[Nuclear magnetic resonance of anorectal malformations and persistent postoperative fecal incontinence]. / Valoración por resonancia nuclear magnética de las malformaciones anorrectales y de la incontinencia fecal postoperatoria persistente.

We review our experience with Magnetic Resonance Imaging (MRI) in the evaluation of 6 patients showing anorectal malformation, and 4 more with persistent postoperative fecal incontinence. Preoperative sagittal, axial and coronal planes were studied with special consideration to the pelvic and vertebral structures. The excellent resolution of MRI allowed accurate identification of the pelvic musculature in all patients, including those with bizarre sacral abnormalities. MRI revealed structural anomalies not detected previously, such as teathering cord, intraspinal lipoma, presacral mass and renal malformation. In our institution, MRI has replaced the CT scan in the study of patients suffering of persistent fecal incontinence. In non operated on cases of anorectal malformations, MRI determines with extraordinary accuracy the location of the rectal atretic pouch, the actual pelvic muscular quality, and the detection of previously unsuspected associated anomalies.

[Pyknodysostosis: extreme cause of sleep apnea]. / Picnodisostosis: causa extrema de apnea del sueño.

We describe a twentieth month old infant who had a pycnodysostosis syndrome. This malformation shows a loss of the normal mandible angle with generalized bone hyperdensification. The first produced and airway obstruction, with special relevance during sleeping hours. A polysomnography revealed an obstructive sleep apnea syndrome. The respiratory picture deteriorated with worsening of the airway obstruction, hypoxemia and finally pulmonary hypertension and cor pulmonale. A tracheostomy was performed, with resolution of the sleep apnea and pulmonary hypertension. The etiology, pathophysiologic consequences and surgical treatment of obstructive sleep apnea syndrome is reviewed.

[A case of total intestinal aganglionosis. 2 years of survival]. / Un caso de aganglionismo intestinal total. Dos años de supervivencia.

A two-year-old male is presented. Small bowel aganglionosis was proved to extend to 3 cm below ligament of Treitz. Gastrostomy and ileostomy was done. He received his caloric intake by cyclic home parenteral nutrition trough implantable venous system. At eighteen months of age, intestinal transplantation was refused and reoperation was done. The child underwent Ziegler's miotomymiectomy on 60 cm of aganglionic jejunum. Now at twenty seven months of age he receives cyclic parenteral nutrition, and enteral feedings are being increased slowly. The weight/height was at 91 per 100 standard, intestinal motility appears much better on contrast study and he is developmentally a normal child.

[Sepsis in children using venous implants with reservoir]. / Sepsis en niños portadores de implantes venosos con reservorio.

Sepsis is a serious situation in patients with chronic central venous access. The infections complications presented with totally implanted reservoirs are studied in this paper. Twenty five venous systems for chemotherapy, total parenteral nutrition, specific drugs and blood drawing, were placed in 24 patients ranging in age from three months to sixteen years. Catheter related sepsis was defined as simultaneous positive blood cultures drawn through the reservoir and peripheral vein. Five children (20 per 100) had septicemia with candida albicans (3), staphylococcus aureus (1) and staphylococcus epidermidis (1). Four systems was removed after unsuccessful antimicrobial therapy. Three symptomatic patients were studied by echocardiography, one of them showed thrombosis in superior vena cava and right atrium, this child underwent an open atriotomy. These data suggest that removal of implantable venous system is required for successful treatment in patients with catheter related sepsis.

[Surgical treatment of differentiated carcinoma of the thyroid. Retrospective study]. / Tratamiento quirúrgico del carcinoma diferenciado de tiroides. Estudio retrospectivo.

This is a review of the surgical treatment in six patients with papillary carcinoma of the thyroid. The mean age at the time of diagnosis was eight years (range four-twelve years). Clinical presentation was: all patients had a palpable thyroid tumor, palpable lymph nodes in four and one with lung metastases. The initial surgical treatment was lobectomy and modified regional lymph nodes resection. Four patients underwent a second operation for multifocal disease, palpable lymph node or thyroid tumor. The interval from initial surgical management to the detection of recurrence ranging from one month to four years. In three patients the remaining thyroid gland was removed. Radioiodine, external irradiation and chemotherapy were given during the postoperative. The median follow-up was eight years (range one-fifteen years). All patients are alive with thyroid hormone therapy by hypothyroidism. There were no hypoparathyroidism or injuries to the recurrent laryngeal nerve. The results of this study suggest that the surgical approach in papillary carcinoma of the thyroid is total thyroidectomy because reoperation were performed in four patients.

[The efficacy of the Childs-Phillips mesenteric plication in intestinal obstruction]. / Eficacia de la plicatura mesentérica de Childs-Phillips en la obstrucción intestinal.

From 1975 to 1988 we studied and valued fourteen pediatric patients, treated in the Department of Pediatric Surgery at Children's Hospital La Paz, with the Childs-Phillips procedure by postoperative recurrent bowel obstruction. Ten newborn infants had the following diagnoses: intestinal atresia, 4; Bochdaleck hernia, 3; Hirschsprung disease, 2; intestinal rotation anomalies, 1. Four patients out of neonatal period had: hiatal hernia, 1; intussusception, 1; appendicitis, 2. Six patients had more than one episode of bowel obstruction. The follow-up was 6.5 years (range four months to 13 years), and no recurrent bowel obstruction occurred.

Papillary cystic tumor of the pancreas.

A 10-year-old girl had fever, abdominal pain and a palpable mass at the left hypochondrium. She was found to have a pancreatic cystic tumour that was biopsied and removed by coeliotomy. The pathologic diagnosis was papillary cystic tumour of the pancreas with chronic pancreatitis. No pathological lesion was found elsewhere. 2 years later she is asymptomatic and without recurrence.

[Effect of prostaglandin (PGE2) in biliary atresia]. / Efecto de la prostaglandina (PGE2) en la atresia biliar.

We studied the choleretic effect of Prostaglandin E2 (PGE2) in patients with biliary atresia. In the last four years PGE2 was used in ten cases with extrahepatic biliary atresia. All of them were treated by Kasai technique and temporary exteriorization of the bilioenteric conduit, permitting analyses of the postoperative bile flow. Mean age al operation was 50 days (range 30-70 days). The histology studies of liver and porta hepatis showed severe hepatic fibrosis in three patients, moderate in seven, bile ducts less than 100 mu in five and more than 100 mu in another five. The mean postoperative bile flow was 15 c.c./day (range 0-30 c.c.). We used PGE2 during postoperative period because the bile excretion was partial or total stopped (PGE2 0.125 mgr./kgr./day). In six cases the bile flow increase until 90 c.c./day (range 40-160 c.c./day) and four patients no response. Eight patients are alive, four without jaundice with normal hepatic function (serum bilirubin 1.04 mgr./dl) and four are waiting for liver transplantation. Two have died 7 and 30 months after Kasai procedure. The long-term follow-up was between 6 to 53 months. In conclusion the PGE2 has choleretic action in biliary atresia and could be a coadjuvant treatment to improve the bile flow.

Surgical Management and follow-up of vascular rings.

Thirty patients with aortic arch anomalies resulting in tracheo-oesophageal compression were treated during the period 1966 through 1987. These anomalies are important causes of upper respiratory and oesophageal obstruction in babies and small children. Although symptoms started within the first months of life in most cases, only 15 underwent surgery before six months and a delay of more than one year occurred in 5. Diagnosis was established by chest roentgenogram, barium oesophagogram and angiography. Thirteen (43%) patients had a double aortic arch, 9 (30%) cases had aberrant right subclavian artery and 6 (20%) patients had right aortic arch with ductus or ligamentum arteriosum. One (3.5%) patient had pulmonary artery sling and 1 (3.5%) case had right aortic arch and ductus arteriosum and aberrant right subclavian artery. Associated malformations were seen in 8 (27%) cases, (4 ventricular septal defects, 1 atrial septal defect, 1 coarctation of the aorta, 1 hypoplasia of left pulmonary artery, 1 left diaphragmatic eventration). Basic surgical procedure includes exposure through a left thoracotomy, complete identification of the anomaly and division of the constricting ring. 85% of the patients are asymptomatic and minimal to moderate stridor persists among the remainder. Severe tracheomalacia was responsible for the only two deaths in the series.

[Incidence and management of surgical pathology in children with cystic fibrosis of the pancreas]. / Incidencia y manejo de la patología quirúrgica en niños con fibrosis quística de páncreas.

This report reviews 110 children of ages ranging from 2 months to 17 years, with a diagnosis of cystic fibrosis, and analyzes the surgical treatments they have undergone. In our series, meconium ileus shows an higher occurrence (20%) than the reported by the literature. No direct relationship could be established between the existence of meconium ileus and a more severe affectation of cystic fibrosis. Rectal prolapse occurred in 10% of the patients, and only 4% underwent surgical treatment. Three children with appendicitis developed a periappendiceal abscess due to delayed diagnosis and 2 other were operated upon for meconium ileus equivalent. From 7 patients with gastroesophageal reflux, 5 were treated non-operatively and 2 required corrective surgery. Fifty five patients out of the 110 (45%) underwent different surgical procedures.

[Digestive leiomyomas in childhood]. / Leiomiomas digestivos en la infancia.

Leiomyoma is a very rare tumor, originating in smooth muscle. During the last 18 year authors treated four of them from 159 digestive tumors. This were located in jejunoileal bowel with occlusion in two cases and bleeding problems in the other two.

[Colonic atresia]. / Atresia cólica.

Nine cases of colonic atresia, treated in the Departament of Pediatric Surgery of "La Paz" Pediatric Hospital, are reported. They account for 11% of all our intestinal atresias. We review the different etiologic and pathologic theories and classificate our group according to the LOUW and HAYS method. In two cases their digestive anomalies and in one renal anomalies were found to be associated mortality was 11%.

[Digestive system duplications]. / Duplicaciones digestivas.

Fifteen digestive duplications, corresponding to fourteen patients, have been surgically treated in "La Paz" Pediatric Hospital between 1966 and 1984. The most common localization (40%) was the large bowel. The first choice treatment was the complete excision of lesions, with led to cure of all patients. Embryologic theories and clinical forms are also reviewed.