Three-Dimensional Printed Model and Virtual Reconstruction: An Extra Tool for Pediatric Solid Tumors Surgery.

Introduction Three-dimensional (3D) technology is increasingly applied for planning challenging surgical interventions. We report our experience using 3D printing and virtual reconstruction for surgical planning of complex tumor resections in children. Methods Data were obtained from preoperative magnetic resonance. imaging analysis and 3D virtual recreations were performed using specialized computer software. 3D real-scale geometry models, including tumor, adjacent organs, and relevant vascularization, were printed in colorimetric scale and different materials for optimal structures discrimination. Results Four complex cases were selected. The first case was a bilateral Wilms tumor. The volumetric reconstruction proved the presence of enough healthy renal tissue, allowing bilateral nephron-sparing surgery. In the second case, reconstruction contributed to the location of pulmonary metastases. The third case was an abdominal neuroblastoma stage L2. The 3D model was of high value for planning and as a reference during the intervention. The last case is a cervico-thoracic neuroblastoma with an anatomopathological diagnosis of ganglioneuroma, located at the cervico-mediastinal juncture, in close relationship with the cervical vessels. Conclusions 3D reconstruction and the full-scale printing models are a useful tool in cases of complex tumor resections as they contribute to a better understanding of the relationships between the tumor and adjacent organs, helping to anticipate certain surgical complications. They also provide additional information to conventional imaging tests, being able to influence therapeutic decisions and facilitate the understanding by the family, improving doctor-patient communication.

Correlación entre el neumoperitoneo y los hallazgos quirúrgicos y morbimortalidad en recién nacidos con enterocolitis necrosante / Correlation between pneumoperitoneum and surgical findings and morbidity and mortality in newborns with necrotising enterocolitis

INTRODUCCIÓN: La intervención quirúrgica en las enterocolitis necrosantes (EN) es precisa cuando existe gangrena intestinal, hecho evidente cuando produce perforación y neumoperitoneo, siendo este la única indicación radiológica aceptada universalmente para la intervención quirúrgica. OBJETIVO: Analizar a los pacientes intervenidos de EN, saber por qué se les intervino, cómo evolucionan y si los pacientes perforados presentan neumoperitoneo. MÉTODO: Estudio retrospectivo de una cohorte de recién nacidos con EN intervenidos durante un periodo de 10 años (2006-2015). Se analizan los hallazgos radiológicos preoperatorios y se correlacionan con los quirúrgicos y con la morbimortalidad, dependiendo de la presencia de neumoperitoneo (N+) o no (N-). Se evaluó la concordancia interobservador con radiólogo pediátrico enmascarado a la clínica mediante el índice de acuerdo kappa. RESULTADOS: Se analizó a 53 pacientes. El 36% se intervino tras la visualización de neumoperitoneo; en el resto, la indicación fue deterioro clínico y metabólico, junto con hallazgos radiológicos asociados. En el 39% del grupo N- se objetivó perforación. No se encontraron diferencias significativas en ambos grupos con respecto a longitud intestinal resecada, días de intubación, día de inicio de nutrición enteral y mortalidad. La comparación entre duración de síntomas y estancia hospitalaria total en ambos grupos (N-/N+) fue significativa (7 vs. 2 días, p = 0,008; 127 vs. 79 días, p = 0,003 respectivamente), siendo más favorable en el grupo N+. Estas diferencias se mantuvieron al ajustar por peso. CONCLUSIONES: La indicación quirúrgica ha de basarse en un conjunto de datos clínicos y radiológicos, ya que el 39% de los pacientes sin neumoperitoneo presentaron perforación. En nuestro estudio la presencia de neumoperitoneo no se correlaciona con peor pronóstico

INTRODUCTION: Surgical intervention in necrotising enterocolitis (NEC) is correct when there is intestinal gangrene. This is evident when gangrene produces perforation and pneumoperitoneum, with this being the only universally accepted radiological indication for the surgical intervention of NEC. OBJECTIVE: To perform an analysis on patients with surgically managed NEC, including determining how the decision to intervene is reached, the outcomes, and if patients with perforation had a pneumoperitoneum. METHODS: Retrospective review of neonates with surgical NEC over a period of 10years (2006-2015). An analysis was made of pre-surgical x-ray findings, which were compared with surgical ones, in addition to the morbidity and mortality, depending on the presence (N+) or absence (N-) of pneumoperitoneum. An evaluation was also made of the interobserver concordance with a paediatric radiologist blinded to the clinical reason using the kappa agreement index. RESULTS: A total of 53 neonates were included in the study. Surgical treatment was indicated after observing pneumoperitoneum in 36%. In the remaining neonates, the surgical decision was made after noting a clinical and metabolic deterioration with classical x-ray findings. Intestinal perforation was observed in 39% of the N- neonates. There were no statistical differences between either group on analysing the excised intestinal length, days of intubation, starting of enteral nutrition, and the mortality rate. Comparisons in terms of duration of symptoms and total hospital stay were statistically significant (7 vs. 2 days, P = .008; 127 vs. 79 days, P = .003, respectively), with both being more favourable in the N+ group. These differences remained when the groups were adjusted by birthweight. CONCLUSIONS: Surgical indication has to be done on an ensemble of clinical and radiological evidence, as 39% of the neonates in the N- groups were perforated. In our study, the presence of a pneumoperitoneum did not correlate with a worse prognosis

Necrotic Lesions Following Elective Urological Surgery in an Infant.

Case Report An 11-month-old female infant presented on the first postoperative (PO) day following an elective pyeloplasty, a dark bluish erythema of her lumbotomy wound, plus a satellite lesion of the same characteristics. Fever and sepsis developed, and despite broad spectrum antibiotics (meropenem and vancomycin) were started, a diagnosis of necrotizing soft-tissue infection (NSTI or necrotizing fasciitis) was established. Surgical debridement of both lesions was performed on day 3 PO, and a surgical contamination (ring retractor blade) was suspected, due to the particular geography of the lesion. Urine and blood cultures yielded no bacteria, but tissue culture grew Pseudomonas aeruginosa . At PO 6th day, lesions still appeared exudative and poorly perfused, so vacuum-assisted therapy (VAT) treatment was started. Exudate control, perfusion, and granulation improved in consecutive days, which permitted direct closure (no graft needed) at PO day 12. Discussion P. aeruginosa can be a fatal cause of type I NSTI. It has been reported rarely in adult series, with a prevalence of 4%, but it can be a major pathogen in pediatric NSTI. Added to an early recognition, aggressive surgery and debridement are required, in combination with antibiotic therapy, to limit the spread of the infection. In our case, despite surgical debridement being performed on day 3 PO, both wounds maintained scarce perfusion, and debris and exudate were poorly controlled with usual silver foams and daily nursery cures. VAT pediatric device was then added, which rapidly improved surgical bed, enhancing tissue perfusion and granulation in the following days.

Benign Scrotal Tumor in a Pediatric Patient: Epididymal Cyst.

A pediatric patient of 12 years consulted for a left scrotal mass of 2 months of evolution. After suspecting a cystic content due to positive transillumination, on ultrasonography a scrotal cyst separated from the testis, of 5 cm in its maximum length, was confirmed. Due to size, parental anxiety, and the referred short evolution, excision was decided. Given the clinical radiological findings, a scrotal incision was chosen, obtaining complete excision. Biopsy confirmed the diagnosis of simple epididymal cyst (EC). ECs usually present as painless, scrotal swelling in adolescents as a result of dilatation of the efferent epididymal tubules. Many cases (up to 60%) regress spontaneously. In these, average time to involute ranges from 4 to 50 months. Although cases of cyst torsion have been described (with pain derived from ischemia and inflammation), conservative management has been suggested in the majority, both in pediatric and in adult series. Surgery is recommended in some patients, due to testicular pain or increased paratesticular mass, as was our case.

[Correlation between pneumoperitoneum and surgical findings and morbidity and mortality in newborns with necrotising enterocolitis]. / Correlación entre el neumoperitoneo y los hallazgos quirúrgicos y morbimortalidad en recién nacidos con enterocolitis necrosante.

INTRODUCTION: Surgical intervention in necrotising enterocolitis (NEC) is correct when there is intestinal gangrene. This is evident when gangrene produces perforation and pneumoperitoneum, with this being the only universally accepted radiological indication for the surgical intervention of NEC. OBJECTIVE: To perform an analysis on patients with surgically managed NEC, including determining how the decision to intervene is reached, the outcomes, and if patients with perforation had a pneumoperitoneum. METHODS: Retrospective review of neonates with surgical NEC over a period of 10years (2006-2015). An analysis was made of pre-surgical x-ray findings, which were compared with surgical ones, in addition to the morbidity and mortality, depending on the presence (N+) or absence (N-) of pneumoperitoneum. An evaluation was also made of the interobserver concordance with a paediatric radiologist blinded to the clinical reason using the kappa agreement index. RESULTS: A total of 53 neonates were included in the study. Surgical treatment was indicated after observing pneumoperitoneum in 36%. In the remaining neonates, the surgical decision was made after noting a clinical and metabolic deterioration with classical x-ray findings. Intestinal perforation was observed in 39% of the N- neonates. There were no statistical differences between either group on analysing the excised intestinal length, days of intubation, starting of enteral nutrition, and the mortality rate. Comparisons in terms of duration of symptoms and total hospital stay were statistically significant (7 vs. 2 days, P=.008; 127 vs. 79 days, P=.003, respectively), with both being more favourable in the N+ group. These differences remained when the groups were adjusted by birthweight. CONCLUSIONS: Surgical indication has to be done on an ensemble of clinical and radiological evidence, as 39% of the neonates in the N- groups were perforated. In our study, the presence of a pneumoperitoneum did not correlate with a worse prognosis.

Tumor parotídeo en un neonato / Parotid tumour in a neonate

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Evaluación funcional y estética de los teratomas sacrococcígeos. No todo termina con la cirugía / Functional and aesthetic evaluation of sacrococcygeal teratomas. Not everything ends with surgery

Introducción: El teratoma sacrococcígeo es el tumor neonatal sólido más frecuente. El aumento de la supervivencia de estos pacientes ha permitido diagnosticar y tratar secuelas posquirúrgicas. Evaluamos el resultado funcional y estético a largo plazo de los pacientes intervenidos en nuestro centro. Material y método: Estudio retrospectivo de los teratomas sacrococcígeos intervenidos en nuestro hospital desde 1977 hasta 2014. Se recogieron datos personales de los pacientes y se realizó encuesta telefónica sobre hábito intestinal, urinario y autoevaluación estética y social. Resultados: En el período de estudio se intervino a 14 pacientes, 11 mujeres y 3 varones, con una edad mediana en el momento de la encuesta de 17 años (8 meses-37 años). Se encuestó a 8 pacientes (57,1%). La edad mediana de esos 8 pacientes fue de 23 años (4-37 años). El 37,5% se intervino por un teratoma sacrococcígeo tipo I, 25% tipo II, 25% tipo III y 12,5% tipo IV. De los pacientes estudiados, 2 (25%) presentan estreñimiento y uno (12,5%) incontinencia fecal. Dos pacientes (25%) presentan infecciones urinarias recurrentes y 3 (37,5%) incontinencia urinaria. Cinco pacientes (62,5%) presentan alterada su percepción física con limitación en su vida social. Conclusiones: En nuestra serie, se ha encontrado un porcentaje similar a la literatura en la incidencia de estreñimiento. La incontinencia fecal está levemente disminuida respecto a series publicadas. Sin embargo, la frecuencia de infecciones y de incontinencia urinarias es mayor en nuestra serie. Cinco pacientes presentan problemas psicosociales según el cuestionario DAS-59. Los pacientes con teratoma sacrococcígeo requieren asesoramiento urológico, digestivo y psicológico, para poder adquirir un completo desarrollo funcional y emocional (AU)

Introduction: Sacrococcygeal teratoma is the most common solid neonatal tumour. The improvement in survival has meant that postoperative sequelae can be diagnosed and treated. The aim of this article is to evaluate the long-term outcomes of patients treated in our centre. Material and methods: Records of patients treated for a sacrococcygeal teratoma in our hospital from 1977 to 2014 were retrospectively reviewed. Personal data was collected and a telephone questionnaire was used to assess long-term bowel and urinary habits, as well as an aesthetic and functional self-assessment. Results: A total of 14 patients were treated during the study period, of whom 11 were females and 3 males, with a mean age at the time of the survey of 17 years (8 months-37 years). Eight patients completed the questionnaire (57.1%). The mean age of the 8 patients was 23 years (4-37 years), of whom 37.5% were operated on due to a sacrococcygeal teratoma type I, 25% type II, 25% type III, and 12.5% type IV. Two of them (25%) had constipation, and one (12.5%) had faecal incontinence. Two (25%) patients suffered from recurrent urinary tract infections, and 3 (37.5%) patients had urinary incontinence. Five patients (62.5%) had a perception of being physically impaired, with limitation of their social life. Conclusions: The incidence of constipation does not differ from that found in the literature. Faecal incontinence is slightly improved compared to what has been published. However, urinary tract infections and incontinence are more prevalent in our series. Five patients out of the eight that responded suffered from psychosocial problems, according to DAS-59 questionnaire. Patients with SCT require urological, bowel, and psychological counselling, until they have a complete functional and emotional development (AU)

[Functional and aesthetic evaluation of sacrococcygeal teratomas. Not everything ends with surgery]. / Evaluación funcional y estética de los teratomas sacrococcígeos. No todo termina con la cirugía.

INTRODUCTION: Sacrococcygeal teratoma is the most common solid neonatal tumour. The improvement in survival has meant that postoperative sequelae can be diagnosed and treated. The aim of this article is to evaluate the long-term outcomes of patients treated in our centre. MATERIAL AND METHODS: Records of patients treated for a sacrococcygeal teratoma in our hospital from 1977 to 2014 were retrospectively reviewed. Personal data was collected and a telephone questionnaire was used to assess long-term bowel and urinary habits, as well as an aesthetic and functional self-assessment. RESULTS: A total of 14 patients were treated during the study period, of whom 11 were females and 3 males, with a mean age at the time of the survey of 17 years (8 months-37 years). Eight patients completed the questionnaire (57.1%). The mean age of the 8 patients was 23 years (4-37 years), of whom 37.5% were operated on due to a sacrococcygeal teratoma type i, 25% type ii, 25% type iii, and 12.5% type iv. Two of them (25%) had constipation, and one (12.5%) had faecal incontinence. Two (25%) patients suffered from recurrent urinary tract infections, and 3 (37.5%) patients had urinary incontinence. Five patients (62.5%) had a perception of being physically impaired, with limitation of their social life. CONCLUSIONS: The incidence of constipation does not differ from that found in the literature. Faecal incontinence is slightly improved compared to what has been published. However, urinary tract infections and incontinence are more prevalent in our series. Five patients out of the eight that responded suffered from psychosocial problems, according to DAS-59 questionnaire. Patients with SCT require urological, bowel, and psychological counselling, until they have a complete functional and emotional development.

Renal Trauma in Pediatrics: A Current Review.

Children are at increased risk of renal injuries from blunt trauma. Four-phase computed tomography with intravenous contrast (noncontrast, arterial, nephrographic, and pyelographic phases) is the choice for initial imaging, although ultrasonography might also be used in children with minimal symptoms. The American Association for the Surgery of Trauma developed the known system for renal injury grading, which was modified in 2011. The management of pediatric renal injuries has largely shifted toward conservative means. However, as long as the child remains hemodynamically unstable, renal exploration might be necessary. There is a trend toward managing high-grade injuries with interventional radiography procedures.

Migration of a ventriculoperitoneal shunt catheter through the processus vaginalis in infants: how serious inguinal hernia and hydrocele might be.

Migration of the catheter tip of a ventriculoperitoneal shunt (VPS) through a patent processus vaginalis (PV) has seldom being reported. In this series, we analyzed all cases of inguinoscrotal migration of an VPS catheter at our center. Between 1975 and 2015, 295 VPS insertions were performed. In seven infants (2.3%), migration of the catheter through a patent PV was demonstrated. Cases with hydroceles had associated VPS malfunction, but cases with hernias were not associated with it. The current recommendation is to delay a PV closure until 2 years of age in cases of infants with hydrocele without further radiological examinations, but this might not be the case in infants with VPS.

Piocele como complicación de peritonitis en un preescolar / Pyocele as a complication peritonitis in an preschooler

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Apendicitis en lactantes. Casuística de 25 años / Appendicitis in infants. 25 year case series

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Dysfunctional Voiding in Pediatrics: A Review of Pathophysiology and Current Treatment Modalities.

In Dysfunctional Voiding (DV), the external urethral sphincter or pelvic diaphragm (consisting of striated muscle) contracts during voiding, contrary to normal physiological mechanism. In Flowmetry plus pelvic Electromyography (EMG), the perineal surface electrodes will show a persistent activity during voiding. The cause is believed to be the persistence of an immature bladder control. The overall prevalence of DV is estimated between 7 and 11% of girls of school age. Symptoms of DV comprise a range of symptoms, deriving from outlet obstruction, incomplete voiding and elevated Post Micturition Residual (PMR): from daytime urine leaks, nocturnal enuresis, Urinary Tract Infections (UTI), or Vesicoureteral reflux (VUR), to the final decompensation of upper urinary tract in extreme cases. The literature reflects a high prevalence of constipation among these patients: between 33 and 56% of dysfunctional voiders are constipated. Initially, therapy of functional voiding disorders such as DV, comprises a series of fundamental principles called Urotherapy or voiding re-education. Currently, Urinary animated Biofeedback (Bfb) is the treatment modality of choice. This therapy discloses patients their own physiological and muscular mechanisms. Majority of the studies on Bfb efficacy reflect an improvement/ cure rate of around 70-80%. Initial flowmetric improvement followed by symptom relief or clinical improvement as the Bfb sessions continue, is likely to occur. It has been observed that there are two variables associated with clinical success of Bfb in DV syndrome: the absence of PMR at the end of treatment and an adequate or even aggressive constipation management.

Radiation-free monitoring in the long-term follow-up of pyeloplasty: Are ultrasound new parameters good enough to evaluate a successful procedure?

INTRODUCTION: Long-term evolution of residual hydronephrosis after successful pyeloplasty is not common. In this report, we have studied new ultrasound parameters, and have investigated the frequency of residual hydronephrosis. We highlight alternative radiation-free monitoring of pediatric pyeloplasties with ultrasound alone. PATIENTS AND METHODS: Children who had undergone successful open Anderson-Hynes pyeloplasties in the period 2001-2010 were followed up. Exclusion criteria included non-clearly obstructed renography scans (tracer clearance half-time < 20 min), crossing vessels, failed pyeloplasty, bilateral disease, other renal concomitant anomaly, absence of ultrasound measurements, and loss to follow up (<1 year). Postoperative (postoperative) ultrasound parameters were anteroposterior (AP) diameter, pelvis-cortex (P/C) ratio, a proportion that takes in account these two values, enhancing sensitivity to evaluate minimal evolutive changes), and percentage of improvement (PI) in AP diameter (which reflects in relative means the evolution of each AP diameter, being 0% no change, and 100% absence of hydronephrosis) (Figure). Echographic checks were made at 3 and 6 months postoperatively and then yearly afterwards. SPSS software (v. 17.0 IBM, College Station, TX, USA) was used. RESULTS: Out of 80 pyeloplasties performed in the above-mentioned period, 44 patients (i.e., 44 renal units) fulfilled the inclusion criteria for the main study. The median age at time of operation was 15 months (range 2 months-10.3 years). The median follow-up was 4.5 years (range 1-12 years). The mean preoperative anteroposterior diameter was 26 mm (range 16-54 mm). At the third postoperative check, the mean the PI was 29%, and rose to 53% at 6 months (p=0.027). Posterior controls showed a stable yearly PI during follow-up, without statistically significant variations (40-59% in subsequent years, p>0.5). The P/C ratio had already downgraded significantly at the third postoperative check (4.6 preoperative vs. 1.8 postoperative; p=0.03). A subgroup analysis of failed pyeloplasty (4 renal units) showed all PI < 15% at the third postoperative month (sensitivity 100%, specificity 86%). Complete resolution of hydronephrosis occurred in nine patients (20%). DISCUSSION: The P/C ratio and PI are new feasible ultrasound parameters in pyeloplasty follow-up. Early improvement in the P/C ratio can be expected and might avoid repeated ionizing scans. A PI>15% in subsequent postoperative checks might be enough for safe monitoring with ultrasound alone. Thus, renograms may be solicited only in these cases where ultrasound parameters do not improve in the first 6 postoperative months. Afterwards, ultrasound parameters often remain stable in the long term. Absence of hydronephrosis could only be documented in the long term in one out of every five patients.

Lymphatic Malformation, Retinoblastoma, or Facial Cleft: Atypical Presentations of PHACE Syndrome.

PHACE syndrome is a neurocutaneous disorder characterized by large cervicofacial infantile hemangiomas and associated anomalies: posterior fossa brain malformation, hemangioma, arterial cerebrovascular anomalies, coarctation of the aorta and cardiac defects, and eye/endocrine abnormalities of the brain. When ventral developmental defects (sternal clefting or supraumbilical raphe) are present the condition is termed PHACE. In this report, we describe three PHACE cases that presented unique features (affecting one of the organ systems described for this syndrome) that have not been described previously. In the first case, a definitive PHACE association, the patient presented with an ipsilateral mesenteric lymphatic malformation, at the age of 14 years. In the second case, an anomaly of the posterior segment of the eye, not mentioned before in PHACE literature, a retinoblastoma, has been described. Specific chemotherapy avoided enucleation. And, in the third case, the child presented with an unusual midline frontal bone cleft, corresponding to Tessier 14 cleft. Two patients' hemangiomas responded well to propranolol therapy. The first one was followed and treated in the pre-propranolol era and had a moderate response to corticoids and interferon.

[Vesicovaginal Reflux and Urocolpos]. / Reflujo Vaginal y Urocolpos.

OBJECTIVE: Vesico-vaginal reflux (VVR) is defined as the reflux of urine into the vaginal vault during voiding, occasionally seen in pre- adolescent girls. The typical history consists in post voiding leaks in the daytime, that correspond to the progressive urine discharge from the vagina, after it has been filled up during micturition. We intend to show two cases presenting with significant urocolpos. METHODS: Description of two clinical cases observed in the pediatric urology office. RESULTS: Clinical case 1.- A 10-year-old girl presented with the complaint of diurnal incontinence (in the immediate post-voiding minutes). The early voiding phase in the cystourethrogram (VCUG) demonstrated progressive gross distension of the vagina (urocolpos) due to retrograde filling as the bladder emptied. The girl was managed with behavioural modifications, and was dry afterwards. Clinical case 2.- A 9-year-old girl presented with history of incomplete voiding. In infancy, she had right-sided vesicoureteral reflux (VUR) and was endoscopically treated at the age of 2. VCUG showed VVR, and no VUR. She was then successfully treated with behavioural modifications. Clinical case 3.- A 10-year-old girl complained of diurnal urinary incontinence, described as post voiding leaks. Again, a VCUG showed VVR and a small urocolpos. After re-education of voiding habits, leaks disappeared, but recurred 6 months afterwards, when she acknowledged no adherence to the therapy. DISCUSSION: Between 12 to 15% of girls referred to Urological clinics because of urine incontinence present VVR. In the absence of a clear anatomical obstruction, reflux happens as the urine flow encounters a natural obstacle in the labia majora usually in girls that close their legs as they void. Instructions on proper voiding form a key element in the management of VVR, and if not enough, the behavioural modification consists on a reverse position during voiding.