Infección necrotizante de tejidos blandos: experiencia durante 15 años en un hospital pediátrico / Necrotizing soft tissue infections: experience over 15 years in a pediatric hospital

INTRODUCCIÓN: La Infección Necrotizante de tejidos blandos (INTB) tiene una elevada morbimortalidad. El objetivo de este trabajo es describir del manejo perioperatorio de menores de 15 años que cursaron con INTB durante 15 años en un Hospital pediátrico. MATERIAL Y MÉTODOS: serie de pacientes identificados INTB entre 2000 y 2015 en el Hospital Roberto del Río. Se describen variables demográficas, clínicas, vacuna, tratamiento, cirugías, complicaciones, microorganismos, seguimiento, y fallecimientos. RESULTADOS: 22 pacientes, con mediana de 2 años y 9 meses de edad. 50% estaban cursando con una varicela. Dos fallecieron. Ninguno era previamente vacunado contra el virus varicela zoster. La localización fue tronco (14), extremidades (7), cuello (1). Los pacientes conscientes presentaron hiperestesia cutánea. Se realizó aseo quirúrgico con una mediana de 6,8 horas desde el inicio de la hiperestesia y 2 horas desde la sospecha diagnóstica. Los microorganismos fueron: S. pyogenes (38%) y E. coli (31%). Los antibióticos más frecuentes fueron penicilina más clindamicina. La herida se manejó con cierre primario, injertos y/o colgajos. Cinco evolucionaron con secuelas que necesitaron tratamiento. CONCLUSIÓN: Se identificó que la INTB puede presentarse con varicela. Debe sospecharse en lesiones cutáneas e hiperestesia desproporcionada. Resección amplia y precoz son fundamentales para el tratamiento.

INTRODUCTION: Necrotizing soft tissue infection (NSTI) has a high morbidity and mortality. The objective of this study is to describe the perioperative management of children under 15 years of age who have had this condition for 15 years in a pediatric hospital. MATERIAL AND METHODS: cases series of patients identified by Pathological Anatomy with NSTI during 2000 and 2015 at the Dr. Roberto del Río Hospital. Demographic variables, treatment, antecedents of vaccination, surgeries, complications, microorganisms, follow-up, and deaths are described. RESULTS: 22 patients were identified, with a median of 2 years 9 months of age (interquartile range: 13 months to 5 years y 10 months). 50% of the cases were associated to chickenpox. Two died. No patient was previously vaccinated against varicella zoster virus. The location was trunk (14), extremities (7) and neck (1). All conscious patients presented cutaneous hyperesthesia. Surgical debridement was performed with a median of 6.8 hours from the onset of hyperesthesia and 2 hours from diagnostic suspicion. The most frequent microorganisms were: Streptococcus pyogenes (38%) and Escherichia coli (31%). The most frequent antibiotics used were penicillin plus clindamycin. The wound was handled with primary closure, grafts and / or flaps. Five patients evolved with sequelae that needed treatment. CONCLUSION: It was identified that STNI in children is associated with chickenpox. It should be suspected in cutaneous lesions and disproportionate hyperesthesia. Aggressive resection is essential for treatment

Brida amniótica abdominal, reporte de un caso / Precordial pain in pediatrics. Update

Existe un amplio espectro de deformidades causadas por bandas amnióticas, siendo las extremidades la ubicación más común. La banda de constricción en el abdomen es un lugar raro para el síndrome de brida amniótica. Presentamos un caso de banda de constricción circunferencial congénita del abdomen sin otras malformaciones. La condición no afectó la ventilación, la alimentación, las deposiciones o el crecimiento. Se planificaron múltiples Z-Plasties en el abdomen antes del primer mes de vida. Revisamos la literatura que hace hincapié en la presentación clínica y el tratamiento de esta rara entidad clínica.

There is a wide spectrum of deformities caused by amniotic bands, being the extremities the most common location. The constriction band in the abdomen is a rare location for constriction band syndrome. We report a case of congenital circumferential constriction band of abdomen without other malformations. The condition did not affect ventilation, feeding, bowel movements, or growth. Multiple Z-plasties were planned in the abdomen before the first month from birth. We reviewed the literature emphasizing on the clinical presentation and management of this rare clinical entity.

[Cleft lip/palate and flour fortification with folic acid in Chile]. / Fisuras labio-palatinas y fortificación de la harina con ácido fólico en Chile: An exploratory study.

BACKGROUND: The effect of food fortification with folic acid on the incidence of lip-palate fissures (FLP) is under discussion. AIM: To calculate the rate of hospital discharges due to cleft lip and palate (CLP) and explore whether they decreased after the start of folic acid fortification in Chile. MATERIAL AND METHODS: The hospital discharge databases published by the Chilean Ministry of Health were analyzed. The trends of discharge rates due to CLP of children of less than one year of life (ICD-10 Q350-379 code) and live births (lb) were calculated from 1986 to 2012. Variables such as incidence of rubella, poverty rate, obesity in pregnant women, and percentage of women who smoked or drank alcohol in the last month were included. The relative risk (RR) of CLP pre-fortification (1986-1999) and post-fortification (2000-2010) was calculated. Mixed and Prais-Winsten models were used to adjust other variables. RESULTS: The hospital discharge rate due to CLP decreased from 1.88 x 1,000 lb during 1986-1996 to 1.68 x 1,000 lb (RR = 1.09 (95% CI 1.05-1.14, p < 0.001). Prais-Winsten and Mixed models, determined that folic acid fortification is the variable explaining this decrease. During 2004-2011, hospital discharge rates for cleft lip decreased but there was an increase in the figures for cleft lip and palate. CONCLUSIONS: The 9% decrease in hospital discharge rates for CLP is concomitant with wheat folic acid fortification.

Fisuras labio-palatinas y fortificación de la harina con ácido fólico en Chile: an exploratory study / Cleft lip/palate and flour fortification with folic acid in Chile

Background: The effect of food fortification with folic acid on the incidence of lip-palate fissures (FLP) is under discussion. Aim: To calculate the rate of hospital discharges due to cleft lip and palate (CLP) and explore whether they decreased after the start of folic acid fortification in Chile. Material and Methods: The hospital discharge databases published by the Chilean Ministry of Health were analyzed. The trends of discharge rates due to CLP of children of less than one year of life (ICD-10 Q350-379 code) and live births (lb) were calculated from 1986 to 2012. Variables such as incidence of rubella, poverty rate, obesity in pregnant women, and percentage of women who smoked or drank alcohol in the last month were included. The relative risk (RR) of CLP pre-fortification (1986-1999) and post-fortification (2000-2010) was calculated. Mixed and Prais-Winsten models were used to adjust other variables. Results: The hospital discharge rate due to CLP decreased from 1.88 x 1,000 lb during 1986-1996 to 1.68 x 1,000 lb (RR = 1.09 (95% CI 1.05-1.14, p < 0.001). Prais-Winsten and Mixed models, determined that folic acid fortification is the variable explaining this decrease. During 2004-2011, hospital discharge rates for cleft lip decreased but there was an increase in the figures for cleft lip and palate. Conclusions: The 9% decrease in hospital discharge rates for CLP is concomitant with wheat folic acid fortification.

Unusual presentation of a Leydig Cell Tumor.

SUMMARY: Leydig Cell Tumors (LCT) in children are very rare, with an incidence of 1-3% for all testicular tumors in children. Clinical presentation is testicular mass, pain and hormone alteration such as precocious puberty and gynecomastia. We present one case of LCT and his conservative management after an incidental finding. Literature is reviewed.

Urethral diverticulum after laparoscopically-assisted anorectal pull-through (LAARP) for anorectal malformation: is resection of the diverticulum always necessary?

SUMMARY OBJECTIVES: With the increased use of minimally invasive surgery, the urethral diverticulum after anorectal surgery has become an issue. The few cases reported have been managed by surgical excision. We hereby report a case of urethral diverticulum after a laparoscopically-assisted anorectal pull-through (LAARP)procedure with a successful outcome after a period of active surveillance. METHODS: A full-term boy who displayed a high anorectal malformation (ARM) and a rectoprostatic fistula underwent colostomy on the first day. He also showed associated malformations: bilateral low-grade reflux, horseshoe kidney and thoracic hemivertebrae; however, there were no signs of spinal cord tethering. Antimicrobial prophylaxis was started. RESULTS: At the age of 3 months, he underwent a LAARP with a 3 abdominal-port approach. After complete dissection of the distal bowel, the recto-prostatic fistula was identified and tied with metallic clips. A 10 mm trocar was inserted through the centre of the sphincteric complex, which had been previously identified under laparoscopic view during perineal electrical stimulation. The anorectal pull-through was accomplished without tension. The bladder remained stented for 14 days. On the 18th postoperative day, a voiding cystourethrogram (VCUG) showed a 15 X 5 mm image of the diverticulum at the level of the membranous urethra. After 6 months, a new VCUG showed a normal urethra with neither signs of the diverticulum nor strictures; persistence of grade 2 reflux on the right side and resolution of the reflux on the left. When the boy was one year old his colostomy was closed uneventfully. Six months later he had not come into the emergency since the operation and voided with a normal flow. CONCLUSION: This report suggests that LAARP is a feasible approach for ARM, although urethral diverticulum is a major concern. It may evolve without complications, and eventually resolve spontaneously. Active surveillance might be an option in selected asymptomatic patients; however a longer follow-up is advised to constitute better evidence supporting that policy.

Divertículo uretral luego de descenso anorectal asistido por laparoscopia (DARAL) en malformación anorectal: Es siempre necesario resecar el divertículo? / Urethral diverticulum after laparoscopic-assisted anorectal pull-through (laarp) for anorectal malformation: is resecting the diverticulum always necessary?

OBJETIVO: Con el uso cada vez mayor de la cirugía mínimamente invasiva, el divertículo uretral tras la cirugía anorectal se ha convertido en un problema. Los pocos casos descritos se han manejado con extirpación quirúrgica. Nosotros describimos un caso de divertículo uretral tras descenso anorectal asistido por laparoscopía (DARAL) con un resultado exitoso despues de un período de vigilancia activa.MÉTODO: Se trata de un paciente de sexo masculino, nacido a término, portador de una malformación anorectal (MAR) alta con fístula recto-prostática fue sometido a una colostomía el primer día de vida. También portador de malformaciones asociadas; reflujo bilateral de bajo grado, riñón en herradura y una hemi-vértebra torácica; sin embargo, sin signos de médula anclada. Se inició profilaxis antibiótica.RESULTADOS: A los 3 meses de edad, fue sometido a un DARAL con un abordaje abdominal de 3 puertos. Despues de la completa disección del intestino distal, la fístula recto-prostática fue identificada y ligada con clips metálicos. Un trocar de 10mm fue insertado a través del centro del complejo esfinteriano, que fue previamente identificado bajo visión laparoscópica durante la estimulación eléctrica perineal. El descenso anorectal se llevó a cabo sin tensión. La vejiga permaneció drenada con catéter uretral por 14 días. En el 18º día post-operatorio, una cistografía miccional mostró una imagen diverticular de 15 x 5mm a nivel de la uretra membranosa. Despues de 6 meses, una nueva cistografía miccional mostró una uretra normal sin signos de divertículo ni estenosis; persistencia de reflujo grado 2 a derecha y resolución de reflujo a izquierda. Al año de vida la colostomía fue cerrada sin problemas. Seis meses después, se ha mantenido libre de infección urinaria y su micción es con flujo normal(AU)

CONCLUSIÓN: Este artículo sugiere que el DARAL es un abordaje factible para MAR, aunque el divertículo uretral es una de las principales preocupaciones. Puede evolucionar sin complicaciones, y eventualmente resolverse en forma espontánea. La vigilancia activa puede ser una opción en pacientes asintomáticos seleccionados, sin embargo se recomienda un mayor seguimiento para constituir una mejor evidencia que apoye esta medida(AU)

OBJECTIVES: With the increased use of minimally invasive surgery, the urethral diverticulum after anorectal surgery has become an issue. The few cases reported have been managed by surgical excision. We hereby report a case of urethral diverticulum after a laparoscopically-assisted anorectal pull-through (LAARP)procedure with a successful outcome after a period of active surveillance.METHODS: A full-term boy who displayed a high anorectal malformation (ARM) and a recto-prostatic fistula underwent colostomy on the first day. He also showed associated malformations: bilateral low-grade reflux, horseshoe kidney and thoracic hemivertebrae; however, there were no signs of spinal cord tethering. Antimicrobial prophylaxis was started.RESULTS: At the age of 3 months, he underwent a LAARP with a 3 abdominal-port approach. After complete dissection of the distal bowel, the recto-prostatic fistula was identified and tied with metallic clips. A 10 mm trocar was inserted through the centre of the sphincteric complex, which had been previously identified under laparoscopic view during perineal electrical stimulation. The anorectal pull-through was accomplished without tension. The bladder remained stented for 14 days. On the 18th postoperative day, a voiding cystourethrogram (VCUG) showed a 15 X 5 mm image of the diverticulum at the level of the membranous urethra. After 6 months, a new VCUG showed a normal urethra with neither signs of the diverticulum nor strictures; persistence of grade 2 reflux on the right side and resolution of the reflux on the left. When the boy was one year old his colostomy was closed uneventfully. Six months later he had not come into the emergency since the operation and voided with a normal flow(AU)

CONCLUSION: This report suggests that LAARP is a feasible approach for ARM, although urethral diverticulum is a major concern. It may evolve without complications, and eventually resolve spontaneously. Active surveillance might be an option in selected asymptomatic patients; however a longer follow-up is advised to constitute better evidence supporting that policy(AU)

Tumor de células de Leydig. Presentación inusual / Unusual presentation for a leydig cell tumour

Los tumores de células de Leydig (TCL) en niños son escasos, con una incidencia de 1-3% de todos los tumores testiculares en niños. Su presentación clínica es masa testicular, dolor y alteraciones hormonales como pubertad precoz o ginecomastia. Presentamos un caso de TCL y su manejo conservador luego de su hallazgo incidental. Se revisó la literatura (AU)

Leydig Cell Tumors (LCT) in children are very rare, with an incidence of 1-3% for all testicular tumors in children. Clinical presentation is testicular mass, pain and hormone alteration such as precocious puberty and gynecomastia. We present one case of LCT and his conservative management after an incidental finding. Literature is reviewed (AU)

Neuroblastoma: forma de presentación y probabilidad de resección quirúrgica / Neuroblastoma: form of presentation and probability of surgical resection

El Neuroblastoma es uno de los tumores sólidos malignos más frecuentes en niños y puede aparecer en cualquiera de los sitios anatómicos a lo largo de la cadena ganglionar simpática, así como en la glándula suprarrenal. A diferencia de otros tumores embrionarios de niños, el pronóstico en este tipo de tumor sigue siendo incierto y está influido por una gran variedad de factores. El peor pronóstico de sobrevida se observa en estadio IV y en presencia de metástasis óseas corticales. El sitio de tumor primario también podría ser considerado predictivo de sobrevida, según algunos investigadores. Objetivo: determinar si existe relación entre la forma inicial de presentación clínica de aquellos pacientes en etapa IV con compromiso óseo y las posibilidades de tratamiento quirúrgico primario. Además, determinar la relación entre la localización del tumor primario y el pronóstico. Material y métodos: se realizó una revisión retrospectiva de 22 fichas de pacientes con neuroblastoma tratados en el Hospital Roberto del Río entre 1994 – 2004. Los datos se analizaron estadísticamente mediante pruebas Fisher- Irwing. Resultados: en 22 pacientes estudiados, la forma inicial de presentación clínica más frecuente fue la de aquellos tumores abdominales y pélvicos, seguida por tumores mediastínicos. La mayoría de los pacientes, al momento del diagnóstico, se encontraban en etapa I o en etapa IV (45 por ciento de los casos para cada etapa). De los casos que se diagnosticaron en la etapa I, la mayoría de los neuroblastomas se hallaban ubicados en mediastino. De los casos que se diagnosticaron que se encontraban en etapa IV, la mayoría tenían localización abdominal. Como tratamiento inicial a la mayoría de los casos, 36 por ciento (8), se le efectuó una biopsia del tumor; con similar frecuencia se realizó resección total del tumor como primer tratamiento (32 por ciento); en 4 casos se decidió comenzar tratamiento con quimioterapia y a 3 casos se le practicó resección parcial del tumor en primera instancia. Conclusiones: no se encontró relación estadísticamente significativa (p=>0.05) entre aquellos casos que tenían neuroblastoma en etapa IV con metástasis óseas corticales y efectuar la resección primaria del tumor.