Functional outcome and health-related quality of life in patients with sacrococcygeal teratoma – a Swedish multicenter study

Background/PurposeThe aims of this study were to evaluate bowel and urinary tract function, to identify predictors for dysfunctional outcome and to evaluate health-related quality-of life (QoL) in patients treated for sacrococcygeal teratomas (SCT).MethodsMedical records of patients with SCT born between 1985 and 2015 treated at three Swedish pediatric surgical centers were reviewed. Questionnaires regarding urinary tract function, bowel function and QoL were sent to patients and parents. Different QoL instruments were used for the different age groups.ResultsTotally 85 patients were identified. Four patients died in the neonatal period. Forty-nine patients answered the questionnaires (60%). Median age at follow-up was 8.9 years (range 3.6–28.8). Bowel dysfunction was reported by 36% and urinary tract dysfunction by 46% of the patients. Univariate analysis revealed that urinary tract dysfunction correlated with gestational age (p = 0.018) and immature histology (p = 0.008), and bowel dysfunction correlated with gestational age (p = 0.016) and tumor size (p = 0.042). Low gestational age was an independent predictor for both urinary tract and bowel dysfunction. Good or very good QoL was reported by 56% of children aged 4–7 years, 90% of children aged 8–17 years and 67% of the adults.ConclusionAlthough a considerable proportion of bowel and urinary tract dysfunction was found, the reported QoL was good in a majority of the patients with SCT. Low gestational age was found to be a predictor for bowel- and urinary tract dysfunction.Level of EvidenceLevel III.     

Urinary tract anomalies and urinary tract dysfunction in children with Hirschsprung disease—Is follow-up indicated?

Background/PurposeUrinary tract function in children with Hirschsprung disease (HD) is rarely considered. Aim: to evaluate the prevalence of urinary tract anomalies and dysfunction in children with HD compared to controls.MethodsThis was an observational cross sectional case–control study. Children with HD who underwent transanal endorectal pull-through technique (TERPT) from 2005 to 2017 were invited to participate. Ultrasound of the urinary tract was performed postoperatively. Children > 4 years were asked to answer a urinary tract function questionnaire. Controls were age-matched healthy children. Ethical approval was obtained.ResultsSeventy two children with HD and TERPT were included. Ultrasound was performed in 58 children (83%) post-TERPT. Ten anomalies were diagnosed in six children (10%). Structural anomalies included abnormal kidney size (7%), renal agenesis (2%), prominent calyces (2%) and renal pelvis anomaly (25). Probable acquired anomalies included hydronephrosis (2%), hydroureter (2%) and parenchymal damage (2%). One child had a prior nephrectomy owing to a Wilms' tumor. All 37 children > 4 years (27 boys and 10 girls), median aged 8 years (range 4–12), answered the questionnaire as did 284 healthy controls (144 boys and 140 girls). Boys with HD reported a higher frequency of enuresis: 65% versus 9% (p = 0.001) and urinary tract infections: 18% versus 3% (p = 0.012). Girls with HD reported enuresis more frequently (60%) than healthy girls (7%) (p = 0.001). Children with HD with constipation reported enuresis more frequently (p = 0.038).ConclusionsUrinary tract anomalies and dysfunction deserve attention in the follow-up of children with HD. We suggest screening for urinary tract anomalies and urinary tract symptoms in follow-up of children with HD.Type of studyTreatment study.LevelIII.     

Outcomes of children with well-differentiated fetal hepatoblastoma treated with surgery only: Report from Children's Oncology Group Trial,AHEP0731

BackgroundHepatoblastoma (HB) requires surgical resection for cure, but only 20–30% of patients have resectable disease at diagnosis. Patients who undergo partial hepatectomy at diagnosis have historically received 4–6 cycles of adjuvant chemotherapy; however, those with 100% well-differentiated fetal histology (WDF) have been observed to have excellent outcomes when treated with surgery alone.Patients and methodsPatients on the Children's Oncology Group non randomized, multicenter phase III study, AHEP0731, were stratified based on Evan's stage, tumor histology, and serum alpha-fetoprotein level at diagnosis. Patients were eligible for the very low risk stratum of surgery and observation if they had a complete resection at diagnosis and rapid central histologic review demonstrated HB with 100% WDF histology.ResultsA total of 8 eligible patients were enrolled on study between September 14, 2009 and May 28, 2014. Outcome current to 06/30/2020 was used in this analysis. The median age at enrollment was 22.5 months (range: 8–84 months) and the median AFP at enrollment was 714 ng/ml (range: 18–77,747 ng/mL). With a median follow-up of 6.6 years (range: 3.6–9.8 years), the 5-year event-free (EFS) and overall survival (OS) were both 100%.ConclusionThis report supports that HB with 100% WDF histology completely resected at diagnosis is curable with surgery only. The development of evidence-based surgical guidelines utilizing criteria based on PRETEXT group, vascular involvement (annotation factors), tumor-specific histology and corresponding biology will be crucial for optimizing which patients are candidates for resection at diagnosis followed by observation.Level of evidencePrognosis study, Level I evidence.     

Ipsilateral nodal recurrence after axillary dissection for breast cancer

IntroductionLevel I/II axillary lymph node dissection (ALND) is the standard operation for patients with node-positive breast cancer. The objective of this study was to assess the incidence of regional nodal recurrence (RNR) after ALND performed for definitive operative treatment for primary breast cancer.Materials and methodsA retrospective, Institutional Review Board-approved query of our single-institution National Comprehensive Cancer Network database was performed for patients undergoing ALND who developed subsequent RNR. All patients were treated from 1999 to 2009. A detailed chart review was performed and clinical, pathologic, treatment, and outcome data were collected.ResultsA total of 1614 patients had an ALND for initial staging; 14/1614 (0.9%) patients had RNR. Two other patients had contralateral breast/axillary recurrences and were excluded. The mean age at diagnosis for the sample group was 52.7 y (range 34–77); mean follow-up time was 47.1 mo (range 12.6–114.6). The median number of nodes for ALND was 16 (range 8–27). The median number of positive nodes was 2.5 (range 0–7). Nine (64.3%) cases were estrogen receptor/progesterone receptor negative. Twelve (85.7%) patients had axillary recurrences, and six of 12 (50.0%) had concurrent chest wall lesions. Twelve patients (85.7%) had distant metastases; nine of 12 (75.0%) died; two were lost to follow-up. Mean time from RNR to distant recurrence was 6.0 mo (range 0–29.3 mo).ConclusionsRNR after ALND is rare but a harbinger of poor outcome. This is apparent regardless of treatment used for initial disease or recurrence. Specifically, RNR after primary ALND is related to increased risk of mortality and distant metastatic disease.     

Short-term respiratory outcomes of neonates with symptomatic congenital lung malformations

IntroductionThe purpose of this study was to evaluate short-term respiratory outcomes in neonates with symptomatic congenital lung malformations (CLM).MethodsConsecutive newborns who underwent surgical resection of a CLM were retrospectively reviewed. Demographic, prenatal, and outcomes data were analyzed as appropriate (p < 0.05).ResultsTwenty-one neonates were managed at a median gestational age of 36.2 weeks [interquartile range (IQR), 33.8–39.0]. Endotracheal intubation was required in 14 (66.7%) for a median of 7.5 days [interquartile range (IQR), 3.0–25.8]. Three (14.3%) children underwent ex utero intrapartum treatment-to-resection, and another 14 (66.7%) had neonatal lung resections performed at a median age of 2.0 days (IQR, 0.08–19.5 days). Excluding one patient who received comfort care at birth, all neonates survived to hospital discharge with a median length of hospitalization of 36.5 days (IQR, 23.8–56.5). More than one-quarter were discharged on supplemental oxygen by nasal cannula. Based on a median follow up of 35.5 months (IQR, 19.0–80.8), CLM-related morbidity was still evident in 55.0%.ConclusionOur study suggests a high incidence of complications and chronic respiratory morbidity after neonatal lung resection for symptomatic CLMs. These data highlight the need to provide realistic expectations in perinatal counseling discussions with families and the importance of coordinating appropriate multidisciplinary follow up for these children.Level of Evidence: Level IV.     

Favorable postoperative outcomes for children with COVID-19 infection undergoing surgical intervention: Experience at a free-standing children's hospital

BackgroundCurrent literature has shown that adult patients with perioperative Coronavirus Disease-2019 (COVID-19) have increased rates of postoperative morbidity and mortality. We hypothesized that children with COVID-19 have favorable postoperative outcomes compared to the reported adult experience.MethodsWe performed a retrospective cohort study for children with a confirmed preoperative COVID-19 diagnosis from April 1st, 2020 to August 15th, 2020 at a free-standing children's hospital. Primary outcomes evaluated were postoperative complications, readmissions, reoperations, and mortality within 30 days of operation. Secondary outcomes included hospital resource utilization, hospital length of stay, and postoperative oxygen support.ResultsA total of 66 children with preoperative confirmed COVID-19 were evaluated with median age of 9.5 years (interquartile range (IQR) 5–14) with 65% male and 70% Hispanic White. Sixty-five percent of patients had no comorbidities, with abdominal pain identified as the most common preoperative symptom (65%). Twenty-three percent of patients presented with no COVID-19 related symptoms. Eighty-two percent of patients had no preoperative chest imaging and 98% of patients did not receive preoperative oxygen support. General pediatric surgeons performed the majority of procedures (68%) with the most common diagnosis appendicitis (47%). Forty-one percent of patients were discharged the same day as surgery with 9% of patients utilizing postoperative intensive care unit resources and only 5% receiving postoperative invasive mechanical ventilation. Postoperative complications (7%), readmission (6%), and reoperation (6%) were infrequent, with no mortality.ConclusionCOVID-19+ children requiring surgery have a favorable postoperative course and short-term outcomes compared to the reported adult experience.Type of studyPrognosis Study.Level of evidenceLevel IV.     

The role of ultrasound in detecting renal tract abnormalities following a single episode of epididymitis

AimThe aim of the study was to establish the utility of ultrasound scan in detecting renal tract abnormalities following a single episode of epididymitis.MethodsA single-centre retrospective review of all boys diagnosed with epididymitis between October 2012 and 2017 including review of follow up imaging and clinical course was completed. Primary outcome was new diagnosis of renal tract abnormality by ultrasound.Main resultsEighty-four boys with a first diagnosis of epididymitis were identified. Sixty-four cases (76%) were diagnosed at scrotal exploration, the remaining twenty clinically. Median age was 7.30 years (range 0.08–15.83 years), and five had a positive urine culture at presentation. Forty-eight boys (57%) had a follow-up ultrasound scan (at median 4.57 weeks [range 1–31 weeks]). Only two renal tract abnormalities were identified by ultrasound scan, both in boys aged < 6 months, and neither was clinically relevant. Recurrent epididymitis occurred in 4 cases at median 26 days after initial presentation, of whom 3 had been followed up by ultrasound after initial presentation, all of which were normal. Further investigation revealed posterior urethral valves in 1 boy (age 6.5 months at initial presentation).ConclusionFollowing a single episode of epididymitis, ultrasound was not helpful at detecting clinically relevant renal tract abnormalities, and furthermore did not identify the only patient with a clinically relevant abnormality. Based on these data, we propose follow-up imaging only in boys ≤ 6 months of age with a positive urine culture or a recurrent episode with consideration given to micturating cystogram even if ultrasound normal.Level of evidenceIV.     

Surgical management of pediatric thyroid disease: Complication rates after thyroidectomy at the Children's Hospital of Philadelphia high-volume Pediatric Thyroid Center

BackgroundRecent studies suggest improved outcomes for children undergoing thyroidectomy at high-volume pediatric surgery centers. We present outcomes after thyroid surgery at a single center and advocate for referral to high-volume centers for multidisciplinary management of these children.MethodsMedical records were reviewed for all pediatric patients undergoing thyroid surgery at a single institution from 2009 through 2017. Routine recurrent laryngeal nerve and parathyroid hormone monitoring was used. Lymph node dissections were performed in appropriately selected cancer patients. Data collection focused on pathologic diagnosis, surgical technique, and surgical complications, including postoperative hematoma, neurapraxia, permanent nerve damage, hypocalcemia, and transient and permanent hypoparathyroidism.ResultsFrom 2009 through 2017, 464 patients underwent thyroid surgery. Median age of the cohort was 15 years (range 2–24). Thirty-three percent were diagnosed with benign nodules (n = 151), 36% with papillary or follicular thyroid cancer (n = 168), 27% with Graves’ disease (n = 124), 3% with medullary thyroid cancer (n = 14), and 1.5% underwent prophylactic thyroidectomy for MEN2a (n = 7). Six patients required return to the OR for hematoma evacuation including 5 patients after surgery for Graves’ disease (RR 8.7, 95% CI 1.06–71.85). In sixteen cases, concern about neurapraxia resulted in laryngoscopy, revealing eleven patients with vocal cord paresis. Two of these patients demonstrated a persistent deficit at 6 months postoperatively (0.4%). Thirty-seven percent of patients had transient hypoparathyroidism (n = 137), and two patients had persistent hypoparathyroidism 6 months after total thyroidectomy (0.6%). There was no significant difference in either hypocalcemia or hypoparathyroidism after total thyroidectomy based on age or diagnosis.ConclusionsCharacterizing outcomes for pediatric patients based on diagnosis will assist in preoperative counseling for patients and their families. This high-volume center reports low complication rates after pediatric thyroid surgery, highlighting that referral to high-volume centers should be considered for children and adolescents with thyroid disease requiring surgery.Level of evidenceLevel IV     

Comparison of subcutaneous analgesic system and epidural analgesia for postoperative pain control in open pediatric oncology operations: A randomized controlled trial

PurposeChildren undergoing open oncologic surgery can have significant post-operative pain. The purpose of this trial was to compare a surgeon-placed subcutaneous analgesic system (SAS) to epidural analgesia.MethodsSingle center randomized controlled trial including children ≤18 years undergoing open tumor resection between October 2018 and April 2021. Randomization to SAS or epidural was done preoperatively and perioperative pain management was standardized. Families were blinded to the modality. Comparisons of oral morphine equivalents (OME) and pain scores for three postoperative days, clinical outcome parameters, and parental satisfaction following unblinding were completed using non-parametric analyses.ResultsOf 36 patients (SAS 18, Epidural 18), median age was 5 years (range <1–17). The Epidural cohort had less OME demand on postoperative day one (SAS 0.76 mg/kg, Epidural 0.11 mg/kg; p<0.01) and two (SAS 0.48 mg/kg, Epidural 0.07 mg/kg, p = 0.03). Pain scores were similar on postoperative days 1–3 (0–2 in both groups). The Epidural cohort had more device complications (SAS 11%, Epidural 50%; p = 0.03) and higher urinary catheter use (SAS 50%, Epidural 89%; p = 0.03). More than 80% of parents would use the same device in the future (SAS 100%, Epidural 84%, p = 0.23).ConclusionFor children undergoing open oncologic abdominal or thoracic surgery, early post-operative pain control appears to be better with epidural analgesia; however, SAS has decreased incidence of device complications and urinary catheter use. Parental satisfaction is excellent with both modalities. SAS could be considered as an alternative to epidural, especially in settings when epidural placement is not available or contraindicated.Type of studyTreatment study, Randomized controlled trial.Level of evidenceLevel 1.     

Musculoskeletal deformities after thoracic surgery in children: An observational long-term follow-up study

PurposeThis study reports the incidence, severity, and predictors of musculoskeletal deformities (MD), including scoliosis and chest wall anomalies, following thoracic procedures in children.MethodsChildren younger than 14 years who had thoracic surgery between 1997 and 2012 and had no other predispositions to MD, underwent longitudinal follow-ups with dedicated musculoskeletal examination performed in an esophageal atresia, orthopedic, or research clinic. Incidence of MD was calculated, and logistic regression methods were used to determine independent predictors, including sex, gestational age, age at procedure, serratus anterior muscle division, and chest tube placement.ResultsThe study cohort consisted of 104 patients followed for a median of 10.8 years (range 3–21). A total of 56 MD developed in 41 patients (39%), including scapular winging (24; 23%), scoliosis (17; 16%), and chest wall anomalies (15; 14%). The majority of MD were subclinical, with only 8 patients [8% (6 thoracotomies, 2 thoracoscopies)] requiring intervention. Among patients who underwent thoracotomies (93, 89%), serratus anterior muscle division was the only significant predictor of the development of MD [OR 8.9; 95% CI 2.8–32.6].ConclusionMusculoskeletal deformities develop in a significant proportion of children following thoracic surgery, but most are subclinical. A muscle-sparing technique decreases the incidence of these deformities.Type of StudyProspective Cohort Study.Level of EvidenceLevel II.     

The Modified Prone Jack-knife Position for the Excision of Female Urethral Diverticula

BackgroundFemale urethral diverticula (UD) are an uncommon and often overlooked aetiology in women presenting with lower urinary tract symptoms, urethral pain, and recurrent urinary tract infection. With increasing awareness, appropriate imaging is more commonly undertaken with consideration given to surgical management.ObjectiveThe video presented demonstrates the technique for excising large and/or complex UD using a modified prone jack-knife position—a position that offers excellent surgical access and allows the surgeon to operate in a more ergonomic position.Design, setting, and participantsA retrospective review of the data on patients undergoing excision of UD at a tertiary referral unit was performed.Surgical procedureUrethral and suprapubic catheters (±insertion of ureteric stents) were placed in supine position. UD excised in the modified prone jack-knife position (±placement of a Martius flap).MeasurementsSubjective cure rate, recurrence rate, rates of postoperative urinary incontinence, need for secondary incontinence procedure, and postoperative complications were measured.Results and limitationsA total of 121 patients were operated on in the study period. The mean follow-up time was 10 mo (range 3–40). The most frequent presenting symptoms included a vaginal mass (n = 76, 63%), followed by dysuria (n = 72, 60%) and pelvic pain (n = 71, 59%). An identifiable aetiological factor was present in 45 patients, including traumatic vaginal delivery (18, 15%), prior periurethral surgery (17, 14%), and urethral dilatation (10, 8%). All patients underwent postvoiding magnetic resonance imaging (MRI) to confirm the diagnosis and plan surgery. UD ranged in maximum diameter from 8 to 48 mm, with a mean of 43 mm (standard deviation 9.24). The most common anatomical location was midurethral (55, 46%), followed by distal (36, 30%), proximal (25, 21%), and full length (5, 4%). Most UDs were single in configuration (74%), followed by multiloculated (15%), saddle shaped (7%), and circumferential (5%). On preoperative videourodynamics, 17 (14%) had stress urinary incontinence. UD excision was undertaken in the modified prone jack-knife position in all cases. A Martius flap was utilised in 36 (30%). The median postoperative postvoiding residual was 26 ml (interquartile range 0–40). In total, 88 (73%) patients were continent postoperatively and 16 (13%) experienced de novo stress urinary incontinence. Of the 37 with pre-existing stress incontinence symptoms, 20 (54%) were continent after operation. A total of 14 patients had subsequent autologous fascial sling at 6 mo. In total, five symptomatic recurrences occurred (4%); of these patients, three elected to undergo surgical excision, all of whom had symptom resolution and were continent after operation. A total of 11 patients (9%) experienced a Clavien-Dindo grade I–II complication within 90 d after operation. Five patients complained of dyspareunia, which resolved by 6 mo.ConclusionsThe modified prone jack-knife position facilitates excellent access for excision of both simple and complex UDs. This positioning of the patient is not widely recognised amongst urologists. Using this approach, there were low rates of symptomatic recurrence and de novo stress incontinence at medium-term follow-up. Associated urinary incontinence resolves in over half of patients following UD excision; hence, we advocate deferring any incontinence procedure until after the results of surgery are established.Patient summarySurgical removal of urethral outpouching (diverticula) in women is challenging due to its potential to damage the nearby sphincter muscle, which controls continence, or the urethra tube. Placement of patients on their front, rather than on their back, provides excellent access for the surgical removal of urethral diverticula. With this approach, we achieved excellent rates of cure and low rates of urinary incontinence at an average follow-up of 10 mo.     

15 years of continent urinary diversion and enterocystoplasty in children and adolescents: the Würzburg experience

Study Type – Therapy (case series)
Level of Evidence 4

OBJECTIVE

To assess the long‐term results of continent urinary diversion (CUD) and enterocystoplasty (ECP) in children with irreversible lower urinary tract dysfunction (LUTD).

PATIENTS AND METHODS

The study included 44 children with irreversible LUTD who had a CUD or ECP between 1992 and 2007. Patients were followed for the functional outcome of surgery with a focus on complications related to the reservoir, bowel, uretero‐intestinal anastomosis and upper urinary tract. Data were collected prospectively and outcomes were evaluated using a standardized protocol.

RESULTS

The median (range) follow‐up was 7.3 (3.5–17) years. Complete continence was achieved in 94% overall, i.e. in 95% of patients with continent cutaneous diversion, 83% with ECP and all children with continent anal diversion. Upper urinary tract and renal function remained stable in 89% and 95%, respectively. Surgical intervention was required for adhesive small bowel ileus in 6%, stoma‐related complications in 39%, ureteric stenosis in 8%, and stone formation in 19%. Of these complications, 54% required only minor interventions; 41% of patients needed prophylactic alkaline substitution. Bowel habits remained unchanged or improved in 68%.

CONCLUSION

Our results show that CUD and ECP in children are effective procedures with acceptable long‐term complication rates. However, conclusions from our data might be limited, as this was a small study including highly selected patients treated at one tertiary academic centre. Being an audit of practice in our institution and given the variety of concepts, these results might differ from those centres using other approaches in the surgical treatment of LUTD. Importantly, this type of surgery should be restricted to carefully selected patients in whom all attempts of restoring the LUT failed.     

Level of agreement on postoperative complications after one-stage hypospadias correction comparing medical records and parent reports

PurposeTo analyze agreement on postoperative complications after hypospadias surgery according to medical records and parents' reports.Materials & methodsIn this retrospective cohort study, data were collected from 409 children who received an initial one-stage hypospadias correction in the Radboudumc, The Netherlands.Postoperative complications according to medical records were compared with parent-reported complications in an online questionnaire. Main complications studied were wound-related complications, urinary tract infections, fistulas, stenosis, and prepuce-related complications. Agreement was determined by Cohen's kappa coefficient.ResultsSlightly less complications were mentioned in medical records (37%) compared to parents' reports (42%). Overall agreement was moderate (κ = 0.50, 95% confidence interval (CI):0.41–0.59), but poor for some specific complications. Agreement was higher for complications that needed reoperation compared to when no reoperation was performed (κ = 0.53, 95% CI: 0.43–0.62 and κ = 0.18, 95% CI: 0.06–0.31) and for patients with recent surgery (< 5 years before questionnaire completion) compared to less recent surgeries (κ = 0.69, 95% CI: 0.55–0.84 and κ = 0.43, 95% CI: 0.33–0.54).ConclusionsAgreement on complications according to medical records and parents' reports was poor to moderate, but better after reoperation and more recent surgery. Some complications mentioned in medical records were missing from parents' reports and the other way around. Better agreement will give physicians and parents a more reliable view on postoperative outcome after hypospadias surgery.Type of studyDiagnostic test.Level of evidenceLevel III.     

Treating papillary and follicular thyroid cancer in children and young people: Single UK-center experience between 2003 and 2018

AimDifferentiated thyroid cancer (DTC) in children and adolescents is rare and data about its presentation and management are not well known. The aim of this study was to provide evidence of the current practice in the United Kingdom before the launch of the Rare National Paediatric Endocrine Tumours Guidelines (to be published in 2020).MethodsSeventy-two children and adolescents with DTC (< 18 years) who were treated at our institution between 2003 and 2018 were identified and their presentation, treatment and outcomes were reviewed.ResultsMedian age at presentation was 12.7 years [range: 1–18] and fifty-two (72%) were girls. Fifty (69.4%) children and adolescents presented with a thyroid nodule. Thirteen (18%) had cervical adenopathy and seven of them (54%) underwent an excision biopsy under GA. Eight patients (11%) had evidence of lung metastases at presentation. Twenty-four patients (33%) underwent a hemithyroidectomy and 22 of those had a completion thyroidectomy subsequently, ten (14%) a total thyroidectomy alone and 37 (51%) a total thyroidectomy with lymph nodes dissection. Seventy patients (97%) underwent adjuvant RAI at our institution. The median number of children and adolescents managed per year was five [range: 0–10]. After an overall median follow-up of 40 months, eight patients (11%) had developed recurrent disease. The 1- and 5-year recurrence-free-survival-rates were 93% and 87%, respectively. Overall survival was 100%, with eight children and adolescents (11%) being alive with disease.ConclusionThis study confirms that DTC in children and adolescents is uncommon, is frequently advanced at presentation and has considerable recurrence rates. Despite this, overall survival is excellent. Although the work-up was generally appropriate (image-guided cytology), open biopsy for the diagnosis of lymph node involvement was still employed. The introduction of a specific UK guideline for this age-group will likely result in more tailored-made treatment-pathways and thereby hopefully improve quality and outcomes even further.Type of studyPrognosis study.Level of evidenceLevel IV.     

Valor pronóstico de la citología urinaria en la recidiva del carcinoma urotelial vesical tras la cistectomía radical

IntroductionUrethral or upper urinary tract (UUT) recurrence of urothelial carcinoma (UC) after radical cystectomy (RC) are rare (4-6%), and their diagnosis usually occurs within the first two years. Although it is known that its early detection offers benefit in terms of survival, currently there are no clear recommendations for the detection of recurrence in the remnant urothelium (RU). Our aim is to determine the diagnostic value of urinary cytology for the detection of recurrences in the RU and to estimate its impact as an early diagnostic method on survival.Material and methodsRetrospective review of patients who underwent RC for urothelial carcinoma between 2008-2016, with a follow-up of at least 24 months.ResultsThe study included 142 patients. In a median follow-up of 68.5 months, nine patients (6.3%) presented recurrences in the RU (urethra: four, UUT: four, synchronous: one). The sensitivity and specificity of urinary cytology for the diagnosis of UUT recurrences were 20% and 96%, respectively. No significant differences were found between overall survival and cancer-specific survival among patients according to the urinary cytology results.ConclusionRecurrences in the RU after RC are infrequent; our study has shown that urinary cytology offers a low sensitivity for their diagnoses. For these reasons, we do not consider that urinary cytology provides useful information for surveillance of these patients.     

Can we increase detection? A nationwide analysis of age-related fractures in child abuse

PurposeThe purpose of this study was to stratify fractures associated with child abuse in relation to the child's age.MethodsThe Kids' Inpatient Database (1997–2012) was queried for all patients (< 18 years old) with a diagnosis of fracture and child abuse. The primary outcome was age-related determinants of fracture distribution. Chi-squared analysis was used for statistical analysis where appropriate, with significance set at p < 0.05.ResultsMore than 39,000 children were admitted for child abuse, and 26% sustained fractures. Most were infants (median age 0 year [IQR 0–1]). 28% sustained multiple fractures, and 27% had skull fractures.By age, infants had the highest rate of multiple fractures (33% vs 16% 1–4 years), and the highest rate of closed skull fractures (33% vs 21% ages 1–4), while adolescents had more facial fractures (43% vs 11% ages 9–12), all p < 0.001. Multiple rib fractures were more commonly seen in infants (28% vs 8% ages 1–4), while children 5–8 years had the highest rates of clavicular fractures (7% vs 3% in infants), all p < 0.001.ConclusionAge-related fracture patterns exist and may be due to changing mechanism of abuse as a child grows. These age-related fracture patterns can help aid in healthcare detection of child abuse in hopes to thwart further abuse.Type of studyRetrospective comparative study.Level of evidenceLevel III.     

Laparoscopic adrenal-sparing approach for children with bilateral pheochromocytoma in Von Hippel-Lindau disease

IntroductionVon Hippel-Lindau disease (VHL) is a rare cause of hereditary bilateral Pheochromocytomas (PHEO). Traditionally, treatment has been total adrenalectomy due to a lifetime risk of developing new tumors. Limited data exists on the surgical management of bilateral PHEO in children with VHL. We reviewed our experience with laparoscopic partial adrenalectomy for bilateral PHEO.MethodsA retrospective review was performed of patients undergoing adrenalectomy for PHEO in children with VHL from 2004 to 2019.ResultsEight children with VHL diagnosed with bilateral PHEO underwent 16 adrenalectomies (10 synchronous, 5 metachronous, 1 for recurrence). Median age at diagnosis was 13 [range 8–17] years with a median tumor size of 2.3 [range 0.5–7.7] cm. Of 16 adrenalectomies, all were performed laparoscopically, 14 were partial adrenalectomies; 2 patients required a contralateral total adrenalectomy due to size and diffuse multinodularity. There were no postoperative complications. No patients required corticosteroid replacement at the end of the study period. Two patients had new ipsilateral tumors identified after a median follow up of 5 [range 4–6] years with one undergoing repeat partial adrenalectomy. There were no mortalities in the study period.ConclusionPartial adrenalectomy for bilateral PHEO in patients with VHL is safe and does not compromise outcomes. When technically feasible, laparoscopic partial adrenalectomy should be considered as a primary surgical approach for children with VHL.Level of EvidenceLevel IV - Case series with no comparison group     

Management of urethral recurrence after orthotopic urinary diversion

Study Type – Therapy (case series)
Level of Evidence 4

OBJECTIVE

To evaluate our experience with urethral recurrences in patients treated by radical cystectomy(RC) and orthotopic neobladder urinary diversion for carcinoma of the bladder.

PATIENTS AND METHODS

We retrospectively reviewed the records of patients treated with RC and orthotopic urinary diversion between January 1980 and July 2004.

RESULTS

In all, 260 patients underwent RC with a Studer or Hautmann orthotopic urinary diversion; the median (range) follow‐up was 5.1 (0–15.6) years. Six patients (2.3%) developed local recurrence of urothelial cancer (UC) within the urethra after this treatment. The median (range) time to presentation with recurrence after RC was 2.4 (0.7–3.6) years for pT1‐4 UC. Recurrences were treated with various methods, including transurethral resection, urethrectomy with conversion of neobladder to continent catheterizable diversion, and chemotherapy. At the last follow‐up, four of these six patients were alive without disease, one was alive with disease, and one had died from disease.

CONCLUSIONS

In our experience, local recurrences involving the urethra are infrequent. Complete surgical excision can provide a good outcome. Neoadjuvant chemotherapy should be considered for recurrences with adverse clinicopathological features.     

Severity of complications following restorative proctocolectomy in children is related to staging not diagnosis

BackgroundRestorative proctocolectomy (RPC) is performed using a variety of staged procedures for several diseases. Our aim was to assess whether the severity of complications, classified according to Clavien–Dindo, was related to the diagnosis or the procedure.MethodsA consecutive series of children receiving an ileoanal pouch was prospectively recorded. Complications were scored by two blinded observers. Major complications were Clavien–Dindo ≥ 3b. Procedures were classified as: colectomy, proctectomy and pouch or proctocolectomy and pouch. Diagnoses were classified as: ulcerative colitis, familial adenomatous polyposis or other: idiopathic constipation, total colonic Hirschsprung’s disease, juvenile polyposis, Crohn’s colitis, fibrosing colonopathy or necrotising enterocolitis.Results128 children underwent 191 procedures: 61 colectomies, 63 proctectomies and 67 proctocolectomies. 84 children had ulcerative colitis, 20 had FAP and 24 had other indications. Major complications were significantly more likely with proctocolectomy (16/67, 24%) than with either colectomy (4/61, 7%) or proctectomy (8/63, 13%), p = 0.01. There was no association between diagnosis and major complications: ulcerative colitis (18/133, 14%), FAP (5/20, 25%), other (5/38, 13%) p = 0.4. There was no increase in major complications following proctectomy if a major complication had occurred during prior colectomy. Overall, 15% of procedures experienced a major complication. 6/9 stoma related complications required operative intervention.ConclusionsThe severity of complications after RPC in children is related to use of a two stage rather than three stage sequence of surgery, not the underlying diagnosis.Type of studyCase control study.Level of evidenceLevel III.     

Video-assisted thoracic surgery resection for pediatric mediastinal neurogenic tumors

Background/PurposeVideo-assisted thoracoscopic surgery (VATS) resection of mediastinal neurogenic tumors is still controversial in children. The aim of this study was to review the cases of VATS resection of such tumors in children from 3 institutions located in different countries.MethodsThis retrospective study included 17 children treated between July 1995 and February 2011. Medical charts were reviewed for collection of data on age, sex, histologic type of tumor, clinical manifestations, age and weight at surgery, tumor size, duration of thoracic drainage, surgical complications, tumor recurrence, and mortality.ResultsThirteen (76.5%) males and 4 (23.5%) females were studied. Median age was 16 months (range, 10.6-60 months), and median weight was 11.9 kg (range, 9.3-27.4 kg). Ten children had neuroblastoma (58.8%), 4 had ganglioneuroma (23.5%), and 3 had ganglioneuroblastoma (17.7%). The median duration of the operation was 90 minutes (range, 45-180 minutes), with complete thoracoscopic resection in all cases. Two children (11.8%) developed Horner syndrome postoperatively. No deaths were reported, and no recurrence was noted during a median follow-up period of 16 months (range, 8.9-28.6 months).ConclusionsVideo-assisted thoracoscopic surgery resection of mediastinal neurogenic tumors in children produced good results, with no recurrence and minimal postoperative complications. The major advantages of this approach are the avoidance of thoracotomy complications and the enhanced surgical accuracy provided by improved visualization.