RESUMEN
Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the pediatric surgeon. We report the case of a new born, who was operated at the age of three days for malrotation with volvulus, treated by Ladd procedure. Postoperatively, we noticed persistent abdominal distension and emission of a small amount of meconium every 4 to 5 days. The barium enema showed a non-functional microcolon. Surgical exploration on the 24th day found an ileo-ileal transition zone located 60âcm distal to the ligament of Treitz. Extemporaneous biopsies from the colon and mid-ileum confirmed the absence of ganglion cells. We performed an ileostomy at 50âcm from duodeno-jejunal flexure. Unfortunately, the patient succumbed to nosocomial infection at 33 days of age.This case was a challenging scenario for us where a diagnosis of complicated malrotation had obscured the Hirschsprung's disease.
Asunto(s)
Colon/anomalías , Enfermedad de Hirschsprung , Ileostomía , Enfermedades del Recién Nacido , Obstrucción Intestinal , Vólvulo Intestinal/cirugía , Intestinos , Enema Opaco/métodos , Biopsia/métodos , Colon/diagnóstico por imagen , Colon/fisiopatología , Infección Hospitalaria/diagnóstico , Infección Hospitalaria/etiología , Resultado Fatal , Femenino , Enfermedad de Hirschsprung/diagnóstico , Enfermedad de Hirschsprung/fisiopatología , Enfermedad de Hirschsprung/cirugía , Humanos , Ileostomía/efectos adversos , Ileostomía/métodos , Recién Nacido , Enfermedades del Recién Nacido/diagnóstico , Enfermedades del Recién Nacido/fisiopatología , Enfermedades del Recién Nacido/cirugía , Obstrucción Intestinal/diagnóstico por imagen , Obstrucción Intestinal/etiología , Obstrucción Intestinal/fisiopatología , Vólvulo Intestinal/diagnóstico , Vólvulo Intestinal/etiología , Intestinos/anomalías , Intestinos/inervación , Intestinos/patología , Intestinos/fisiopatologíaRESUMEN
Cardiovascular events are more prevalent in chronic kidney disease than in the general population, being the main cause of morbi-mortality. The physiopathology explaining this association remains complex. Thus, research for new therapies to prevent cardiovascular events in chronic kidney disease is a major issue. Epoxyeicosatrienoic acids, products of the arachidonic acid metabolism, are endothelium-derived hyperpolarizing factors with vasodilatory, anti-inflammatory, thrombolytic, pro-angiogenic and anti-apoptotic properties. A decrease in the bioavailability of epoxyeicosatrienoic acids has been observed in many cardiovascular diseases such as hypertension, myocardial infarction or diabetes. Moreover, human studies of genetic polymorphisms of soluble epoxide hydrolase, the enzyme degrading epoxyeicoatrienoic acids, have shown that allelic variants related to an increase in its activity is associated with higher risk of cardiovascular events. Modulation of epoxyeicosatrienoic acids by soluble epoxide hydrolase inhibitors in some cardiovascular diseases induces structural improvements in the heart, vessels and kidneys, including decrease in cardiomyocyte hypertrophy, reduction in cardiac and renal interstitial fibrosis, improvement in renal hemodynamics, and prevention of endothelial dysfunction. In this context, increasing the bioavailability of epoxyeicosatrienoic acids appears to be an interesting therapeutic option in the prevention of cardiovascular events related to chronic kidney disease.
Asunto(s)
Enfermedades Cardiovasculares/etiología , Enfermedades Cardiovasculares/prevención & control , Eicosanoides/antagonistas & inhibidores , Insuficiencia Renal Crónica/complicaciones , Eicosanoides/metabolismo , Eicosanoides/fisiología , Compuestos Epoxi , HumanosRESUMEN
Ovarian masses requiring surgical intervention are uncommon in the pediatric population. Our aim is to report results of a multicentric Tunisian study concerning the clinical practice and the management of pediatric ovarian masses and to identify the factors that are associated with ovarian preservation. Between January 2000 and December 2015, 98 pediatric patients (<14 years) were surgically treated for ovarian masses at the five pediatric surgery departments in Tunisia. Ninety-eight patients were included in this study. The mean age of the patients at time of surgery was 8.46 ± 4.87 years. Sixty-three ovarian masses (64.3%) were non-neoplastic lesions, 24 (24.5%) were benign tumors, and 11 (11.2%) were malignant neoplasms. Conservative surgery (ovarian-preserving surgery) was successfully performed in 72.4% of the benign lesions, whereas only three patients (27.3%) with malignant tumors underwent ovary-sparing tumor resection (p < .001). The mean diameter of the tumors in the patients who underwent oophorectomy was significantly larger than that in the patients who underwent conservative surgery (7.8 ± 3.9 cm vs. 5.7 ± 2.9 cm, respectively, p = .001). In our study, the risk factors for oophorectomy were a malignant pathology and large tumor size. In accordance with the Gynecologic Cancer Intergroup consensus, we recommend that surgical management of ovarian masses in children should be based on ovarian-preserving surgery.
Asunto(s)
Neoplasias Ováricas/cirugía , Ovariectomía , Ovario/cirugía , Adolescente , Niño , Preescolar , Femenino , Preservación de la Fertilidad , Humanos , Neoplasias Ováricas/patología , Ovario/patología , Estudios Retrospectivos , TúnezRESUMEN
This study presents a methodological approach for the visualization of the glycocalyx by electron microscopy. The glycocalyx is a three dimensional network mainly composed of glycolipids, glycoproteins and proteoglycans associated with the plasma membrane. Since less than a decade, the epithelial and endothelial glycocalyx proved to play an important role in physiology and pathology, increasing its research interest especially in vascular functions. Therefore, visualization of the glycocalyx requires reliable techniques and its preservation remains challenging due to its fragile and dynamic organization, which is highly sensitive to the different process steps for electron microscopy sampling. In this study, chemical fixation was performed by perfusion as a good alternative to conventional fixation. Additional lanthanum nitrate in the fixative enhances staining of the glycocalyx in transmission electron microscopy bright field and improves its visualization by detecting the elastic scattered electrons, thus providing a chemical contrast.
Asunto(s)
Endotelio/ultraestructura , Glicocálix/ultraestructura , Mucosa Intestinal/ultraestructura , Microscopía Electrónica de Transmisión/métodos , Coloración y Etiquetado/métodos , Animales , Lantano/química , Masculino , Perfusión , Ratas , Ratas Wistar , Manejo de Especímenes/métodosRESUMEN
We report 2 patients with rectal and low sigmoid atresia operated on, respectively, at 6 and 3 months of age using the transanal approach, similar to the transanal technique for Hirschsprung disease, after exploratory laparotomy with colostomy at birth. There were no intraoperative or postoperative complications after a follow-up time of 2 years. After closure of the colostomy, both patients had no fecal incontinence. The transanal approach is a safe and effective technique in the management of rectal and sigmoid atresia.
Asunto(s)
Colon Sigmoide/anomalías , Atresia Intestinal/cirugía , Recto/anomalías , Canal Anal , Colon Sigmoide/diagnóstico por imagen , Colon Sigmoide/cirugía , Colostomía , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Atresia Intestinal/diagnóstico por imagen , Laparotomía , Masculino , Radiografía , Recto/diagnóstico por imagen , Recto/cirugíaRESUMEN
BACKGROUND: Esophageal perforation is uncommon and often iatrogenic. AIM: To report a neonatal case of esophageal perforation. CASE: A premature newborn boy was admitted with a diagnosis of oesophageal atresia after several unsuccessful attempts to insert an orogastric tube. A chest x-ray showed a "high pouch" with a gasless intestine. At operation, no atresia was found and a large traumatic perforation of the lower cervical segment was identified, requiring cervicotomy and primary closure. Oral feeding was started after 3 weeks. As a result, nosocomial sepsis occurred, causing death in the third month after surgical treatment. CONCLUSION: Oesophageal perforation in the neonate is often iatrogenic and may mimic oesophageal atresia. The authors highlight the importance of early diagnosis and management.
Asunto(s)
Perforación del Esófago/diagnóstico , Perforación del Esófago/etiología , Enfermedad Iatrogénica , Intubación Intratraqueal/efectos adversos , Diagnóstico Diferencial , Atresia Esofágica/diagnóstico , Perforación del Esófago/cirugía , Humanos , Recién Nacido , MasculinoAsunto(s)
Abdomen Agudo/etiología , Duodeno/anomalías , Páncreas , Enfermedad Aguda , Niño , Femenino , HumanosRESUMEN
Hydatid cyst disease is still a significant clinical problem in endemic regions. The lungs and the liver are most often involved in the child. A mediastinal localisation is rare and represents 0.1 to 4% of all cases of hydatidosis. The authors reports on two cases and provides a review of the literature. In a retrospective review of the last 12 years, the authors treated two cases with a mediastinal localisation out of total of 235 intrathoracic hydatid cysts arising in 222 patients (0.85%). Two girls, one 12 and the other 13 years old, were admitted with a previous history of chest pain. The chest x-ray revealed an homogenous opacity of 150 mm in the first case and 50 mm in the second. Computed tomography in the second case revealed a cyst in the posterior mediastinum. The abdominal ultrasound was normal in both cases. The patients were approached through a posterolateral thoracotomy and the diagnosis was confirmed macroscopically. The cysts were intact and located on the left side of the posterior mediastinum. The operative field was isolated and protected with hypertonic saline solution. Hydatid fluid was aspirated, followed by the extraction of the hydatid membrane and subtotal excision of the residual cavity in both cases. The postoperative course was uneventful without any relapse or other organ involvement during four and three years, respectively. The authors notes that hydatidosis should be considered in the differential diagnosis of a mediastinal cyst mass. Because of the benign nature of the disease, they emphasize that surgical treatment must be conservative.
Asunto(s)
Equinococosis/diagnóstico , Enfermedades del Mediastino/diagnóstico , Adolescente , Niño , Diagnóstico Diferencial , Equinococosis/patología , Equinococosis/cirugía , Femenino , Estudios de Seguimiento , Humanos , Quiste Mediastínico/diagnóstico , Quiste Mediastínico/patología , Quiste Mediastínico/cirugía , Enfermedades del Mediastino/patología , Enfermedades del Mediastino/cirugía , Estudios Retrospectivos , Toracotomía , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVES: This report describes different clinical pictures of cystic pulmonary malformation (CPM) and problems in diagnosis. PATIENTS AND METHODS: Cases of CPM between 01 January 1994 and 31 December 2004 diagnosed in our institution were reviewed. RESULTS: Thirty-three cases of CPM were diagnosed in 30 children. They consisted of 17 boys and 13 girls ranging from 20 days to 16 years of age at the time of the diagnosis. The CPM included: 17 cases of congenital lobar emphysema (CLE), seven bronchogenic cysts (BC), five cystic adenomatoid malformations (CAM) and four pulmonary sequestrations (PS). Three patients presented two associated lung malformations. The mean ages at the time of diagnosis varied from 2 to 88 months. The symptoms consisted of respiratory distress (n=14, 46.6%); recurrent attacks of respiratory embarrassment (n=6, 20%); pulmonary infection (n=8, 26.6%) associated with haemoptysis in two cases; haemothorax (n=1) and a chance discovery (n=1). Radiological investigations led to the diagnosis in all cases of CLE and CAM although it contributed less to the diagnosis of BC and PS. Twenty-nine patients required chirurgical treatment involving lobectomy (n=22), pneumonectomy (n=2) and cystectomy (n=8). The histopathological examinations confirmed the diagnosis in all cases and rectified the preoperative diagnosis in four cases. Except for one patient with CLE, who died a few days after a lobectomy due to acute nosocomial pneumonia, the postoperative period was uneventful in 26 children with a mean of follow-up of 24 months (4 months to 7 years). Three patients developed transient and episodic attacks of dyspnoea. CONCLUSION: CPM may be responsible for many clinical and radiological pictures that present difficulties in their diagnosis. Polymorphism is related to the type of malformation, its topography and the evolutive complications.
Asunto(s)
Quiste Broncogénico/congénito , Quiste Broncogénico/diagnóstico , Secuestro Broncopulmonar/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Ecocardiografía Doppler , Imagen por Resonancia Magnética , Enfisema Pulmonar/congénito , Enfisema Pulmonar/diagnóstico , Tomografía Computarizada por Rayos X , Adolescente , Quiste Broncogénico/cirugía , Secuestro Broncopulmonar/cirugía , Niño , Preescolar , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Neumonectomía , Enfisema Pulmonar/cirugía , Sensibilidad y Especificidad , Ultrasonografía PrenatalRESUMEN
Bronchial foreign body inhalation is a frequent and potentially serious accident in children. It can provoke long-term complications such as bronchiectasis and recurrent pulmonary infections. The foreign body is generally blocked in a bronchus and induces obstructive emphysema with superinfection. We report 2 cases of bronchial inhalation of a migrating cereal-ear, eliminated by parietal fistulization, in 9- and 11-year-old boys.
Asunto(s)
Accidentes , Grano Comestible , Migración de Cuerpo Extraño/complicaciones , Migración de Cuerpo Extraño/diagnóstico , Axila/patología , Fístula Bronquial/diagnóstico , Fístula Bronquial/etiología , Bronquiectasia/etiología , Broncoscopía , Niño , Fístula Cutánea/diagnóstico , Fístula Cutánea/etiología , Humanos , Inhalación , Masculino , Plantas , Neumotórax/etiología , Enfermedad Pulmonar Obstructiva Crónica/etiología , Remisión Espontánea , Tórax/patología , Factores de Tiempo , Resultado del TratamientoAsunto(s)
Secuestro Broncopulmonar/diagnóstico por imagen , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico por imagen , Disnea/diagnóstico por imagen , Fiebre de Origen Desconocido/diagnóstico por imagen , Hernias Diafragmáticas Congénitas , Angiografía , Secuestro Broncopulmonar/cirugía , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Diagnóstico Diferencial , Femenino , Hernia Diafragmática/diagnóstico por imagen , Humanos , Lactante , Neumonectomía , Tomografía Computarizada por Rayos XRESUMEN
Foetus-in-fetu (FIF) is a rare congenital condition in which a vertebrate foetus is incorporated within its twin. The authors report the case of a newborn girl with prenatal ultrasonographic diagnosis of an intra-abdominal mass. Abdominal ultrasonography and computed tomography of the abdomen showed a heterogeneous cystic mass containing multiple calcifications. The patient had a laparotomy at 18 days of age with excision of a well-encapsulated 6 x 5 cm retroperitoneal mass, containing many organs. Anatomicopathologic examination showed a relatively well-differentiated FIF attached to an amniotic sac by a rudimentary umbilical cord. Encephalon, coroidal plexus, vertebral bodies, rudimentary limbs, thyroid gland and teeth were identified. The postoperative period was uneventful.
Asunto(s)
Feto/anomalías , Abdomen/anomalías , Bromhexina , Calcinosis/etiología , Calcinosis/patología , Femenino , Muerte Fetal/patología , Humanos , Recién Nacido , Embarazo , Resultado del Embarazo , Radiografía Abdominal , Gemelos Monocigóticos , Ultrasonografía PrenatalRESUMEN
The nasal angiocentric T-cell-natural killer cell lymphoma is an aggressive tumor, exceptional in Tunisia, which is observed especially in Asia, Mexico and South America. We report the case of an 11-year-old boy, presenting with an exuberant and ulcerative tumor of the right nasal pit. Radiological investigation evidenced a right ethmoïdo-frontal tumor. Evolution was characterized by the installation of abdominal pain and fever, the evidenciation of a mass within the head of the pancreas that compressed the extra hepatic biliary duct and Wirsung's canal and was associated to hyperamylasemia. Biopsy under ultrasound revealed a histiocytic nature. Nasal biopsy found histiocytic and lymphocytic cells and led to a diagnosis of rhinoscleroma. The absence of improvement under antibiotic treatment and the development of fever with leuconeutropenia oriented towards the diagnosis of a non-Hodgkinian malignant lymphoma. Medullary biopsy and immunohistochimic study confirmed a T/NK cells lymphoma, with medullary invasion. The child died from acute respiratory distress syndrome. The authors insist on the rarity of this bipolar location, in particular in children, emphasize the difficulty of the diagnosis and review the literature.
Asunto(s)
Células Asesinas Naturales , Linfoma de Células T Periférico/patología , Cavidad Nasal/patología , Neoplasias Nasales/patología , Neoplasias Pancreáticas/patología , Dolor Abdominal/etiología , Biopsia , Niño , Resultado Fatal , Humanos , Inmunohistoquímica , Masculino , Invasividad Neoplásica , Úlcera/etiologíaRESUMEN
We report a retrospective study of nine neonatal observations of antenatal isolated pyelectasis during a period of two years. Pyelectasis associated with other congenital abnormalities and in utero died foetus were excluded. Finding interesting sex, gestational age at diagnosis, echographic aspect, antenatal management and postnatal follow-up were assigned. Foetal kidneys was noted in two cases and an oligoamnios was noted in two other cases. No foetal urinary intervention was assessed. Postnatal exploration revealed a transitional pyelectasis in four cases, an ureteropelvic junction obstruction in four cases and a congenital megauretere in one case. Postnatal renal function was decreased in two cases. Postnatal surgery was assessed in two cases and a spontaneous regression under a sequential treatment occurred in the other three cases. Isolated foetal pyelectasis can have a pathologic significance.This examination permits, in plus, to evaluate the renal prognosis. Antenatal therapeutic implications of interruption of pregnancy or urinary intervention are still not clear and those after birth depend essentially on renal function determined by scintigraphy.
Asunto(s)
Enfermedades Renales/diagnóstico , Pelvis Renal/diagnóstico por imagen , Diagnóstico Prenatal , Dilatación Patológica/diagnóstico , Dilatación Patológica/cirugía , Femenino , Humanos , Recién Nacido , Enfermedades Renales/cirugía , Pelvis Renal/cirugía , Masculino , Embarazo , Estudios Retrospectivos , UltrasonografíaRESUMEN
Child is an ideal patient for day care surgery. So more than 60% of paediatric surgery could benefit by ambulatory surgery. Preoperative visit may select patients for ambulatory surgery. Medical exam may lead to choose pre operative screening. The ideal ambulatory anesthesia is locoregional technic or inhalatory one. Tracheal intubation don't contre indicate ambulatory surgery. Recovery of mental abilities following general anesthesia has not the same significance as in adult. Many studies confirm the safety of paediatric outpatients anesthesia.