Surgical treatment of neuroblastoma: twenty-three years of experience at a single institution.

PURPOSE: Neuroblastoma (NB) is treated with surgery, chemotherapy and radiotherapy. We assessed the effects of surgical resection on the outcome over a 23-year period at our institution. METHODS: 85 children were included with a median age at diagnosis of 2.0 (range 0.1-15) years. We assessed the correlation of the complete surgical resection (CR) rate, metastases, NMYC amplification (NMYCA) and chemotherapeutic response with the 5-year overall survival (OS). RESULTS: The INSS stage of NB was 1 in 11 (13 %) patients, 2 in 10 (11 %), 3 in 13 (17 %), 4 in 46 (53 %) and 4S in five patients (6 %). Fifty-two (61 %) patients had high-risk NB and 22 (26 %) had NMYCA. The resection was complete in 72 (85 %) patients, incomplete (ICR) in seven (8 %) and six (7 %) patients did not undergo surgery. Fifty-five patients were administered neoadjuvant and 61 were administered adjuvant chemotherapy (high-dose, n = 50). The OS (5 year) was 68 %: stage 1 (100 %), 2 (90 %), 3 (77 %), 4 (52 %), 4S (80 %) and high-risk NB (52 %). The OS in high-risk NB patients was correlated with a good chemotherapeutic response of the primary tumour, with a RR for mortality = 0.3 (95 % CI 0.1-0.7; p = 0.01), but not with the CR, which had an RR = 0.9 (95 % CI 0.3-2.4; p = 0.84). CONCLUSIONS: The OS in high-risk NB patients was related to a good histological chemotherapeutic response, but not with complete excision of the primary tumour.

Vocal Cord Paralysis after Repair of Esophageal Atresia.

OBJECTIVE: Etiology of vocal cord paralysis (VCP) and laryngeal dysfunction may be congenital or surgical trauma of recurrent and superior laryngeal nerves. We assessed the incidence, risk factors, and morbidity of VCP after repair of esophageal atresia (EA). METHODS: Medical records of 201 EA patients from 2000 to 2022 were reviewed for this retrospective study. Postrepair vocal cord examination (VCE) included awake nasolaryngeal fiberoscopy by otolaryngologist or laryngoscopy under spontaneous breathing anesthesia. Before 2017, postoperative VCE was performed in symptomatic patients only and routinely after 2017. MAIN RESULTS: Overall, VCE was performed on 79 (38%) patients (52 asymptomatic), whereas 122 asymptomatic patients underwent no VCE. VCP was diagnosed in 32 of 79 patients (right 12, left 10, and bilateral 10; symptomatic 25 and asymptomatic unilateral 7) corresponding with extrapolated overall VCP incidence of 16 to 24% among 201 patients including asymptomatic ones. Ten patients (bilateral VCP 8 and left VCP 2) required tracheostomy. Of 10 patients with bilateral VCP, three underwent laryngotracheal expansion surgery (left VC lateralization in one and laryngoplasty in two with acquired subglottic stenosis), three remained tracheostomy dependent, three were off tracheostomy, and one died of complications after redo esophageal reconstruction. All patients with unilateral VCP managed without tracheostomy. Cervical dissection or ostomy formation was a major risk factor of VCP. CONCLUSION: Repair of EA is associated with a considerable risk of VCP and associated morbidity. Cervical EA surgery significantly increased the risk of VCP. Bilateral VCP may eventually require laryngotracheal expansion surgery.

Dynamic defecography in the diagnosis of paediatric rectal prolapse and related disorders.

BACKGROUND AND AIM: Paediatric rectal prolapse (RP) is rarely a diagnostic problem and resolves often spontaneously. We studied whether the assessment of recurrent RP (RP), postoperative relapsed RP (RRP) or anorectal discomfort without RP (ARD) benefits from dynamic defecography (DD) and describe DD findings in relation with outcome. PATIENTS AND METHODS: Fifteen patients (7 males), median age of 10 (range 3.7-15) years, underwent 19 DD with a synchronic small bowel contrast study. Indications for DD were RP (n = 11), RD (n = 3) and RRP (n = 1). Three patients had solitary rectal ulcer and one juvenile rectal polyps (n = 1). Three patients underwent a total of four postoperative DD because of suspected relapse. RESULTS: In 11 patients with a clinically diagnosed prolapse DD displayed a simple prolapse (n = 3), prolapse with enterocele (n = 1), prolapse with small bowel interposition (n = 1), rectal intussusception with anterior rectocele (n = 1) and rectal intussusception (n = 1) and no pathology (n = 4) (37 %). In four patients with ARD DD displayed rectal prolapse originating from sigmoid intussusception (n = 1), enterocele (n = 1) and anterior rectocele (n = 1) and no pathology in one. Median follow-up was 8.1(range 3.0-44) months. Ten patients underwent surgery. Three patients with RP underwent simple laparoscopic rectopexy, five with RP or RD with enterocele or anterior rectocele had rectopexy with anterior peritoneoplasty and two (RP n = 1, RRP n = 1) with sigmoid intussusception had sigmoid resection with rectopexy. Two symptomatic patients (RP, negative DD) are scheduled for rectopexy. Three patients PPRP (n = 2) RD (n = 1) had spontaneous cure. Postoperative DD confirmed relapsed RP in one patient. CONCLUSION: In patients, RP and associated disorders' DD can disclose significant pathology (enterocele, rectocele or sigmoid intussusception) and thereby guide surgical treatment, and should be included in the pre-treatment assessment.

Botox injection treatment for anal outlet obstruction in patients with internal anal sphincter achalasia and Hirschsprung's disease.

BACKGROUND AND AIM: Botox injection treatment (BIT) is a potentially effective but yet unproven treatment of functional anal outlet obstruction that is caused by non-relaxing internal anal sphincter. We present a single institution experience of BIT from 2005 to 2008. PATIENTS AND METHODS: Sixteen patients (11 males), eight with Hirschsprung's disease (HD) (one with total colon aganglionosis, TCA) and eight with internal sphincter achalasia (ISA) were included. Median ages were 3.8 years (0.4-9.3) for HD and 8.1 years (range 1.5-11.4) for ISA. ISA was defined as the absence of rectoanal inhibitory reflex with normal rectal biopsies. Seven HD patients had previous coloanal pull-through (CAPT), and one (TCA) colectomy and ileoanal J-Pouch anastomosis. Two of the ISA patients had undergone internal sphincter myectomy and two had Malone procedure [antegrade colonic enema (ACE)]. Indication for BIT in 16 patients was anal outlet obstruction (n = 11) with soiling and recurring HD-associated enterocolitis (n = 5) and in one patient (HD, TCA) soiling with enterocolitis (n = 1). Before BIT, all patients underwent anorectal manometry, rectal biopsies and barium enema. The effect of BIT was evaluated after 2 months and BIT was repeated if necessary. Effect of BIT was scored as follows: 0 no, 1 little, 2 significant effect and 3 symptoms disappeared. RESULTS: Median follow-up was 19 months (range 3-43). The median number of injections was two per patient (range 1-4) and the median Botox dose was 80 U (range 40-100). Scores of BIT effect were 3 or 2 in five (31%) and 0 or 1 in 11 (69%). After adjunctive treatment modalities (myectomy n = 1, CAPT n = 1, adjusted ACE/laxative treatment), the end result was good or satisfactory in 11 (69%) but remained poor in 5 (31%) patients. Patient age, diagnosis, anorectal resting pressure or findings in barium enema were not correlated with BIT efficiency score (R range -0.06 to 0.39, P = 0.12-0.91). CONCLUSION: Although successful in some patients, the role of BIT remains undetermined. It is difficult to predict which patients will profit from BIT. Continuing other treatment modalities after BIT may improve the results.

Antegrade continence enema in the treatment of congenital fecal incontinence beyond childhood.

PURPOSE: The role of antegrade continence enema for the treatment of congenital fecal incontinence in adult patients remains unclear. MATERIALS: Twenty-seven patients, median age 19 (range, 17-43) years, with congenital fecal incontinence underwent surgery for antegrade continence enema and were prospectively followed up for functional outcome after a median of 25 (range, 3-117) months. RESULTS: The diagnoses included myelodysplasia (n = 14), anorectal malformations (n = 6), and others (n = 7). Antegrade continence enema conduits included appendicostomy (n = 22) and cecal (n = 2), ileal (n = 2), and sigmoid (n = 1) tubes. Thirteen (48 percent) patients had complications. Eighteen (66 percent) patients became fully continent, six (23 percent) had minor, and three (11 percent) major soiling. Antegrade continence enema became unnecessary in three patients (11 percent). Treatment with antegrade continence enema failed in three cases. Of the 21 patients who continued with antegrade continence enema, 16 (76 percent) are fully continent, and bowel function and quality of life was improved in 15 (71 percent) and 13 (62 percent) patients, respectively. The scores of convenience (1 = easy, 5 = difficult) and overall satisfaction (1 = poor, 10 = excellent) were median 2 (range, 1-4) and 8 (range, 3-10). CONCLUSIONS: Despite numerous complications and occasional treatment failures, 90 percent of adult patients with congenital fecal incontinence benefited from antegrade continence enema.

Outcome of Surgery for Pediatric Gastroesophageal Reflux: Clinical and Endoscopic Follow-up after 300 Fundoplications in 279 Consecutive Patients.

PURPOSE: Clinical and endoscopic assessment of the outcome after fundoplication for pediatric gastroesophageal reflux. BASIC PROCEDURES: Hospital records of 279 consecutive patients who underwent fundoplication for gastroesophageal reflux from 1991 to 2014 were reviewed. Underlying disorders, clinical and endoscopic findings, imaging studies, pH monitoring, and surgical technique were assessed. Main outcome measures were patency of fundoplication, control of symptoms and esophagitis, complications, redo operations, and predictive factors of failures. MAIN RESULTS: A total of 279 patients underwent 300 fundoplications (277 primaries and 23 redos). Underlying disorders in 217 (72%) patients included neurological impairment (28%) and esophageal atresia (22%). Indications for fundoplication included recalcitrant gastroesophageal reflux symptoms (44%), failure to thrive (22%), respiratory symptoms (15%), esophageal anastomotic stricture (4%), apneic spells (2%), and regurgitation (2%). Preoperative endoscopy was performed in 92% and pH monitoring in 49% of patients. Median age at primary fundoplication was 2.2 ((IQR = 0.5-7.5)) years. Fundoplication was open in 205 (74%; Nissen n = 63, Boix-Ochoa n = 97, Toupet n = 39, and other n = 6), laparoscopic in 72 (24%; Nissen n = 67 and Toupet n = 5), and included hiatoplasty in 73%. Clinical follow-up was a median of 3.9 (IQR = 1.2-9.9) years. Mortality related to surgery was 0.3%. Symptom control was achieved in 87% of patients, and esophagitis rate decreased from 65% to 29% (p < 0.001). Fundoplication failed in 41 (15%) patients. Failure was predicted by esophageal atresia risk ratio = 3.9 (95% confidence interval = 1.3-11, p = 0.01), any underlying disorder risk ratio = 3.1 (95% confidence interval = 1.1-9.1, p = 0.04), and hiatoplasty risk ratio = 2.6 (95% confidence interval = 1.1-6.6, p = 0.03). Of the 23 redo-fundoplications, 32% failed. CONCLUSION: The majority of patients who underwent fundoplication had an underlying disorder. Primary fundoplication provided control of symptoms in almost 90% of patients and also reduced the rate of esophagitis. Failure of primary fundoplication occurred in 15% of patients, and an underlying disorder, esophageal atresia, and hiatoplasty increased the risk of failure.

Cholesterol, non-cholesterol sterols and bile acids in paediatric gallstones.

BACKGROUND: Depending on underlying aetiopathogenetic factors human gallstones contain various amounts of cholesterol, non-cholesterol sterols and bile acids, which have remained unexplored in paediatric gallstone patients. AIMS: To evaluate sterol and bile acids compositions of paediatric gallstones. PATIENTS AND METHODS: Study group included 21 consecutively cholecystectomised children. Gas-liquid chromatography was used to quantitate gallstone sterols and bile acids. Results were compared to adult gallstones (n=194). RESULTS: Cholesterol stones (n=9) had higher proportions of cholesterol and lathosterol, but lower those of lanosterol and phytosterols than pigment stones (n=12) (p<0.05 for each). Patients with gallstone cholesterol content over 70% were female. Gallstone cholesterol positively reflected body mass index and, in cholesterol stones-group, age (r=approximately +0.700, p<0.05). Three patients on parenteral nutrition had brown pigment stones consisting of high amounts of campesterol and sitosterol ranging 483-9303 microg/100 mg of stone. Pigment stones had 13-fold higher amount of bile acids than cholesterol stones (p<0.05). Black pigment stones contained approximately 3-fold higher phytosterol proportions, and pigment stones and cholesterol stones had approximately 43% lower proportions of deoxycholic acid than adults (p<0.05). CONCLUSION: Gallstones in patients on parenteral nutrition are rich in phytosterols. With respect to gallstone sterols, gallstone disease of adolescent girls resembles that of adults. Composition of bile acids in paediatric gallstones is different from adults.