Survival outcome in severe left-sided congenital diaphragmatic hernia with and without fetal endoscopic tracheal occlusion in a country with suboptimal neonatal management.

OBJECTIVE: To evaluate the impact of fetal endoscopic tracheal occlusion (FETO) on improving survival of fetuses with severe left-sided congenital diaphragmatic hernia (CDH), as compared with contemporaneous cases managed expectantly during pregnancy, in a country with suboptimal neonatal management. METHODS: In this prospective cohort study, consecutive fetuses with isolated left-sided CDH, normal karyotype and severe pulmonary hypoplasia (defined as liver herniation and observed/expected lung-to-head circumference ratio below 26%) were selected for FETO at less than 32 weeks of gestation in a single tertiary referral center in Queretaro, Mexico. Postnatal outcome (survival up to 28 days after birth) was compared between fetuses treated with FETO and contemporaneous cases with similar lung size managed expectantly during pregnancy. RESULTS: Twenty-five fetuses with isolated severe left-sided CDH treated with FETO were matched individually with 25 cases managed expectantly during pregnancy. Endotracheal placement of the balloon was performed successfully on the first attempt in all cases. The median gestational age (GA) at balloon placement was 29.1 (range, 25.6-31.8) weeks and 34.1 (range, 30.0-36.1) weeks at balloon removal. There were no technical problems with the introduction or removal of the balloon in any cases. The median GA at delivery was significantly lower in the group treated with FETO than in those managed expectantly (35.3 vs 37.7 weeks; P = 0.04). The survival rate was significantly higher in the group treated with FETO than in those without fetal intervention (32% vs 0%; P < 0.001). CONCLUSION: In settings with suboptimal neonatal management, FETO was associated with improved neonatal survival in fetuses with isolated left-sided CDH and severe pulmonary hypoplasia. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

Resultado de supervivencia en una hernia diafragmática congénita grave del lado izquierdo, con y sin oclusión traqueal endoscópica fetal en un país con un tratamiento neonatal subóptimo OBJETIVO: Evaluar el impacto de la oclusión traqueal endoscópica fetal (OTEF) en la mejora de la supervivencia de los fetos con hernia diafragmática congénita (HDC) grave del lado izquierdo, en comparación con los casos actuales tratados como embarazo gestante, en un país con un tratamiento neonatal subóptimo. MÉTODOS: En este estudio prospectivo de cohortes, se seleccionaron fetos consecutivos con HDC aislada del lado izquierdo, cariotipo normal e hipoplasia pulmonar grave (definida como hernia hepática y una proporción observada/esperada de la circunferencia pulmonar-cabeza inferior al 26%) para una OTEF antes de las 32 semanas de gestación, en un único centro de medicina especializada terciaria en Querétaro (México). El resultado postnatal (supervivencia hasta los 28 días después del nacimiento) se comparó entre fetos tratados con OTEF y los casos contemporáneos con tamaño pulmonar similar, tratados como embarazo gestante. RESULTADOS: Veinticinco fetos con HDC grave aislada del lado izquierdo que habían sido tratados con OTEF fueron emparejados individualmente con 25 casos tratados como embarazo gestante. La colocación endotraqueal del globo se realizó con éxito en el primer intento en todos los casos. La mediana de la edad gestacional (EG) en el momento de la colocación del globo fue de 29,1 (rango, 25,6-31,8) semanas y 34,1 (rango, 30,0-36,1) semanas cuando se retiró el globo. En ningún caso hubo problemas técnicos con la introducción o la retirada del globo. La mediana de la EG en el momento del parto fue significativamente menor en el grupo tratado con OTEF que en el grupo tratado como gestante (35,3 vs 37,7 semanas; P=0,04). La tasa de supervivencia fue significativamente más alta en el grupo tratado con OTEF que en los casos sin intervención fetal (32% vs 0%; P<0,001). CONCLUSIÓN: En los entornos con un tratamiento neonatal subóptimo, la OTEF se asoció con una mejora de la supervivencia neonatal en los fetos con HDC aislada del lado izquierdo y con hipoplasia pulmonar grave. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

[Laparoscopic splenectomy in hematologic diseases in children. Analysis of 11-year experience]. / Esplenectomía laparoscópica por enfermedades hematológicas en niños. Análisis de 11 años.

OBJECTIVES: A common indication for splenectomy in children is hematologic diseases. Indications, clinical course and complications of laparoscopic splenectomy are shown; factors associated with open conversion are analyzed. MATERIAL AND METHODS: Retrospective, longitudinal, descriptive series of laparoscopic splenectomy during 11 years and its long-term follow-up. RESULTS: We studied 88 laparoscopic splenectomies. Average age was 9 years and weight 30 kg. The indication was hemolytic anemia in 77% and thrombocytopenic purpura in 18%. Eleven percent was operated with severe thrombocytopenia and two patients urgently; cholecystectomy was done in 13%. The median surgical time was 180 minutes and bleeding 100 ml. Conversion rate was 5.7% mainly because of persistent bleeding, being risk bleeding greater than 300 ml and the need for red cell concentrate transfusion. The median length of stay was 3 days, longer in those affected by purpura. Three patients developed pneumonia and one, intra-abdominal abscess. Follow-up was 3.6 years average with two late complications. Treatment response was cure in 72%, improvement in 11.3% and failure in 6.8%. DISCUSSION: It is feasible to operate patients under 5 years of age and patients with anemia or thrombocytopenia at the time of surgery without major problems. The conversion rate is low and risk factors for conversion are bleeding more than 300 ml and needing to transfuse erythrocyte concentrate.

INTRODUCCION: Una indicación frecuente de esplenectomía en niños son las enfermedades hematológicas. Se muestran las indicaciones, curso clínico y complicaciones en niños con esplenectomía laparoscópica y se analizan los factores de conversión a cirugía abierta. MATERIAL Y METODOS: Estudio de cohorte, longitudinal, descriptivo de esplenectomía laparoscópica en un periodo de 11 años y su seguimiento a largo plazo. RESULTADOS: Se analizaron 88 esplenectomías laparoscópicas. Edad promedio 9 años y peso 30 kg. La indicación fue anemia hemolítica en el 77% y púrpura trombocitopénica en el 18%. El 11% se operó con trombocitopenia grave y dos pacientes en forma urgente, se hizo colecistectomía en el 13%. La mediana de tiempo quirúrgico fue de 180 minutos con sangrado de 100 ml. Se convirtió el 5,7% por sangrado persistente, siendo los factores de riesgo el sangrado mayor a 300 ml y la necesidad de transfusión de concentrado eritrocitario. La mediana de estancia fue 3 días, superior en los pacientes afectos de púrpura. Tres pacientes desarrollaron neumonía y uno, absceso intra-abdominal. El seguimiento promedio es de 3,6 años con dos complicaciones tardías. Hubo curación con normalización del hemograma y sin necesidad de transfusiones en el 72%, mejoría con necesidad de transfusiones ocasionales en el 11,3% y fallo con requerimientos transfusionales iguales a los preoperatorios en el 6,8% de los pacientes. DISCUSION: Es posible operar a pacientes menores de 5 años y a pacientes con anemia o trombocitopenia en el momento de la cirugía, sin mayor complicación. La tasa de reconversión es baja y los factores de riesgo para reconversión son un sangrado mayor de 300 ml y la necesidad de transfundir concentrado de hematíes.

Esplenectomía laparoscópica por enfermedades hematológicas en niños. Análisis de 11 años / Laparoscopic splenectomy in hematologic diseases in children. Analysis of 11-year experience

Introducción. Una indicación frecuente de esplenectomía en niños son las enfermedades hematológicas. Se muestran las indicaciones, curso clínico y complicaciones en niños con esplenectomía laparoscópica y se analizan los factores de conversión a cirugía abierta. Material y métodos. Estudio de cohorte, longitudinal, descriptivo de esplenectomía laparoscópica en un periodo de 11 años y su seguimiento a largo plazo. Resultados. Se analizaron 88 esplenectomías laparoscópicas. Edad promedio 9 años y peso 30 kg. La indicación fue anemia hemolítica en el 77% y púrpura trombocitopénica en el 18%. El 11% se operó con trombocitopenia grave y dos pacientes en forma urgente, se hizo colecistectomía en el 13%. La mediana de tiempo quirúrgico fue de 180 minutos con sangrado de 100 ml. Se convirtió el 5,7% por sangrado persistente, siendo los factores de riesgo el sangrado mayor a 300 ml y la necesidad de transfusión de concentrado eritrocitario. La mediana de estancia fue 3 días, superior en los pacientes afectos de púrpura. Tres pacientes desarrollaron neumonía y uno, absceso intra-abdominal. El seguimiento promedio es de 3,6 años con dos complicaciones tardías. Hubo curación con normalización del hemograma y sin necesidad de transfusiones en el 72%, mejoría con necesidad de transfusiones ocasionales en el 11,3% y fallo con requerimientos transfusionales iguales a los preoperatorios en el 6,8% de los pacientes. Discusión. Es posible operar a pacientes menores de 5 años y a pacientes con anemia o trombocitopenia en el momento de la cirugía, sin mayor complicación. La tasa de reconversión es baja y los factores de riesgo para reconversión son un sangrado mayor de 300 ml y la necesidad de transfundir concentrado de hematíes

Objectives. A common indication for splenectomy in children is hematologic diseases. Indications, clinical course and complications of laparoscopic splenectomy are shown; factors associated with open conversion are analyzed. Methods. Retrospective, longitudinal, descriptive series of laparoscopic splenectomy during 11 years and its long-term follow-up. Results. We studied 88 laparoscopic splenectomies. Average age was 9 years and weight 30 kg. The indication was hemolytic anemia in 77% and thrombocytopenic purpura in 18%. Eleven percent was operated with severe thrombocytopenia and two patients urgently; cholecystectomy was done in 13%. The median surgical time was 180 minutes and bleeding 100 ml. Conversion rate was 5.7% mainly because of persistent bleeding, being risk bleeding greater than 300 ml and the need for red cell concentrate transfusion. The median length of stay was 3 days, longer in those affected by purpura. Three patients developed pneumonia and one, intra-abdominal abscess. Follow-up was 3.6 years average with two late complications. Treatment response was cure in 72%, improvement in 11.3% and failure in 6.8%.Discussion. It is feasible to operate patients under 5 years of age and patients with anemia or thrombocytopenia at the time of surgery without major problems. The conversion rate is low and risk factors for conversion are bleeding more than 300 ml and needing to transfuse erythrocyte concentrate

Anesthetic risk factors associated with early mortality in pediatric liver transplantation.

INTRODUCTION: Early mortality in pediatric patients after liver transplantation (30 days) may be due to surgical and anesthetic perioperative factors. OBJECTIVE: To identify anesthetic risk factors associated with early mortality in pediatric patients who undergo liver transplantation (OLT). MATERIALS AND METHODS: This retrospective study of all patients who underwent a deceased or living donor liver transplantation evaluated demographic variables of age, weight, gender, degree of malnutrition, and etiology, as well as qualitative variables of anesthesia time, bleeding, massive transfusion, acid-base balance, electrolyte and metabolic disorders, as well as graft prereperfusion postreperfusion characteristics. Chi-square tests with corresponding odds ratio (OR) and 95% confidence intervals as well as Interactions were tested among significant variables using multivariate logistic regression models. P < or =.05 was considered significant. RESULTS: We performed 64 OLT among whom early death occurred in 20.3% (n = 13). There were deaths associated with malnutrition (84.6% vs 43.6%) in the control group (P < .01); massive bleeding, 76.9% (n = 10) versus 25.8% in the control group (P < .05) including transfusions in 84.6% (n = 11) versus 43.6% in the control group (P < .03); preperfusion metabolic acidosis in 84.6% (n = 11) versus 72.5% (n = 37; P < .05); posttransplant hyperglycemia in 69.2% (n = 9) versus 23.5% (n = 12; P < .01); and postreperfusion hyperlactatemia in 92.3% (n = 12) versus 68.6% (n = 35; P < .045). CONCLUSION: Prereperfusion metabolic acidosis, postreperfusion hyperlactatemia, and hyperglycemia were significantly more prevalent among patients who died early. However, these factors were exacerbated by malnutrition, bleeding, and massive transfusions. Postreperfusion hypokalemia and hypernatremia showed high but not significant frequencies in both groups.

Experience of a successful pediatric liver transplant program in Mexico.

Orthotopic liver transplantation (OLT) has been very difficult to develop in Mexico and for many years its occurrence was anecdotal. This report presents the results of a pediatric liver transplant program, analyzing the variables that affect outcomes. Between June 1998 and March 2004, 35 OLT were performed in 34 recipients including 80% cadaveric whole-organ grafts and 20% segmental grafts, with 11% from cadaveric and 9% from living donors. Most of the recipients were infants or toddlers weighing less than 15 kg. There was only 1 case of arterial thrombosis (2.8%); the graft was saved with a Kasai procedure. Biliary complications were present in 22% of cases, all resolved with reoperations. Posttransplant cytomegalovirus infection or reactivation (28%), acute rejection (25%), or posttransplant lymphoproliferative disorders (5.7%) were not a cause of graft or patient loss. Overall, 1- and 5-year patient survival rates are 77.1% and 74.2%, respectively; however, when the 1998-2000 cohort was compared with the 2001-2004 cohort, there was a significant difference in survival (P = .004). The 1-year patient survival for the later group is 91.6%. We performed the first successful living donor liver transplantation and the first simultaneous liver-kidney transplantation in a child in our country. Our results demonstrate that pediatric liver transplantation is a feasible undertaking in Mexico, with survival rates comparable to those of foreign centers.

Infantile hypertrophic pyloric stenosis: a comparative study of pyloric traumamyoplasty and Fredet-Ramstedt pyloromyotomy.

PURPOSE: The aim of this study was to compare the incidence of surgical complications (duodenal perforation, postoperative vomiting, wound infection or dehiscence, incisional hernia) between 2 different surgical techniques for the resolution of hypertrophic pyloric stenosis in children. METHODS: A clinically controlled, randomized study with follow-up from 24 to 36 months was conducted. One hundred children between 15 days and 2 months old, who underwent surgical resolution of hypertrophic pyloric stenosis, were put randomly into 2 groups: I, pyloric traumamyoplasty (n = 43); II, Fredet-Ramstedt pyloromyotomy (n = 57). Both groups were controlled for the main demographic variables. Postoperative follow-up was blind for the surgical team. Statistical analysis was done with simple frequencies, percentages, Student's t test, and chi(2). RESULTS: There was not a single case of duodenal perforation, incomplete pyloromyotomy, wound infection, dehiscence, or incisional hernia in any group (P value, not significant). Postoperative emesis was present in 8 patients, uniformly distributed between groups. The operating room time for traumamyoplasty was 39.3 +/- 16.4 minutes versus 54 +/- 16.4 minutes for pyloromyotomy (P =.0003). CONCLUSIONS: This controlled study proves that traumamyoplasty is a simple procedure, quicker to perform, and as safe as pyloromyotomy for the treatment of infantile hypertrophic pyloric stenosis in children. For these reasons, the authors believe it should be considered as an alternative.

Intestinal anastomosis in children: a comparative study between two different techniques.

PURPOSE: The aim of this study was to compare the incidence of surgical complications between two different surgical techniques for intestinal anastomosis in children. METHODS: This was a clinically controlled, randomized study with blind follow-up from 18 to 36 months performed at the Reference Government Hospital in Mexico City. Eighty-six children required intestinal anastomosis, ages ranged between 1 month and 16 years, with emergency or elective surgery. Anastomoses of duodenum, rectum, with enteroplasty or protected with a proximal stoma were excluded. Two randomized groups were formed: (1) anastomosis with one layer of suture (Gambee stitches) and (2) with two layers of suture (first with Connel-Mayo stitches then with Lembert). Both groups were controlled in the principal variables without differences, and the follow-up concerning postoperative recovery was blind for the surgical team. RESULTS: Forty-two cases in group 1 and 44 in group 2 were compared. Intestinal dehiscence was found in 5 of 86 (5.8%), two from group I and three from group II (P value, not significant). Surgical time for anastomosis with one layer was an average of 26 minutes versus 43 minutes with two layers (P<.001). There were no stenoses within the follow-up period. CONCLUSIONS: This study proves that intestinal anastomosis with one layer of suture is as safe as anastomosis with two layers in children, and the time spent for completion of the procedure is significantly less with one plane of suture. For those reasons, it is the method of choice for intestinal anastomosis in children.

[Posterior perineal hernia. Report of a case and review of the literature]. / Hernia perineal posterior. Informe de un caso y revisión de la literatura.

We report a case of a seven months old female patient with diagnosis of generalized primary elastosis or cutis laxa, that also presented a posterior perineal hernia; it was treated with surgery (abdominal and perineal) and the postoperatory evolution was favorable. In the literature it had been reported around 100 cases of posterior perineal hernia; but most of them occurred in adults patients of the female sex, with a high proportion of pregnancy or as a result of radical cancer surgery like pelvic exenteration. This is the first case of posterior perineal hernia in the Hospital Infantil de Mexico Federico Gomez and the third founded in pediatric age in the literature.

[Adrenal abscess in the newborn: a case report and review of the literature]. / Absceso adrenal en el recién nacido: informe de un caso y revisión de la literatura.

This is a case report concerning a 22 day newborn referred to us having an abdominal tumor, fever, an irritable nature and feeding refusal, born dystocically due to the retention of the shoulders and having an Apgar score of 5/8. When admitted the presence of the tumor was corroborated in the right flank occupying the right renal fossa and measuring 5 x 8 cm, non-mobile and painless. Studies revealed a diagnosis of calcified right adrenal hemorrhage versus neuroblastoma. During surgery a right adrenal abscess was found which was drained and a biopsy sample was taken for further study. The culture of the pus developed Proteus mirabilis. Pathology reported it to be an abscessed right adrenal hematoma which was partially calcified. The patient's wound became infected but was later sent home under good conditions. Only 15 other cases with this pathology are reported in the literature, this would be the 16th case. An emphasis is placed on including this pathology as part of the differential diagnosis of retroperitoneal tumors in the newborn.

[Congenital hepatic fibrosis associated with choledochal cyst]. / Fibrosis hepática congénita asociada a quiste del colédoco.

The purpose of this work is to report the case of a one-year and eleven-month-old female child who presented with an asymptomatic hepatic mass; on x-ray examination a well limited round tumor was seen as well as renal cysts. A choledochal cyst was found upon laparotomy; the liver biopsy exhibited congenital hepatic fibrosis. This association is not clearly defined in the literature review made, making this perhaps the first well-documented case with this combination. We analyze the pathogenic mechanisms of both disorders and conclude that choledochal cyst is probably not related to hepatorenal fibropolycystic disease; this could be a casual association in our patient.

Malacoplaquia del colon: utilidad del betanecol. / Malakoplakia of the colon usefullness of bethanechol

Se presenta un caso de malacoplaquia del colon en un nino de ocho anos siete meses de edad, cuya evolucion clinica fue torpida a pesar del manejo intensivo presentando mejoria clinica muy evidente al asociar betanecol al tratamiento. Asi mismo se revisa la literatura medica sobre la experiencia en ninos y se comentan los aspectos bigentes en relacion a la fisiopatologia del padecimiento asi como el posible efecto especifico del medicamento en esta enfermedad. Finalmente, se sugiere que el tratamiento debe hacerse con: antibioticos de amplio espectro, betanecol y acido ascorbico, asociacion el manejo intensivo

Aganglionosis del colon. / Total colonic aganglionosis

Se presenta la experiencia del Hospital de Pediatria del C.M.N., en cuatro pacientes con aganglionosis total del colon. Se hace hincapie en el diagnostico oportuno y se valora el resultado obtenido con la tecnica de Lester-Martin. En los dos pacientes en quienes se practico esta, las pruebas de absorcion intestinal demostraron deficit para la vitamina B12 y grasas, encontrandose el resto dentro de la normalidad.Son continentes y su desarrollo y crecimiento estan ligeramente abajo de lo normal