Eventración diafragmática congénita: causa infrecuente de dificultad respiratoria / Congenital diaphragmatic eventration: Rare cause of respiratory distress

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Úlcera rectal solitaria como causa infrecuente de rectorragia / Solitary rectal ulcer syndrome as a rare cause of rectal bleeding

La úlcera rectal solitaria es un trastorno crónico, benigno, excepcional en pediatría, caracterizado por un conjunto de síntomas, como tenesmo, dolor perianal y prolapso rectal ocasional, con unos hallazgos anatomopatológicos típicos. La histología pone de manifiesto un engrosamiento de la mucosa y la muscularis mucosae, la elongación y distorsión de las glándulas, un edema de la lámina propia y, a veces, una ulceración de la lesión. Las lesiones se localizan con frecuencia en la pared anterior del recto, y la ulceración no siempre está presente. El diagnóstico suele ser tardío, dada la inespecificidad y la variabilidad clínica, y se basa en los estudios endoscópicos e histológicos. Aunque se trata de una entidad bien conocida en la edad adulta, resulta inusual en pediatría. Presentamos el caso de un adolescente de 16 años diagnosticado 4 años después del inicio de los síntomas(AU)

Solitary rectal ulcer syndrome is a rare condition in children, with benign chronic curse that is characterised by a complex of clinical and histological features. It usually appears with rectal bleeding, tenesmus and perinial pain, occasional rectal prolapsed and typical anatomopathological findings. Histologically, the presence of thickening of the mucous portion and muscularis mucosae, elongation and distortion of the glands and edema of the lamina propria and occasionally, ulceration of the lesion. The lesions are most often located on the anterior wall of the rectum, and ulceration is not always present. The diagnosis is usually delayed due to the nonspecific and clinical variability and is based on samples obtained by colonoscopy and biopsy. Although this entity is well recognized in adults, there are few pediatric cases reported in literature. We reporta 16 year-old boy case who was diagnosed 4 years after the onset of the symptoms(AU)

Addison disease after appendicitis.

A 14-year-old boy manifested acute abdominal pain, vomiting, high temperature and diarrhea. He also underwent increasing hyponatremia and hyperkalemia after appendectomy. Further testing confirmed Addison disease. The serum adrenal antibody test was positive, and other autoimmune diseases were excluded.

Antenatal diagnosis and management of a ranula.

We describe a case of congenital ranula or retention salivary cyst diagnosed at 26 weeks of gestation. The fetal karyotype was obtained by amniocentesis and chorionic villus sampling, and was normal. Follow-up ultrasound scans revealed no changes in the size or the position of the cyst. Surgical treatment was performed 2 days after a normal vaginal delivery, with excellent results.

Role of endothelium-related mechanisms in the pathophysiology of renal ischemia/reperfusion in normal rabbits.

The present study addressed the effect of interventions aimed to increase NO in the setting of acute renal ischemia/reperfusion (I/R) in uninephrectomized rabbits. In the 60-minute post-I/R period, L-arginine+superoxide (O2.-) dismutase (SOD) synergistically improved the renal functional (69.4% versus 10.4% of the pre-I/R glomerular filtration rate with or without L-arginine+SOD, respectively; p < .01) and histological parameters (82.9% decrease of medullary congestion in L-arginine+SOD, P < .01 versus vehicle) and blocked the I/R-dependent neutrophil accumulation (89.3% reduction). In spite of these results over the short term, a second set of experiments disclosed that the protection by L-arginine+SOD was no longer present at 24 and 48 hours (plasma creatinine in vehicle-treated versus L-arginine+SOD-treated animals [mg/100 mL]: 24 hours after I/R, 9.4 +/- 1.9 versus 8.07 +/- 0.65; 48 hours after I/R, 11.6 +/- 3.6 versus 9.7 +/- 0.9; P = NS in all the cases). Additional experiments were conducted using a milder 30-minute ischemic model, which showed no significant functional or histological protection by using L-arginine+SOD. In conclusion, our experiments disclosed the following: (1) the critical importance of the interaction between NO and O2.- in the acute protective effect of L-arginine (this effect not only improved renal function and histology but also reduced neutrophil accumulation) and (2) the discordance existing between the immediate protection afforded by L-arginine+SOD and the lack of protection observed at 24 and 48 hours. This finding suggests that a punctual intervention on the NO system at the time of I/R is not sufficient to reduce renal damage over the long term.

[An asymptomatic splenic hamartoma treated by conservative surgery]. / Hamartoma esplénico asintomático tratado con cirugía conservadora.

We present the case of an 8-year-old boy accidentally diagnosed of a splenic tumor by ultrasound and computed tomography scan of the abdomen. The patient was treated surgically with a conservative surgical technique (wedge resection and splenorraphy). The histologic study revealed a hamartoma and by immunohistologic study a clear differentiation with the splenic capillary hemangioma could be made.

[Meckel's diverticulum in childhood. Personal experience and actualization]. / El divertículo de Meckel en la infancia. Actualización y experiencia personal.

The authors present 40 cases of Meckel's diverticulum in pediatric patients (average age 6.8 years old). 31 of these (77.5%) were discovered casually in surgery and the other nine (22.5%) presented specific symptoms (three intestinal intussusceptions, three perforations, one hemorrhage, a wet umbilicus and an intestinal obstruction). In this group, seven abdominal radionuclide scan were practiced with 99mTc, resulting in two positive diagnosis (28.5%). All patients were treated with short ileal resection. The histopathological study revealed in nine cases (22.5%) the presence of gastric ectopic mucosa and, in one of them, pancreatic tissue was also found. The only morbidity was a postoperative intestinal obstruction that occurred several months later and that required surgery. The mortality was zero and the average hospital stay was 8.6 days.

[Bleeding intestinal angiodysplasia. Report of a pediatric case]. / Angiodisplasia intestinal sangrante. Aportación de un caso pediátrico.

This report presents a pediatric case of right colic angiodysplasia and perhaps jejunoileal with lethal evolution by the great intensity of bleeding. Are discussed the different etiopathogenesis and the difficulties for the clinicopathological diagnosis and the actual trend towards a conservative treatment (electrocoagulation versus intestinal resection).

[Sequential surgery in familial hyperlipemia. Comments on 2 pediatric cases]. / Cirugía secuencial en la hiperlipemia familiar. Comentarios a dos casos pediátricos.

The present report analyzes two cases of homozygotic familiar hypercholesterolemia. An actualization on the etiopathogenesis, the poor short term prognosis and the bad results obtained with the conservative therapy are discussed. The different surgical approaches excluding liver transplantation are reviewed. Both cases underwent staged portocaval shunts and ileal exclusions afterwards. Short and intermediate results are exposed.

[Results of surgical treatment of juvenile portal hypertension. Analysis of 31 vascular shunts]. / Resultados del tratamiento quirúrgico de la hipertensión portal infantil. Análisis de 31 derivaciones vasculares.

Thirty-one patients with ages ranging from 4 to 16 years (average 10.3 years) with portal intrahepatic hypertensión 15 and extrahepatic hypertensión 15 are presented. Decompressive surgery on an electrive basis was carried out in all patients but one in whom it was applied as a prophylactic procedure. Twelve portocaval and five splenorrenal shunts were performed in the first group while twelve mesentericocaval and five splenorrenal shunts were carried out on the second one. The overall postoperative mortality was 12.90% (three patients from the first group and one from the second one). Anastomotic thrombosis was 19.35% (one patient from the first group and five from the second one). Recurrent hemorrhage was 3.22% (one patient in the second group). Neither encephalopathy nor long-term mortality has been noticed on survivors of both groups that were followed up from three months to fifteen years. Comments are made emphasizing the importance of surgical technical aspects as well as the surgical control of the anastomotic permeability in order to achieve better results that will make possible to increase the number of indications and their precocity.

[Juvenile hepatic hydatidosis. Analysis of 40 cases]. / Hidatidosis hepática infantil. Análisis de 40 casos.

A series of 40 children (average age 8,7 years) with hepatic hydatic disease is presented. The clinical diagnosis was based on palpation of the tumour and/or hepatomegaly in 95% (38 patients). Other symptoms such as pain, fever and jaundice were less frequent (47,5%, 22,50% and 12,50%, respectively). Other diagnostic tests yielded the following results: the Casoni skin test was positive in 68,57%, the investigation of antibodies in 40% and the Weinberg reaction in 38,70%. The ultrasonography which was carried out only on the last two patients was definitive. The scintigraphy carried out on 30 patients was positive in 93,33%. Due to the polymorphism of the disease all surgical proposals are considered to be initially acceptable. However, the high rate of bile involvement resulting from our experience (55%) has induced us to favour the Lagrot surgical technique with routine surgical colangiography in 16 patients in order to achieve short postoperative periods (10,3 days) and 6,25% of morbidity (one case of biliary fistula). Following all surgical procedures, there was no mortality. No recurrence has been found within a follow-up time ranging from 1 to 14 years.

[Esophagocoloplasty in childhood. Analysis of 14 cases]. / Esofagocoloplastias en la infancia. Análisis de 14 casos.

A series of fourteen colon esophagoplasties carried out in children suffering from different pathologies (seven caustic stenosis, five of the peptic type and two atresias) is reported. The intestinal segments used were the terminal ileum right colon in seven cases and the transverse left colon in the other seven. A decreasing number of indications has been observed. The main technical aspects are commented. The results of this technique have been rewarding: a reduced morbidity with no mortality and excellent long-term function were obtained in twelve patients.

[Splenic conservative surgery in infants]. / Cirugía reparadora esplénica infantil.

We emphasize the present change of surgical attitude towards splenic injuries from radical surgery to conservative techniques. The research results from experimenting upon 12 dogs with splenorrhaphies, segmentary resections and partial heterotopic autotransplantations are presented. Splenorrhaphy clinical application was tried in 12 children followed by satisfactory results, without morbi-mortality. We considered scintigraphy with 99m technetium-sulfide colloid to be the best method known for early diagnosis of spleen injury. Likewise, we suggest the possibility of partial heterotopic autotransplantation in cases with necessary splenectomy.