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ABSTRACT: Multiple endocrine neoplasia (MEN) 2a consists on medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. The identification of the RET proto-oncogene in 1993 has changed the prognosis of the disease. We have retrospectively studied the patients diagnosed of MEN 2a in our centre for the last 7 years in order to establish the most adequate age to undergo surgery. We present ten patients diagnosed with MEN 2a, whose ages ranged from 1.5 to 11 years old. Mean age at time of operation: 6,4 years An ultrasound study, calcitonin determinations and cathecholamines and urinary metanephrine levels were obtained before surgery. The surgical treatment is based on total total thyroidectomy, in selected cases lymph node resection in the central zone lf the neck. The most frequent RET mutation is the one affecting codon 634 (exon 10), which was found in children. Both of them had an alteration in codon 611 (exon11). No complications appeared after surgery and hospital discharge took place in the 2nd-4th day after surgery. Pathological findings were medullary thyroid microcarcinoma (MTMC) in 3 out of 10 patients, calcitonin preoperative tests were high in one of them. No tumoral cells were found in the lymph nodes. During the follow up period, 9 out of 10 from the operated patients, maintained normal calcitonin, CEA, PTH, calcium, cathecholamines and urinary metanephrine levels. Since there are 3 cases of MTC in patients between 3 and 6 years old, and diagnostic test data are not conclusive, we thoroughly recommend prophyilactic thyroidectomy at early ages, from 3 to 4 years old.
Cirugía pediátrica: organo oficial de la Sociedad Española de Cirugía Pediátrica 10/2009; 22(4):189-92.
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ABSTRACT: The authors report two patients with extrahepatic portal hypertension and repeated massive bleeding from esophageal varices who underwent urgent distal splenorenal shunting (DSRS) after having proved refractory to medical treatment, endoscopic sclerotherapy, and ligation of esophageal varices. Their ages and weights were 18 months/10 kg, and 11 months/6.4 kg, respectively. The splenic veins were 6 and 4 mm in diameter. During follow-up of 4 and 3 years, respectively, the shunts have remained patent in both patients as demonstrated by Doppler Ultrasound. Gastrointestinal hemorrhage has not recurred, splenomegaly regressed, and platelet and white blood cell counts increased gradually. Neither patient developed a significant encephalopathy, and liver function tests showed no significant changes throughout the observed period. The authors considered the shunting feasible despite the relatively narrow splenic veins. In both patients the anatomic position of the splenic vein--more caudal to the posterior wall of the pancreas--facilitated its isolation. In the smaller infant, the inferior mesenteric vein was sutured to gain a satisfactory segment of splenic vein. The successful use of a DSRS to control actively bleeding varices in a child weighing 6.4 kg has not been previously reported.
Pediatric Surgery International 12/2001; 17(8):646-8. · 1.25 Impact Factor
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ABSTRACT: Within the last ten years and by the introduction of some new instruments, laparoscopy is a safe and effective method that has been further extended in children. We report our experience in the treatment of laparoscopy on a seven day old newborn affected by malrotation with an intermittent duodenal obstruction. The treatment consisted of a duodenal liberation after the section was easily performed in congenital Ladd's bands. The spiral twists of the small intestine found in the upper gastrointestinal series, disappeared in a new study done after surgery. We consider this operation as a new indication for an operative laparoscopy. It allows a good visualization of this congenital abnormality, and it is easy to perform with a significantly reduced operative trauma.
Cirugía pediátrica: organo oficial de la Sociedad Española de Cirugía Pediátrica 02/1999; 12(1):41-3.
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ABSTRACT: We report two cases of primary psoas abscess in two patients of 15 months and 4 years of age. As the first case showed the natural history of this process the second one was large enough to produce a huge ureterohydronephrosis and to drain through the rectal wall to the rectum spontaneously, although this natural way did not achieve complete drainage. Both were treated by open drainage and systemic antibiotics with good response. They were discharged at the 7th and 12th postoperative day. 5 months later no complication has come up. Etiological, clinical and therapeutic aspects of this unusual pathology are reviewed.
European Journal of Pediatric Surgery 05/1998; 8(2):111-3. · 0.81 Impact Factor
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ABSTRACT: A retrospective and transversal study of our patients with portal cavernomatosis is developed, with the aim of comparing the evolution and the present status of the patients that have undergone different therapeutical approaches. Between 1975 and 1995, 15 patients with portal cavernomatosis have been treated. Personal history, signs and symptoms at the moment of diagnosis, and treatment were analyzed. Now, the controls have been made by physical examination and ECO-Doppler of the espleno-portal system. In 6 cases the absence of bleeding made surgery unnecessary. In the 9 patients with digestive haemorrhage, it could be controlled pharmacologically, with a later surgical approach. Now, all non-operated and 6 operated patients show splenomegaly, without bleeding. The ECO-Doppler shows colateral flow, the splenomegaly in the non-operated and the surgical shunts and the hepatofugal circulation in the operated patients. Looking at our results, we believe that prophylactic surgery is not indicated. In those cases of digestive bleeding, surgery after the complete resolution of the acute disease shows good results at a short and long term.
Cirugía pediátrica: organo oficial de la Sociedad Española de Cirugía Pediátrica 08/1997; 10(3):90-2.
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ABSTRACT: Injection of sclerosing solutions into the rectal submucosa or into the perirectal space is one of the many therapies designed to correct rectal prolapse. Since 1994, four patients affected with this condition have been treated with fibrin adhesive percutaneous injection. Up to now, this product had not been used to this aim. Age range was from 1 month to 8 years. The medium duration of prolapse prior to therapy was 19 months and three of them had an associated pathology (myelomeningocele, cystic fibrosis and severe psychomotor retardation). There were no post-injection complications. In one patient with an irreducible prolapse an anus encirclement with a rectal tube was associated. Except for this patient, the whole process take less than 24 hours. With a medium follow up of 11 months this therapy has resulted effective in three of our four patients. It has failed in a girl with severe psychomotor retardation.
Cirugía pediátrica: organo oficial de la Sociedad Española de Cirugía Pediátrica 02/1997; 10(1):21-4.
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ABSTRACT: We report a case of biliary atresia promptly detected by its association with a choledochal cyst, prenatally diagnosed at 20 weeks' gestation. The baby was operated at 14 days of life. A choledochal cyst was found and fibrosis of the extrahepatic bile ducts was also noted. So, excision of the choledochal cyst and a Roux-en-Y porto-jejunostomy was done. Nine months later, despite an appropriate biliary drainage, echographic and histological changes compatible with liver cirrhosis have been detected. Including this one, six cases have been reported.
Cirugía pediátrica: organo oficial de la Sociedad Española de Cirugía Pediátrica 11/1996; 9(4):135-7.
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ABSTRACT: Recurrent tracheoesophageal fistula (RTF) after repair of esophageal atresia with tracheoesophageal fistula is a serious complication, with the need for a second operation. The mortality rate is high. The authors report on the eradication of RTF using fibrin glue.
Journal of Pediatric Surgery 01/1995; 29(12):1567-9. · 1.45 Impact Factor
