postoperatively. All patients but two died at home. CONCLUSIONS: Onset and clinical features do correlate with severity. Newborns and infants seem to be more likely to develop serious life-threatening complications, particularly in case of associated cardiovascular malformations. Although enterostomies do not have protective effects over cardiovascular issues, prophylactic stoma should be considered in high-risk patients. Radical treatment (pull-through) should be performed as soon as possible. Alternatively, protected and cautious discharge of newborns with associated heart anomalies is strongly recommended in order to prevent serious and uncontrolled complications, regardless of the presence of a protective enterostomy. Postoperatively, close follow-up appointments are recommended. Families should be acknowledged and educated for prompt recognition and treatment of severe life-threatening complications.

Laparoscopic approach for children with inflammatory bowel diseases.

BACKGROUND: Minimally invasive surgery is being increasingly applied to inflammatory bowel diseases (IBDs). Few pediatric series from selected research have been described to date. This study describes a unicentric experience of laparoscopic treatment of children with IBDs. MATERIALS AND METHODS: All consecutive patients with IBDs between February 2006 and February 2010 who underwent laparoscopic treatment were included. We reviewed notes and recorded demographic data, indications, perioperative management, surgical details, length of surgery, complications, postoperative management, length of hospitalization and functional outcome. RESULTS: We performed 25 procedures on 16 patients (12 ulcerative colitis, 3 Crohn's disease, and 1 indeterminate colitis). Median age was 12 years. A total of 50% patients underwent elective surgery; 11 underwent staged laparoscopic subtotal colectomy (LSTC) followed by J-pouch ileorectal anastomosis (JPIRA). Three patients underwent straight LSTC + JPIRA. All procedures included protective ileostomy. Length of surgery ranged between 120 and 380 min depending on the procedure (LSTC ± JPIRA). No conversion was required. Length of hospitalization ranged between 3 and 18 days. We observed six complications (24%) mainly represented by adhesions that were effectively treated laparoscopically. Ten patients were restored (ileostomy closure) and were assessed for continence that turned out to be good in 80%. CONCLUSIONS: Laparoscopy proved to be feasible, safe and effective for the treatment of IBD in children. Although we observed a relatively low incidence of complications, stoma site adhesions still remain the major issue, which can be effectively dealt with laparoscopically. Functional outcome as well as cosmesis is satisfactory. As results are encouraging, at present we prefer laparoscopy for the surgical treatment of IBD in pediatric patients.

Nephrectomy for multicystic dysplastic kidney and renal hypodysplasia in children: where do we stand?

OBJECTIVES: Little is reported in literature regarding correct management of benign lesions of the kidney. The aim of our study is to present a series of total and partial nephrectomies performed in the last 5 years and to discuss indications. MATERIALS AND METHODS: Patients with benign lesions who underwent nephrectomy and partial nephrectomy at our institution in the period 2003-2008 were retrospectively included in the study. Notes were carefully reviewed and demographic data, symptoms onset, preoperative diagnosis, investigations, medical and/or surgical treatment, postoperative complications and definitive histological reports were collected. RESULTS: Forty procedures were performed. Twelve patients were preoperatively diagnosed of having multicystic dysplastic kidney (MCDK), which was confirmed in 10, whereas the remaining 28 patients of having severe dysplasia or hypodysplasia. Thirty-four patients underwent total nephrectomy, six underwent partial nephrectomy. Histopathological analysis confirmed segmental or complete abnormalities of the involved kidney in all cases. No malignancies were detected. DISCUSSION: Our study confirmed the extremely low malignancy rate of MCDK and hypodysplastic kidneys. The 20% mismatch of pre- and post-operative diagnosis suggests a common aetiology and shared therapeutic strategies for MCDK and hypodysplasia. At present, there is no consensus regarding correct indications for nephrectomy in paediatric age. As nephrectomy seems not to provide any advantage over preservation, but surgical and anesthesiological risks, we should be prudent in preserving every asymptomatic poorly or non-functioning kidneys maintaining a strict follow-up. Randomised controlled studies on larger multicentric series are strongly warranted to define this topic.

The nuss procedure after breast augmentation for female pectus excavatum.

Pectus excavatum, the most common congenital chest wall malformation, has a higher incidence among men. Since 1987, when Donald Nuss performed his technique for the first time, the minimally invasive approach has become the most widely used technique for treating pectus excavatum. Few reported studies have focused on the repair of female pectus excavatum. Women with pectus excavatum often present with breast asymmetry that may require breast augmentation, either before or after pectus excavatum repair. To the authors' knowledge, no reports on the Nuss procedure after breast implant surgery have been published. This report describes the case of a 26-year-old woman who underwent minimally invasive repair after breast implant surgery. The authors believe that for women with severe pectus excavatum, the Nuss procedure should be the first choice for surgical correction. Moreover, for breast implant patients, this technique is absolutely feasible without major complications.

Poland syndrome with bilateral features: case description with review of the literature.

Poland syndrome (PS) has been described as unilateral pectoral muscle deficiency variably associated with ipsilateral thoracic and upper limb anomalies. Bilateral hypoplasia/aplasia of the pectoralis muscle and upper limb defects in association with variable thoracic muscles, chest wall deformities and lower limb defects have been infrequently reported in the literature. We report on a 3(1/2)-year-old girl with clinical features consisting in bilateral asymmetric pectoral muscle defects (complete agenesis on the left side and agenesis of the sternocostal head on the right side), nipple hypoplasia, left rib defect, and right hand symbrachydactyly. In this study, we reviewed the bilateral features present in our patient and those described in the literature. Hypotheses explaining bilateral features in PS are reviewed.

Bilateral endoclose approach for the stabilization of the bar in pectus repair.

BACKGROUND: Mini-invasive repair of pectus excavatum with Nuss technique is the preferred technique in most centers. One of the most important technical points for the final result is the stabilization of the bar, usually obtained by one or more stabilizers and few stitches fixing the bar to some ribs. Our aim is to show how to get the bar more stable by passing bilaterally some stitches around the bar and the ribs close to it. By a right thoracoscopy and a 30-degree optic, we are able to pass the stitches bilaterally by using an Endoclose needle (Covidien Ltd., Hamilton HM, Bermuda). The left hemithorax is visualized from the right side, pushing the optic through the mediastinum following the bar and staying just below it. MATERIALS AND METHODS: We have operated on 230 patients in two centers. We have used only one stabilizer in more than 90% of the patients. In all patients, we have passed four absorbable stitches on the right side, using an Endoclose needle. In the last 45 cases, we have adopted bilateral fixation of the bar by the Endoclose needle. RESULTS: The maneuver takes only around 10 minutes. We did not have complications related to that maneuver. We did not observe destabilization of the bar in those cases. The overall destabilization rate was 1.3%. CONCLUSIONS: We suggest this technique to give the bar more stability on both sides. We think the use of a bilateral stabilizer can be avoided.

Spleen surgery in pediatric age: seven-year unicentric experience.

BACKGROUND: Either "open" and laparoscopic spleen surgery in pediatric age are well known and performed with ease in children. Yet, few data regarding follow-up and outcome are discussed in the international literature. MATERIALS AND METHODS: Clinical notes of all patient who underwent spleen surgery in a single center between 2000 and 2007 were reviewed and a specific follow-up questionnaire was administered, aiming to evaluate pre- and postoperative data, especially considering underlying disease, cosmetic results, and quality of life after surgery. RESULTS: Fifty-one patients underwent spleen surgery in our series, 33 of whom returned a complete follow-up questionnaire and were included in the study. Splenectomy was performed in 26 patients, whereas 7 patients underwent a partial splenectomy; 19 cases (57.6%) were approached laparoscopically. A total of 4 complications (12%) occurred in our series, none of them being intraoperative. Hospital stay resulted as significantly shorter in laparoscopic cases (5.5 +/- 2.9 vs. 8.7 +/- 4.8 days; P < 0.01), with better results in terms of cosmetics. Quality of life is strictly related to underlying disease, as well as long-term survival. CONCLUSIONS: Whatever surgical approach is adopted, spleen surgery is safe, effective, and reproducible. When feasible, the laparoscopic approach should be preferred to the traditional open approach, as far as efficacy and safety are similar, in order to reduce hospital stay, abdominal wall traumatism, and consequently, improve postoperative pain control and cosmetic results.

Fast-track surgery of the colon in children.

INTRODUCTION: The aim of this study is to present the "fast-track" experience in children who underwent colon resection. MATERIALS AND METHODS: Forty-six children who underwent laparoscopic colon resection were prospectively included in the study. Anomalies of colon innervation and inflammatory bowel disease represented the main surgical indications. RESULTS: Left colon/sigmoid resection was performed in 37, total colon resection was done in 5, and right colon resection in 4 children. Total colon resection was always associated to ileostomy. Anastomosis was performed in 41 cases. Patients were postoperatively monitored for pain, return to normal activity, feeding, bowel movements, and complications. Stool passage and oral feeding were started on postoperative day 1, and all patients were discharged before postoperative day 4. One child was readmitted the day after discharge because of an anastomotic leak. No other major complications were recorded. DISCUSSION: Minimally invasive surgery is safe and effective in pediatric colonic surgery and allows a fast recovery time (fast-track).

Risk management in pediatric surgery.

PURPOSE: To present the experience documented over 1 year of analysis and quality control on surgical complications and organizational accidents. METHODS: All children admitted during the study period at our Institution were included in the analysis, which consisted of four phases: (1) definition and standardization of perioperative diagnostic and therapeutic tracks; (2) staff education; (3) documentation and data implementation, and (4) "Morbidity and Mortality" audit. RESULTS: Over a 1-year study period, 3,116 children were admitted to our Institution: 2,222 out of 3,116 (71.3%) children underwent a surgical procedure. A total number of 184 complications were recorded in 149 patients. One hundred and seventy-one (92.9%) complications occurred following a surgical procedure. Fifty-six out of 149 complicated patients (37.6%) required a re-operation. Thirty-five out of 184 (19%) complications were classified as organizational. Infection represented the most common complication. All cases of anastomotic dehiscence and perforation, bowel obstruction, and stoma malfunction required reintervention. None of the postoperative bleedings required a second surgical procedure. CONCLUSION: Although a proper statistical comparison with literature complication rates is not feasible, our experience confirms the importance of quality-control audit in health care systems. Prolonged observation, long-term follow up, and comparison with previous results will represent our future goal.

Outcome of primary endorectal pull-through for the treatment of classic Hirschsprung disease.

INTRODUCTION: Endorectal pull-through (ERPT) is a widely accepted procedure for the treatment of Hirschsprung's disease (HSCR). This study was aimed at presenting the long-term results of patients with classic HSCR who were operated on with a laparoscopic-assisted Georgeson procedure and to compare them to patients treated with a Soave-Boley procedure. PATIENTS AND METHODS: Patients treated for Hirschsprung disease in the period 1997-2006 with a minimum follow-up of 6 months were prospectively included in this study. Demographic details, associated anomalies, surgical technique, length of aganglionosis, and postoperative complications were collected. A questionnaire was submitted to all families to assess general health, bowel adaptation, fecal and urinary continence, cosmetic results, and patients' and parents' perspective of overall outcome. RESULTS: Overall, 162 patients underwent a pull-through procedure: 25 patients treated with Georgeson and 21 with Soave-Boley ERPT were eligible for this study. Conversion was required in 3 of 28 patients approached laparoscopically. Hospitalization was shorter for patients treated laparoscopically (P < 0.05), whereas length of surgery was comparable. Complication rate was similar for both groups, as well as growth that remained within normal ranges for age. Long-term outcome, in terms of bowel movements, was similar. None of the patients experienced fecal and/or urinary incontinence. Cosmetic results proved to be excellent to good in all patients undergoing the Georgeson and in 67% of patients undergoing the Soave-Boley procedure (P < 0.05). Patients' perspective of overall outcome was excellent in more than 90% of patients from both groups. CONCLUSIONS: Overall results proved to be similar. Likewise, long-term bowel function did not show significant differences. Nonetheless, if we consider hospitalization and cosmetic results, it becomes clear that the minimally invasive approach should be preferred, when possible, to improve patients' comfort, perspective of overall health status, and psychologic acceptance.

Transcriptional regulation of TLX2 and impaired intestinal innervation: possible role of the PHOX2A and PHOX2B genes.

TLX2 (also known as HOX11L1, Ncx and Enx) is a transcription factor playing a crucial role in the development of the enteric nervous system, as confirmed by mice models exhibiting intestinal hyperganglionosis and pseudo-obstruction. However, congenital defects of TLX2 have been excluded as a major cause of intestinal motility disorders in patients affected with intestinal neuronal dysplasia (IND) or pseudo-obstruction. After demonstrating the direct regulation of TLX2 expression by the homeoprotein PHOX2B, in the present work, we have focused on its paralogue PHOX2A. By co-transfections, electrophoretic mobility shift assays and chromatin immunoprecipitation, we have demonstrated that PHOX2A, like PHOX2B, is involved in the cascade leading to TLX2 transactivation and presumably in the intestinal neuronal differentiation. Based on the hypothesis that missed activation of the TLX2 gene induces the development of enteric nervous system defects, PHOX2A and PHOX2B have been regarded as novel candidate genes involved in IND and pseudo-obstruction and consequently analyzed for mutations in a specific set of 26 patients. We have identified one still unreported PHOX2A variant; however, absence of any functional effect on TLX2 transactivation suggests that regulators or effectors other than the PHOX2 genes must act in the same pathway, likely playing a non redundant and direct role in the pathogenesis of such enteric disorders.

Appendicectomy for simple appendicitis: video-assisted or intracorporeal?

BACKGROUND: We reviewed our experience with laparoscopic appendicectomy (LA) to compare the video-assisted and intracorporeal approaches. MATERIALS AND METHODS: One hundred and sixty-one (161) patients undergoing LA for simple appendicitis were considered. The procedure was video-assisted with an extracorporeal appendicectomy in 74 cases operated on during the first 5 years of our experience (group A), whereas it was entirely intracorporeal in the subsequent 87 (group B). In the latter group, the dissection of the mesoappendix was accomplished by using titanium clips in 38 cases, with monopolar coagulation in 42 and other devices in 7. The base of the appendix was closed by using endoloops in 11 patients and a stapler in 76. In all the intracorporeal LAs, the appendix was delivered through a port site. A very low position of the two accessory ports was adopted in 34 group B patients. RESULTS: No difference in outcome was found between the two groups, except in operating time (48 vs. 29 minutes) and length of hospital stay (3 vs. 1 day). The complication rate was not statistically different among the various techniques used in group B to divide the mesoappendix and to close the base of the appendix. The cost of disposable instruments for intracorporeal LA was 3- to 6 folds higher than for the extracorporeal one. An extremely low position of the ports did not interfere with the procedure in any case. CONCLUSIONS: LA for simple appendicitis can be performed safely with many techniques. The intracorporeal procedure allows for a shorter operating time, but can dramatically increase the costs of the disposable instruments that are required.

A Provisional Experience with Robot-Assisted Soave Procedure for Older Children with Hirschsprung Disease: Back to the Future?

Hirschsprung disease is a congenital disease characterized by intestinal aganglionosis of various extents. Most patients are younger than 1 year of age. Though, a minority of cases can be older or even adult. Older the patient the more difficult and prolonged is the endorectal dissection required for the pull-through procedure. Longer surgery leads to longer anal dilatation and trauma with subsequent higher likelihood of continence impairment. The article aims at describing the first case series of robot-assisted Soave procedure, which was adopted as an alternative minimally invasive approach to older patients with Hirschsprung disease. The technical principles are represented by intraoperative seromuscolar leveling biopsies, intracorporeal endorectal cranial dissection, and endorectal pull-through with colo-anal anastomosis. The authors report three procedures that were carried on without complication with a limited requirement for anal dilatation and trauma, given the reduced need for endorectal caudal dissection. The results demonstrate the feasibility of the proposed approach. The robotic approach should be considered as an alternative minimally invasive approach for older children or adults with Hirschsprung disease.

Use of failure modes, effects, and criticality analysis to compare the vulnerabilities of laparoscopic versus open appendectomy.

PURPOSE: To measure the feasibility of using FMECA applied to the surgery and then compare the vulnerabilities of laparoscopic versus open appendectomy by using FMECA. METHODS: The FMECA study was performed on each single selected phase of appendectomy and on complication-related data during the period January 1, 2009, to December 31, 2010. The risk analysis phase was completed by evaluation of the criticality index (CI) of each appendectomy-related failure mode (FM). The CI is calculated by multiplying the estimated frequency of occurrence (O) of the FM, by the expected severity of the injury to the patient (S), and the detectability (D) of the FM. RESULTS: In the first year of analysis (2009), 177 appendectomies were performed, 110 open and 67 laparoscopic. Eleven adverse events were related to the open appendectomy: 1 bleeding (CI: 8) and 10 postoperative infections (CI: 32). Three adverse events related to the laparoscopic approach were recorded: 1 postoperative infection (CI: 8) and 2 incorrect extractions of the appendix through the umbilical port (CI: 6). In the second year of analysis (2010), 158 appendectomies were performed, 69 open and 89 laparoscopic. Four adverse events were related to the open appendectomy: 1 incorrect management of the histological specimen (CI: 2), 1 dehiscence of the surgical wound (CI: 6), and 2 infections (CI: 6). No adverse events were recorded in laparoscopic approach. CONCLUSION: FMECA helped the staff compare the 2 approaches through an accurate step-by-step analysis, highlighting that laparoscopic appendectomy is feasible and safe, associated with a lower incidence of infection and other complications, reduced length of hospital stay, and an apparent lower procedure-related risk.

Thymus tuberculosis poorly responding to anti-mycobacterial therapy in a young girl with primary infection.

Tuberculous infection of the thymus is a rare condition, previously described only in young adults. A case of a young girl with primary pulmonary tuberculosis, hilar and mediastinal lymph node enlargement and a partially necrotic mass in antero-superior mediastinum is described. Treatment with three anti-mycobacterial drugs was started, effective in markedly reducing all the intrathoracic abnormalities with the exception of the antero-superior mediastinal mass. Since the radiographic and ultrasonographic appearance of the thymus tuberculous infection may be indistinguishable from other serious conditions, including lymphoma and thymoma, a diagnostic procedure was performed. Evaluation of the resected specimen showed foci of caseation and multiple granulomas with extensive central necrosis within the thymic tissue.