Francesco Molinaro

Postoperative bowel obstruction after laparoscopic and open appendectomy in children: a 15-year experience

PURPOSE The purpose of the study was to determine and evaluate the incidence of postoperative bowel obstruction (PBO) after laparoscopic and open appendectomy in children. MATERIAL AND METHODS The medical files of children who have undergone an appendectomy, either via the laparoscopic or open approach, at our department from 1992 until 2007 were reviewed. Collected data included age at appendectomy, initial surgical approach, time interval to PBO, and type of definitive treatment. The incidences of PBO after laparoscopic and open appendectomy were compared with the chi(2) analysis. RESULTS From the 1684 children who were found, 1371 had nonperforated appendicitis and 313 had perforated appendicitis. Laparoscopic appendectomy was performed in 954 patients of the nonperforated group and in 221 of the perforated group. Open appendectomy was performed in 417 and 92 patients of the 2 groups, respectively. Overall, the incidence of PBO development was 2.2%. In the laparoscopic appendectomy population, a significantly low incidence of 1.19% of PBO development was detected, compared with the 4.51% of the open appendectomy group (P < .0001). CONCLUSION Laparoscopic appendectomy diminishes the potential of PBO development. The overall incidence of PBO is not related to the severity of the disease but only to the initial operative approach.

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.

Untangling the web of systemic autoinflammatory diseases.

The innate immune system is involved in the pathophysiology of systemic autoinflammatory diseases (SAIDs), an enlarging group of disorders caused by dysregulated production of proinflammatory cytokines, such as interleukin-1β and tumor necrosis factor-α, in which autoreactive T-lymphocytes and autoantibodies are indeed absent. A widely deranged innate immunity leads to overactivity of proinflammatory cytokines and subsequent multisite inflammatory symptoms depicting various conditions, such as hereditary periodic fevers, granulomatous disorders, and pyogenic diseases, collectively described in this review. Further research should enhance our understanding of the genetics behind SAIDs, unearth triggers of inflammatory attacks, and result in improvement for their diagnosis and treatment.

Herlyn–Werner–Wunderlich syndrome: An “early” onset case report and review of Literature

Highlights • We describe a case of a 3 years old girl, with all features of Herlyn–Werner–Wunderlich syndrome (HWWS) who came to our attention for lower abdominal mass.• Only six cases are reported in Literature with early onset of this syndrome under 5 years.• The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment.• The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis.• An error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome.• Our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cisto-vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis.

Preoperative distraction in children: hand-held videogames vs clown therapy.

Anxiety in children undergoing surgery is characterized by feelings of tension, apprehension, nervousness and fear which may manifest differently. Postoperative behavioural changes such as nocturnal enuresis, feeding disorders, apathy, and sleep disturbances may stem from postoperative anxiety. Some Authors pointed out that over 60% of children undergoing surgery are prone to developing behavioural alterations 2 weeks after surgery. Variables such as age, temperament and anxiety both in children and parents are considered predictors of such changes.1 Studies were published describing how psycho-behavioural interventions based on play, learning and entertainment in preparing children for surgery, may reduce preoperative anxiety. Clown-therapy is applied in the most important paediatric facilities and has proved to diminish childrens emotional distress and sufferance, as well as consumption of both analgesics and sedatives and to facilitate the achievement of therapeutic goals. The aim of our study was to evaluate the efficacy of clown-therapy during the childs hospital stay, with a view to optimizing treatment and care, preventing behavioural alterations and enhancing the childs overall life quality.

Laparoscopic pyeloplasty for repair of pelviureteric junction obstruction in children.

PURPOSE The aim of this study was to report our initial experience with laparoscopic pyeloplasty (LP) in children with pelviureteric junction (PUJ) obstruction and describe the evolution and evaluate the results. MATERIALS AND METHODS We retrospectively reviewed the records of 32 consecutive infants and children with unilateral ureteropelvic junction obstruction and deterioration of renal function on isotope renography, who underwent LP (19 on the right, 13 on the left) between May 2003 and January 2007. Twenty-three were males and 9 females. The mean age was 7.7 years old (range, 2 months to 17 years); the patient was placed in a three quarter lateral position and three ports were used. The PUJ was resected and the anastomosis was made by using absorbable sutures. A JJ stent was inserted by laparoscopy in most patients. Follow-up included clinical and ultrasound assessment, followed by isotopic renography at 6 months. RESULTS LP was feasible in 29 of 32 patients (91%). The procedure could not be completed by laparoscopy in 3 patients; the main reason was difficulty in completing the anastomosis. Only 1 patient with a big redundant renal pelvis underwent a reduction. Stent insertion was successful in all, except 1 patient. An aberrant crossing vessel was found in 12 patients. We held up the aberrant crossing vessel and PUJ with two- or three-point-not absorbable-sutures, without the needed pyeloplasty in 2 of them. The other 10 underwent a LP enabled ureteric transposition. Three patients presented with postoperative complications: pyelonephritis in 2 patients and PUJ leakage in 1 who underwent nephrostomy with a further uneventful course. Mean operative time was 152 minutes (range, 120-270), and average hospital stay was 4.7 days (range, 1-8). In 1 patient, cystoscopy showed that the JJ stent was not in the bladder at the time of removal, and ureteroscopy was used to retrieve it. Mean follow-up was 22 months (range, 2-56). A total of 29 patients (91%) were asymptomatic after removal of the double JJ stent, showing a reduction of the degree of hydronephrosis in all patients, and had also improved PUJ drainage on isotope renography or sonography. CONCLUSIONS LP is effective and safe in children with minimal morbidity and gives excellent short-term results. The feasibility is also excellent in patients younger than 1 year. The transabdominal approach revealed good exposition without disadvantages for the patient.

The protean ocular involvement in monogenic autoinflammatory diseases: state of the art

Ocular involvement is frequent in the monogenic autoinflammatory disorders and generally occurs as spontaneously recurring inflammatory events at different ocular sites caused by the aberrant release of proinflammatory cytokines, mainly IL-1β. Over the past decade, we witnessed a significant growth of eye abnormalities associated with idiopathic granulomatous disorders, familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, mevalonate kinase deficiency, and cryopyrin-associated periodic syndrome. The pathogenetic mechanisms of these disorders have shown the evidence of disrupted cytokine signaling, but the explanation for the heterogeneous ocular involvement remains to be elucidated. We herein review the monogenic autoinflammatory disorders affecting the eye, describing their main clinical features with specific regard to the ocular involvement, which can lead to decreased visual acuity and even blindness, if the primary disorder is undetected or left untreated.

Thoracoscopic Thymectomy in Children: Our Preliminary Experience

INTRODUCTION The aim of this study is to present a preliminary series of six children affected by an anterior mediastinal mass, resulting in a thymoma. We treated this pediatric population by a video-assisted thoracoscopic thymectomy. PATIENTS AND METHODS We performed a retrospective study from January 2000 to January 2012 of all children affected by an anterior mediastinal mass, resulting in a thymoma. Data included sex, age at surgery, clinical and radiological features, surgical procedure, follow-up, and complications. RESULTS Video-assisted thoracoscopic thymectomy was performed in six children (five boys and one girl). Four patients presented with dyspnea and/or thoracic pain treated with analgesic and corticosteroid therapy without any benefits. Two patients were asymptomatic. Preoperatively, exams included radiological imaging, blood, and immunological test. All 6 patients were treated with a thoracoscopic approach; of these patients, 5 were treated with a thoracoscopic right-side thymectomy and 1 with a left-side approach. Patients were treated by a three-trocar technique, and thymectomy was performed using Valleylab (now Covidien) (Boulder, CO) LigaSure™. The mean operative time was 120 minutes. The mean hospital stay was 5 days. In all cases histological findings revealed no malignancy. The mean follow-up was 38 months. DISCUSSION Thoracoscopic thymectomy facilitated the goal of early thymectomy. The right-side approach is preferred because it gains a good visualization of the venous anatomy for dissection. The left side allows a good extended resection of both the thymus and perithymic fat. Thoracoscopic thymectomy should be consider a valid, less invasive alternative to the most radical open approach.

Juvenile granulosa cell tumor of the testis: prenatal diagnosis and prescrotal approach.

Neonatal testicular tumors are rare and should be considered in the differential diagnosis of newborn scrotal masses. Juvenile granulosa cell tumor (JGCT) accounts for about 5% of all prepubertal testis tumors. As a benign neoplasm, radical orchiectomy is sufficient for treatment. We report a case of a newborn with a prenatal diagnosis of scrotal mass. After surgery, the histological diagnosis was juvenile granulosa cell tumor. To date the patient is healthy.

Minimally invasive surgery for achalasia: combined experience of two European centers.

Background: The present report summarizes 12 cases with achalasia treated with laparoscopic Heller myotomy and anterior fundoplication according to the method of Thal and Dor. Patients and Methods: From 1997 to 2005 at 2 institutions in Europe, 12 patients (7 male and 5 female, ages ranging from 3.5 to 7 years) were treated for esophageal achalasia (EA) with laparoscopic Heller myotomy and anterior fundoplication according to Thal and Dor. In 1 case a perforation of the esophageal mucosa occurred, which was promptly treated during surgery. Follow-up ranged from 18 to 60 months and included clinical examination and barium radiography of the upper digestive tract. Results: Postoperatively, 10 patients showed a normal weight curve and complete resolution of symptoms. All patients showed complete regression of digestive and respiratory symptoms from the first examination, with a normal oral meal intake and an improvement of weight and height parameters. One case required repeat intervention after 2 years because of persisting pain; in this case surgery revealed an insufficient myotomy. Conclusions: Modified extramucosal Heller cardiomyotomy associated with 180° anterior antireflux plastic surgery (according to Thal and Dors procedures) is a useful and safe procedure in the treatment of EA in pediatric patients. Our data, which are supported by long-term follow-up, also stress the relevance of anterior fundoplication in preventing postoperative gastroesophageal reflux.