Outcomes of a pediatric surgical oncology fellowship in a pediatric cancer institution.

Surgery plays an important role as part of the treatment plan in most children with malignant solid tumors in regards to initial biopsy, upfront resection, and delayed resection. Surgeons also play a critical role in the treatment of surgical complications that may arise during medical treatment. The pediatric surgical oncologist should be familiar with the current treatment guidelines, histology implications, chemotherapy and radiation side effects, tumor staging, and overall care of the child with cancer. Specific training in pediatric surgical oncology is not widespread internationally and it represents a potential undervalued intervention for improving global pediatric cancer care.

Long-term outcomes of pediatric and adolescent mediastinal germ cell tumors: a single pediatric oncology institutional experience.

PURPOSE: To evaluate the epidemiologic, demographic, and clinical characteristics, as well as prognostic factors and long-term outcomes of mediastinal germ cell tumors (MGCT) in children. PATIENTS AND METHODS: A retrospective study of pediatric patients diagnosed with a primary MGCT between January 1963 and August of 2014 was performed. RESULTS: Twenty-five patients were identified. Six children with teratomas were treated with resection alone (median age 7.8 years, range newborn to 15 years) and were cured without recurrence or progression. Nineteen children were treated for a malignant MGCT (median age 11.7 years, range 7 months-18 years); 5 year overall survival (OS) was 0.39 ± 0.12. For malignant non-seminomatous mediastinal germ cell tumors, platinum-based chemotherapy regimen (OS 0.56 vs 0.14, p = 0.03), complete surgical resection with negative margins (OS 0.73 vs 0.11, p = 0.03); and localized disease (OS 0.76 vs 0.0, p = 0.004) demonstrated a survival advantage. CONCLUSIONS: Initial surgical resection is appropriate for teratomas. Localized disease, complete resection, and platinum-based chemotherapy are associated with improved survival in malignant non-seminomatous mediastinal germ cell tumors. Neoadjuvant, platinum-based three drug regimens followed by delayed surgical resection is the appropriate treatment modality for malignant mediastinal germ cell tumors.

Guidewire Catheter Exchange in Pediatric Oncology: Indications, Postoperative Complications, and Outcomes.

BACKGROUND: Maintaining long-term central venous catheters (CVCs) in children undergoing chemotherapy can be challenging. Guidewire catheter exchange (GCE) replaces a CVC without repeat venipuncture. This study evaluated the indications, success rate, and complications of GCE in a large cohort of pediatric cancer patients. PROCEDURE: Medical records of pediatric cancer patients who underwent GCE at our institution between 2003 and 2013 were retrospectively reviewed. Variables analyzed included gender, age at GCE, primary cancer diagnosis, indication for GCE, absolute neutrophil count (ANC) at GCE, vein used, success rate, and postoperative complications (<30 days after exchange). RESULTS: A total of 435 GCEs performed in 407 patients (230 males and 177 females) were reviewed. Median age at GCE was 8 years (range, 0.2-24). Acute lymphoblastic leukemia was the most common diagnosis (50.6%). The primary indication for GCE was the desire to have an alternative type of CVC (71%). Other indications included catheter displacement (17%), catheter malfunction (11%), and catheter infection (1%). Median ANC at GCE was 2,581/mm(3) (range, 0-43,400). Left subclavian vein was more commonly used (57.7%). The success rate of GCE was 93.4% (406 of 435 procedures, 95% confidence interval: 91.0-97.5%). A total of 33 (7.5%) postoperative complications occurred including central line associated bloodstream infection (CLABSI) (n = 20, 4.5%), catheter dislodgement (n = 6, 1.4%), and catheter malfunction (n = 7, 1.6%). CONCLUSIONS: We conclude that GCE in pediatric cancer patients is associated with a high success rate and a low risk of complications. The most common postoperative complication, CLABSI, occurred at a rate significantly lower than following de novo CVC placement.

[Study of the expression of neural stem cell markers in neuroblastoma tumor samples and correlation with prognostic factors]. / Estudio de expresión de marcadores de células madre neurales en neuroblastoma y correlación con factores pronóstico.

INTRODUCTION: The existence of cancer stem cells (CSC) in neuroblastoma (NB) has been associated with the development of metastasis, resistance to chemotherapy and recurrence. Our objective is to analyze the expression of proliferation and differentiation markers of neural progenitor cells in NB samples, and to correlate this expression with clinical variables such as histology, genetics and response to conventional therapy. MATERIAL AND METHODS: We performed a retrospective-experimental study with neuroblastoma samples obtained from biopsies or tumor resections between 2010-2012 in our Hospital. Fluorescence immunohistochemistry was used to analyze the expression of the different markers: CD44, CD74, CD133, tyrosine hydroxylase, endothelin receptors type A (ETA) and B (ETB), p75, nestina y and Phox2b, all of them related to neural stem cell biology. The level of expression of the markers was then correlated with clinical variables. RESULTS: Nestin expression was positive in 72.2% of samples and ETA in 66.7%. PHOX2B and CD74 expression were lower, being positive in less than 30%. The markers CD44, ETB and PHOX2B were expressed in more aggressive tumors. ETA expression correlated significantly with unfavorable histology tumors (p= 0.01), N-myc amplification (p= 0.05) and recurrence/progression (p= 0.05). CONCLUSION: The expression of CD44, ETB and ETA was associated with more aggressive tumors and poor prognostic factors. These markers are in the membrane of neural stem cells and may be useful to identify and isolate by flow cytometry CSCs of NB for the study of new therapeutic targets.

Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation. / Cirugía guiada por tromboelastometría en neuroblastoma complicado con coagulación intravascular diseminada.

BACKGROUND: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. OBSERVATION: A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. CONCLUSION: Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.

INTRODUCCION: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. CASO CLINICO: Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. CONCLUSION: El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.

[Clinical follow up and histological findings of patients with MEN2 treated with prophylactic thyroidectomy]. / Hallazgos histológicos y seguimiento clínico de pacientes con MEN 2 tras tiroidectomía profiláctica.

OBJECTIVE: To present our experience in the prophylactic management of the medullary thyroid carcinoma (MTC), reviewing the correlation between clinical, analytical, histopathological, and genetic findings on subjects with type 2A multiple endocrine neoplasia (MEN 2A) and familial MTC. MATERIAL AND METHODS: A retrospective study was done by reviewing the medical records of patients diagnosed with MEN 2A or familial MTC, between 1997 and 2011. The variables studied were sex, age at the time of diagnosis, age at the time of surgery, pre and post operative Calcitonin levels, pre and post op Metanephrine levels in patients with MEN 2A, histopathological findings, follow up and overall survival. RESULTS: Thirteen patients were identified with family history of MTC, 9 females and 4 males. Eleven carriers of mutation on RET proto-oncongene for MEN 2A and no carriers for Familial MTC. The median age at the time of diagnosis was 4.2 years (range: 1.8 to 8.2). All patients were treated with total thyroidectomy, with a median age of 6 years (range: 4.08 to 8.5). The histopathological findings demonstrated 7 cases of C-Cells nodular hyperplasia, 2 micro-carcinomas, 1 multicentric carcinoma, 1 lymphocytic thyroiditis and 2 without evidence of disease. Elevated pre operative Calcitonin levels were found in 3 cases, correlated with one histopathological finding of micro-carcinoma. All patients are disease free. CONCLUSION: In patients with genetic predisposition to suffer the disease, early prophylactic thyroidectomy is the only current available approach to prevent and cure MTC. The creation of a multidisciplinary team (Endocrinology, clinical genetics, and pediatric surgery), is necessary to study, manage and follow up patients with MEN 2A and their families.

[Propranolol for the treatment of severe hemangiomas of infancy: results from a series of 28 patients]. / Tratamiento con propranolol oral para hemangiomas infantiles graves: serie de 28 pacientes.

BACKGROUND AND OBJECTIVES: Recent reports have described the successful use of propranolol to treat severe hemangiomas of infancy. The few case series that have been reported, however, have included only a small number of patients. The aim of this study was to describe the results of oral propranolol treatment for severe hemangiomas of infancy in terms of treatment outcome and the occurrence of adverse events. PATIENTS AND METHODS: A descriptive, observational study was undertaken in a series of children with severe hemangiomas of infancy seen in the pediatric dermatology unit at Hospital Universitario Virgen del Rocío in Seville, Spain between July 2008 and December 2009. Patients were included if they had hemangiomas in the proliferative phase or involuting lesions with substantial residual deformity. All children were treated with oral propranolol (2 mg/kg/d) and followed until September 2010. Epidemiologic characteristics were analyzed along with treatment response at 3, 6, 9, 12, and 18 months; adverse events were also recorded at those times. RESULTS: Thirty-six hemangiomas were treated in 28 patients. Propranolol treatment was effective in all cases, with a good or complete response in 88.2% at 6 months. Effects were apparent within a few hours of treatment, which was effective in both growing and involuting hemangiomas. In ulcerated hemangiomas, the mean healing time was 61 days. Adverse events were mild and self-limiting. Only 2 patients discontinued treatment due to hypotension. CONCLUSIONS: In the majority of cases, oral propranolol produced rapid and sustained improvements in hemangiomas of infancy and shortened the natural course of the disease with few side effects. However, no significant reductions in symptoms or healing time were observed in ulcerated hemangiomas.

Ileocolic intussusception: Ultrasound-guided hydrostatic reduction with sedation and analgesia.

INTRODUCTION: The first-choice treatment for ileocolic intussusception is imaging-guided reduction with water, air, or barium. The objectives of the current study were to evaluate the efficacy and safety of ultrasound-guided reduction of intussusception using water in patients under sedation and analgesia. We compare this approach with our previous experience in reduction using barium under fluoroscopic guidance without sedation and analgesia and investigate what factors predispose to surgical correction. MATERIAL AND METHODS: We retrospectively reviewed cases of children with ileocolic intussusception treated in a third-level pediatric hospital during a 52-month period: during the first 24 months, reduction was done using barium and fluoroscopy without sedoanalgesia, and during the following 28 months, reduction was done using water and ultrasound with sedoanalgesia. A pediatric radiologist and a pediatrician reviewed the clinical history, surgical records, and imaging studies. RESULTS: In the 52-month period, 59 children (41 boys and 18 girls; mean age, 16.0 months) were diagnosed with ileocolic intussusception at our hospital. A total of 33 reductions (28 patients and 5 recurrences) were done using barium under fluoroscopic guidance, achieving a 61% success rate. A total of 38 reductions (31 patients and 7 recurrences) were done using water under ultrasound guidance with patients sedated, achieving a success rate of 76%. No significant adverse effects were observed in patients undergoing ultrasound-guided hydrostatic reduction under sedation, and the success rate in this group was higher (p = 0.20). The factors that predisposed to surgical reduction were greater length of the intussusception (p = 0.03), location in areas other than the right colon (p = 0.002), and a greater length of time between symptom onset and imaging tests (p = 0.08). CONCLUSION: Ultrasound-guided hydrostatic reduction of ileocolic intussusception under sedoanalgesia is efficacious and safe.

Dental caries as a side effect of infantile hemangioma treatment with propranolol solution.

We report the case of an 18-month-old boy who presented with caries in the upper central incisors associated with the use of propranolol solution for the treatment of an infantile hemangioma. This side effect of propranolol solution has not been reported before, and it may result from a sucrose-based excipient of the solution, or decreased salivation caused by beta-adrenergic antagonist effect of propranolol.

[PHACES syndrome]. / Síndrome de PHACES.

INTRODUCTION: PHACES syndrome associates a segmental facial hemangioma with cerebral malformations, aortic branches/cranial arteries anomalies, cardiac defects, eye anomalies or ventral wall defects. The aim of this study is to analyze our experience with this syndrome. MATERIAL AND METHODS: Retrospective study of the cases seen at our unit in the last year. RESULTS: We treat 4 cases; 3 girls and 1 child. Besides the segmental hemangioma they presented: 3 vascular cerebral malformations; 2 structural cardiopathies; 2 cerebral malformations, 1 microftalmia. We did not find ventral wall defects. A case received treatment with two cycles of metilprednisolone i.v. and oral prednisone, with favourable course; two cases received initial treatment with oral prednisone continued of oral propanolol in rising pattern up to 2 mg/kg/day, Obtaining both the detention of the tumour growth and regression of the lesion, with very good tolerance. A 7-year-old patient has been treated with colouring pulse laser for her residual lesions. CONCLUSIONS: When we see a segmental facial hemangioma we must perform a wide diagnostic study in order to discard a PHACES syndrome. Multidisciplinar approach to the patient by a wide expert's group gets an earlier diagnose and improves the outcome. Propranolol is a promising therapeutic alternative.

Parkes-Weber syndrome associated with a congenital short femur of the affected limb.

The association of capillary malformation, high-flow arteriovenous fistulas, and limb hypertrophy corresponds to Parkes-Weber syndrome. Most of cases are sporadic, although a first familial case has been recently reported. We report the first observation of a Parkes-Weber vascular anomaly with an underlying congenital short femur.

[Surgical management of bilateral Wilms' tumor: our experience with 18 cases]. / Manejo quirúrgico del tumor de Wilms bilateral: nuestra experiencia con 18 casos.

AIM: To show our experience in the surgical management of bilateral Wilms' tumor. METHODS: We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms' tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up. RESULTS: 65% of patients with synchronous Wilms' tumor was stage I-II, 30% stage III and 5% stage IV. 100% of patients with metachronous Wilms' tumor was stage I-II. All the tumors had favourable histology. Surgical complications were: 4 bowel pseudobstructions, 2 ureteropielic fistulae and 1 urinary cyst. 15 patients are alive (83%) with a mean follow-up of 12 years. 3 of these patients had a renal trasplant with a good evolution. One patient died of a progressive renal failure and two patients died of the evolution of the oncological disease. CONCLUSIONS: Preoperative chemotherapy allows a conservative surgical resection with a high overall survival (80-90%). Individualized surgical treatment offers a conservative surgical resection with a lower incidence of long-term renal failure.

[Incidence of metachronic contralateral inguinal hernia]. / Incidencia de la hernia inguinal contralateral metácrona.

INTRODUCTION: There are many observational studies about the incidence of metachronous contralateral inguinal hernia (MIH). Metaanalysis allows to resume the results of individual studies in one with a higher level of evidence. AIM: to know the incidence of MIH in order to decide the contralateral exploration. MATERIAL AND METHODS: We do an extensive bibliographic review in Medline, Embase and Cochrane Central. Data analysis is done with RevMan 4.2. RESULTS: 632 abstracts were screened by only one revisor who selected 27 studies: 17802 inguinal hernias and 1209 MIH. Follow up is between 6 months and 10 years. Due to high heterogeneity, a randomized effect analysis (Der Simonian and Lard) is done. The incidence of MIH is 6,96% (6,07-7,85); 14 hernia must be operated to avoid one MIH (NNT). If the original side of the hernia is left, the probability of MIH is 1.81 times higher (NNT =10). The secondary analysis indicates that there is no important publication bias. CONCLUSIONS: Rutinary bilateral inguinal exploration is not justified. Only in high anaesthetic risk patients, bilateral exploration should be suggested.

[Fast track antibiotic protocol in acute appendicitis]. / Protocolo antibiótico "fast-track" en la apendicitis aguda.

INTRODUCTION: The increase of antimicrobial resistances, has affected the efficacy of antimicrobial triple therapy, increasing appendicitis morbidity. We decided to change to a fast-track protocol of 72 hours of Cefuroxime-Metronidazol or Ertapenem. AIM: to know if our infectious morbidity rate has improved and if early dischargement is possible. MATERIAL AND METHODS: Analytic historic cohort study: Historical cohort (HC): patients intervened of appendicitis between October 2005 and October 2006. Current cohort (CC): started in June 2007. A data collection protocol is designed for both groups. Homogeneity among cohorts is proved and data are analysed (a=0,05). Statistics are analyzed by SPSS 15.0. RESULTS: 226 patients were controlled, being 110 complicated appendicitis (HC: 61/135; AC: 49/91). There are no differences among cohorts about evolution time, temperature and leukocytes rate at admission, interval to intervention, use of laparoscopy, drainage or antimicrobial prophylaxis. There is difference about the use of pre-surgical ultrasound. Admission rate has been reduced in 2.82 days, from 8.41 days in HC to 5.58 with the new antimicrobial protocol (median 7 to 4). The relative risk of developing intraabdominal abscess is 0.366 (HC: 15.7%; CC: 6.4%) (p=0.145) and readmission rate has been reduced from 9.8% to 0% (p=0.028). In 33% of cultures we have found different levels of antimicrobial resistance. No Enterococcus has been isolated. CONCLUSIONS: 1) Infectious morbidity rate has decreased with the new therapy. 2) Antimicrobial resistance and new antibiotics have exceeded Triple antimicrobial therapy. 3) It is possible an early dischargement in perforated appendicitis without an increasing of readmission rate. 4) We must know the bacterial flora in order to adapt our antibiotic therapy.

[Chromophobe renal cell carcinoma, an exceptional entity in childhood: a case report]. / Carcinoma renal de células cromófobas, una entidad excepcional en la infancia: caso clínico.

Chromophobe renal cell carcinoma (CRCC) is a rare variant of renal carcinomas arising from the intercalated cells of the distal renal tubule and representing 5% among all renal tumors. Its biological behaviour is variable, less aggresive than clear cell renal carcinoma. Histochemical, ultrastructural and molecular genetic characteristics are different from other renal carcinomas. Age at presentation is about the 6th decade of life. We report an exceptional 10 year-old boy case with a CRCC. Diagnostic and therapeutic aspects for the management of this tumor are reviewed.

[Biofeedback and electrostimulation in the treatment of non monosymptomatic enuresis]. / Biofeedback y electroestimulación como tratamiento de la enuresis no monosintomática.

AIM: To show our experience with biofeedback and electrostimulation techniques in the treatment of non monosymptomatic enuresis. METHODS: We reviewed biofeedback and electrostimulation techniques in the treatment of complicated enuresis carried out in our institution between 1995 and 2000. We report 99 patients (65 girls and 34 boys) with ages ranging from 5 to 14 years old. A complete urodinamy study divided the patients into 5 groups: detrusor-sphincter dysfunction (DSD) (47 patients), detrusor overactivity (DO) (25 patients), urge syndrome (US) (15 patients), sphincter hypertony (SH) (10 patients) and "retentionist" bladder (RB) (2 patients). Success was defined as less than three wet nights in a month. Biofeedback and electrostimulation program was designed in 10 sessions per week (20 minutes each session) with SIGMAX-Biomedical software. Tibial electrostimulation with SANS-UroSurge equipment was indicated when biofeedback and electrostimulation techniques failed (12 sessions per week, 30 minutes each session). RESULTS. The success rate has been 78.9% in DSD group, 80% in DO group, 85% in US group, 75% in SH group, 100% in RB group. CONCLUSIONS: Effectiveness of biofeedback and electrostimulation techniques in the treatment of non monosymptomatic enuresis has been reported and seems to be permanent in time.

[Ureteral meatotomy associated with vesicostomy, a possible urinary derivation?]. / Meatotomía ureteral asociada a vesicostomía, una posibilidad de derivación urinaria?

AIM: To show our experience in urinary derivation by ureteral meatotomy associated with vesicostomy. METHODS: We reviewed uni or bilateral ureteral meatotomy associated with vesicostomy carried out in our institution between 1989 and 2006. We report 20 patients (15 boys and 5 girls) with ages ranging from 1 month to 13-years-old (median 4,4-years-olds). Nine (45%) had neuropathic bladder, 6 (30%) posterior urethral valves and 5 (25%) vesicoureteral stenosis. RESULTS: Improvement of the ureteral dilatation controlled by ultrasound was demonstrated in 100% of the patients. Improvement of the renal function was observed in the group of patients (5) with renal failure. COMPLICATIONS: 2 bladder prolapses, 1 calculus and 1 postoperative bladder bleeding. CONCLUSIONS: Ureteral meatotomy associated with vesicostomy is an effective urinary derivation in children with obstructive urological conditions with an important dilatation in which ureteral reimplantation seems to be difficult. This derivation avoids bladder dysfunction and the management of the vesicostomy is comfortable by the use of a bag or the nappies.

[Neonatal diagnostic protocol of the Hirschsprung's disease]. / Fiabilidad de la manometría anorrectal para el diagnóstico de la enfermedad de Hirschsprung en neonatos: experiencia de 25 años.

UNLABELLED: Suction rectal biopsy (SRB) is the gold-standard for the diagnosis of Hirschsprung's disease (HD), but may not be applied before the first month of age. We propose a diagnostic protocol that allows the management of these patients. MATERIAL AND METHODS: A retrospective analytic study is done, analysing the results of our protocol. A rectal manometry (RM) is done to any patient with clinical symptoms of HD. When first test is positive (no reflex), we practice a weekly RM till the end of the neonatal period when we propose the SRB for the confirmation of the diagnosis. RESULTS: From 1980 to 2006 we have performed 503 tests in 391 HD's suspected neonates. Mean age in the first study was 15.19 days. In 54 cases, final diagnosis was EH. Rate of false-negative was 0.9%, sensibility 99%. Rate of non-usefull studies without sedation was 8%, being minor (2.97%) with sedation (OR = 2.853; p = 0.008). CONCLUSIONS: The protocol with a weekly RM allows the management of these patients because of a reliable diagnosis. Sedation has two positives effects: the reduction of the time of the study and the improvement of the results.

[Intraluminal impedance technique in the diagnosis of apparent life-threatening events (ALTE)]. / Importancia de la impedanciometría esofágica en el diagnóstico de los episodios aparentemente letales.

BACKGROUND: Apparent life threatening events (ALTE) affect children younger than one year. This syndrome is characterized by several symptoms: apnoea, change in colour or muscle tone, coughing or gagging. Approximately 50 percent of these children are diagnosed of an underlying pathology that explains the ALTEs, being gastroesophageal reflux one of the most important conditions to investigate. AIM: To demonstrate the effectiveness of intraluminal impedance technique for the diagnosis of the gastroesophageal reflux associated to ALTEs in infants. MATERIALS AND METHODS: Stationary esophageal manometric is used to define the low esophageal sphincter, a pH and impedance 24 hours was recorded. We determinate association between gastroesophageal reflux-ALTEs and characterisation of the reflux that occurs. RESULTS: Intraluminal esophageal impedance has been made to 16 children with the diagnosis of ALTEs between 1 and 6 months of age (mean of age 3.04 months). 23.4 episodes of reflux (9,500-31,275, P25 and P75 respectively) have been diagnosed by pHmetry whereas with impedance the number of reflux obtained were 70.88 (60.25 - 80.00), 36.21% acid and 63.78% weakly acidic. We have found 4 episodes of apnoea in one patient that seems to be connected with gastroesophageal reflux (3 nonacid and one acid event) in a statistically positive relation (SI > or = 50%; SSI > or = 10%). CONCLUSIONS: Intraluminal gastroesophageal impedance is a useful method for the diagnosis of gastroesophageal reflux in infants who present a gastric alkaline content most of time, allowing in addition, to differentiate between acid and nonacid reflux. According to our sample, the patients with the diagnosis of ALTEs do not present greater rate of gastroesophageal reflux than the healthy population of the same age. Only in one patient we have found a statistically significant relation between reflux and apnoea.

Cirugía guiada por tromboelastometría en neuroblastoma complicado con coagulación intravascular diseminada / Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation

Introducción: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. Caso clínico. Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. Conclusión. El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.(AU)

Background: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. Observation. A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. Conclusion. Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.(AU)