[Use of mitomycin C in esophageal stenosis]. / Empleo de la mitomicina C en las estenosis esofágicas.

INTRODUCTION: Esophageal stenosis, due to its important implications on the patients' quality of life, poses a serious problem, often difficult to resolve, that requires therapeutical solutions that could irreversibly affect vital conditions and quality of life of those affected. For this reason our group has tried over the last thirty years to employ conservative solutions in the damaged aesophagus, showing that a complete cure is possible in most cases although the way to achieve this can be dramatically long. MATERIAL AND METHODS: We present our experience employing topical Mitomicyn C over the last year and a half in eight patients, six of them affected with caustic stenosis and two with secuelae of esophageal atresia, highlighting the three cases that received at least five applications of the product. We describe the method employed that consists in the spraying of the correct dose using a flexible fibroscope on the dilated area, loading the dose in the container of the cleaning water of the fibroscope, employing a system that permits only to apply the exact quantity necessary of the product. The application time is five minutes and the dose is 0.4 mg/ml applying 5ml or 10 ml according to the patient's weight ( under 12 kg = 2.5 ml and over 25 kg=5 ml). Upon completion of the application, a washing of the product is performed using the same fibroscope. RESULTS: From the eight cases in which it was applied, we analysed the three that received at least five doses. These were two boys of seven and eight years and a 25 year-old woman. In the children the dilatations interval has passed from 4-5 weeks to 8-11 respectively. The woman has passed from 12 to15 weeks. The children have been in the dilatation program more than 3 years and the woman more than 18. CONCLUSIONS: Although the time period that we have been applying Mitomicyn C is still short, the symptom-free interval after dilatations is proving to be highly promising. This fact is highlighted in those patients included in the dilatation program recently. We have not seen any adverse side effect from the application of the product.

[Hirschsprung-neurointestinal dyspasia: diferential diagnosis and reliability of diagnostic procedures]. / Diagnóstico diferencial Hirschsprung-neurodisplasia intestinal. Fiabilidad de las pruebas diagnósticas.

The suspicion of Hirschsprung's disease (HD) or Neurointestinal Dysplasia (NID) arises with the appearance of a common symtomatology : delay in meconium evacuation, abdominal distention, vomiting, intestinal occlusion and ultimately, chronic constipation. The need to perform a correct differential diagnosis between both pathologies is essential, given that the treatment of HD is surgical while NID is expectant. The objective of this paper is to define the necessity of using or not all the diagnostic tests simultaneously to obtain a precise diagnosis. The authors performed a complete clinical review of a series of 42 HD and 18 NID analysing the results of diagnostic procedures (Radiology / transition zone, anorrectal manometry / absence of anal inhibitory reflex, and suction biopsy / AcHE study). The authors concluded that is necessary to perform all the three diagnostic procedures simultaneously in all patients with symtomatology given that not one test has the capacity to provide a diagnosis alone. Anorectal manometry has proven to be the most reliable test to diagnose HD. The authors agree with the guidelines published by Meier Ruge in 2004 to diagnose NID.

[Biliary atresias operated with favourable results: predictable outcome]. / Atresias de vías biliares intervenidas con buen resultado. Evolución previsible.

Since 1975, our experience in the treatment of biliary atresia with Kasai's technique has improved little by little, achieving 65% favourable outcome in the last five years. We define "good results" as the complete restoration of biliary flow and normalization of bilirrubin levels. The long-term evolution of these good results can be diverse. The objective of the present work is to analyze the outcome of patients in our series in whom a favourable initial response was achieved, as well as evaluating their present situation and future perspectives. The authors present a total of 17 patients operated by Kasai's technique since 1985, that constitutes the group with good results in our series. The controls were based on general analysis, liver function and periodic ultrasound explorations. All received a standardized medical treatment consisting of vitamin supplements (A, D3, E, K) minerals (zinc, calcium, phosphate, iron) ursodexoxicolic acid, luminal,as well as close control of calorie intake. In two patients the levels of bilirrubine were progressively increased with time, stabilizing at between 5/6 mgs/100 ml, with progressive hepatic hardening, appearance of splenomegalia, indirect signs of portal hypertension and a slight deterioration of hepatic function. One received a transplant at age 12 with Quick levels below 50%. The other, aged 16, continues with an acceptable hepatic function and good quality of life under recommendation of transplant. Eleven patients with ages ranging from fourteen months to seventeen years presented slight and firm hepatomegalia, moderate portal hypertension, GOT 71 +/- 8 mg/100 ml, GPT 97 +/- 11 mg/100 ml and normal bilirrubine levels. From this group, 3 patients, all under five years of age, experienced bleeding from esophageal varices which were controlled by sclerosis and medical treatment (propanolol and isosorbide dinitrate). Recently, one three year-old patient developed a hepatocarcinoma of rapid, mortal evolution. Since then, the determination of alfa-feto protein in follow-up controls has been introduced. Four other patients of 5, 6, 14, 16, years of age are completely assymptomatic with an excellent clinical evolution. In our experience,the patients that overcome the third year after surgery without serious complications seem destined to reach puberty with a good quality of life. However, some cases show signs of hepatic fibrosis and portal hypertension, 77% in our series. Only 23% of patients with a favorable initial evolution appear to present a complete normalization of their hepatic lesion in the long term.

Minimum nephrostomy.

A new type of nephrostomy is described with the following main characteristics: it uses a small multiperforated Silastic tube; its crosswise location allows perfect drainage of all of the calyces and the pelvis; it does not become obstructed nor move from its position; and it causes minimum traumatization of the renal parenchyma. It has a double purpose: its serves as a drain and safety measures as well as being used for the treatment of renal infection.

Extracorporeal renal surgery. Work bench surgery.

In cases in which renal repair through conventional in situ surgery is not possible, we have proceeded to remove the organ outside of the human body and placed in on a work bench where exsitu repair is aided by microsurgery, x-ray films, and image amplifiers. In most cases the damaged kidney has recovered its function and a grave problem has been solved. Extracorporeal surgery means a new tactical solution to extreme situations.

Twenty-four hour exophageal pH monitoring in gastroesophageal reflux.

Twenty-four hour pH esophageal monitoring has been studied in 123 children with gastroesophageal reflux (GER) with ages ranging between 6 mo to 6 yr. The technique is described and the scoring system modified to evaluate gastroesophageal reflux in these patients. For the first time the normal patterns are presented in pediatrics (n = 20). The results obtained in 103 patients are presented, divided into pre- and post-nonoperative and surgically treated groups. The test is more objective, has greater precision, sensitivity, and reliability than contrast studies, endoscopy, acid perfusion or acid reflux tests. The study seems to indicate that esophagitis depends more on the duration of the reflux than on the number, for which the concept of esophageal acid clearance is of paramount importance. The prone position generally lessens the number of refluxes but in this position the esophageal acid clearance is altered. With a greater knowledge and more experience in this technique, we would be able to identify children at risk to develop severe complications, control them and aid in the selection of those patients requiring surgical intervention.

[Current controversies on the treatment of urinary lithiasis]. / Controversias actuales en el tratamiento de la litiasis urinaria.

With the discovery during the 1960s of an access to the renal sinus and intra-operative radiological monitoring, the lithiasis surgery reached its summit of technical perfection. A comparative study on the various therapeutical options is presented: NLP, URN, ESWL and open surgery, as the single or combined treatment in complex renal lithiasis. Also, intrasinusal and transparenquimatous accesses to the intrarenal tract are compared, evaluating the morbidity, surgical time and economical interpretation of both techniques. Establishment of each treatments' indications and contraindications as well as likely short- and long-term adverse effects of the new technologies.

[Testicular and epididymal malacoplakia. Presentation of a case and review of the literature]. / Malacoplaquia testicular y epididimaria. Aportación de un caso y revisión de la literatura.

A case of testicular malacoplakia and epididymis in a 62 year old man is presented. A literature review is made (only 6 cases describing epididymal malacoplakia have been reported), commenting on the clinical, diagnostic, anatomopathological, pathogenic and therapeutic aspects of malacoplakia.

[Nineteen years experience with posterior sagittal anorectoplasty as a treatment of anorectal malformation]. / Diecinueve años de anorrectoplastia sagital posterior como tratamiento de la malformación anorrectal.

UNLABELLED: Posterior sagittal anorectoplasty (PSARP) is considered today the best surgical technique for the treatment of the anorectal malformations. With the aim of evaluating the results of the PSARP in our patients, the charts of 39 children (27 male, 12 female) were reviewed. Mean age was 11 years (3 to 20 years) and the mean follow-up period from the closure of the colostomy was 9.4 years (1 to 18 years). Patients with any alteration of the extrinsic innervation or those with rare malformations were excluded of the study. According to the Wingspread classification, 14 patients had a high defect, 13 had an intermediate type and 12 had a low one. One patient had a PSARP in the newborn period whereas the rest of them had a descending loop colostomy. Definitive repair consisted in PSARP in low and intermediate forms and in six of the high type patients. The rest (8) of the high type patients had a Rehbein abdominoperineal pull-through (DA) plus a PSARP. Evaluation for the surgical results was made by means of an anorectal manometry (existence or not of anal inhibitory reflex [RIA], the symmetry and pressure of the canal anal, and the existence of extrinsic innervation) and a clinical questionnaire. Patients or parents were asked for fecal continence, age at which the continence was achieved and the existence of severe constipation. RESULTS: Low defects: Eleven patients were continent between 3 and 5 years old. Seven patients had RIA. Intermediate defects: Seven patients were continent. Five (all with RIA) achieved continence between 2 and 8 years old; the other 2 were continent at the age of 10. The six incontinent patients had absence of RIA and/or low-pressure anal canal. High defects: Five patients were continent, between 11 and 16 years old. All had an DA plus PSARP. None of them had RIA. All the incontinent patients had an anomalous anal canal. CONCLUSIONS: The lowest the type of an anorectal malformation, the better the prognoses. In these patients, there is a relationship between the achievement of continence, the presence of RIA and the symmetry and high pressures at the anal canal. Among the patients with high defects, the results are better when they had an abdominoperineal pull-through plus PSARP.

[Total parenteral nutrition. A 1-year experience with 96 patients]. / Nutrición parenteral total. Experiencia en un año con 96 pacientes.

In 1987, ninety-six surgical patients, of whom 44 were premature or newborn babies and 52 were infants or older children, received total parenteral nutrition (T.P.N.) at our hospital. A peripheral venous line was utilized on forty patients in the first group and only on 9 in the second group. The excellent results yielded by T.P.N. are self-evident from the low mortality showed by these patients. Thirty-three out of the 44 neonates and premature infants with serious surgical problems survived, being noteworthy the occurrence of just 3 deaths within a series of 13 necrotizing enterocolitis. In the series of 52 infants and older children, composed of 13 digestive patients (with 5 liver transplants), 6 tumoral, 9 neurologic, 4 renal (2 transplantations), 18 cardiac, 1 thoracic and 1 burnt, only 9 patients died. The average duration of T.P.N. was 10 days in the group of premature and newborn infants and 6 days for the nursing infants and older children, save for the 8 patients in whom the treatment had to be prolonged for a few months. We conclude that T.P.N., when applied in time and of short duration should entail no risk at all. In neonate and premature infant a peripheral vein shall be the route of choice. In the nursing infant and older child a central venous line is preferable, if possible the superior vena cava or its major tributary veins.

[Laparoscopy for the undescended testis]. / La laparoscopia en el testículo no palpable.

The authors show their experience in the use of the laparoscopic approach in 18 cases of unilateral exploratory anorchia. They highlight the advantages that this method offers in efficiency, speed, security and minimal aggression compared with conventional exploration of the inguinal channel in the search of the undetectable testis. In the 18 cases studied through the insertion of 5 mm lense in the umbilical area, it was established that a testicular hypoplasia was present in 8 cases. In another 5 cases testicular agenesia was diagnosed, and in the remaining 5, intraabdominal testis were found. In four of these testicles, a laparoscopic approach helped in their correct placement and also in the removal of the fifth in a patient with recurrent leukemia.

[Pulmonary biopsy by thoracoscopy: techniques and results in 9 patients]. / Biopsia pulmonar mediante toracoscopia: técnicas y resultados en 9 pacientes.

Video-assisted endoscopic techniques have decreased the surgical aggression in abdominal and thoracic surgery. In our country, pediatric laparoscopy has been developing slowly, but this is not the case for thoracoscopy. The aim of this paper is to present the techniques and results of thoracoscopy pulmonary biopsy in our first patients. Pulmonary biopsies with this approach had been done in 9 patients (5 males, 3 females). Their age ranged between 30 months and 16 years. In all cases this was the last resort for the diagnosis of pulmonary condensation of unknown etiology. The biopsies were done with the pretied knot in 5 cases, stappler in 1 case and with biopsy forceps in 2 cases. Thoracotomy was necessary in one patient, due to intraoperative haemorrhage. Enough tissue for bacterial and pathological diagnosis was obtained. There was not mortality nor important morbidity related with the technique. Postoperative recovery is better when compared with conventional thoracotomy. Thoracoscopy is an adequate approach to perform pulmonary biopsies in children. The advantages if we compare with open thoracotomy are: 1. The possibility to choose the are to perform a minimally invasive biopsy. 2. To take samples of different pulmonary lobes. 3. Less postoperative pain and shorter hospital stay (36-48 hours).

[The usefulness of manometry in the determination of the morphology of the anal canal]. / Utilidad de la manometría en la determinación de la morfología del canal anal.

The appearance of the new lecture systems for the manometry studies by computer, like the Polygram by Synectics (vector volume), offer the possibility to see the circumferential pressure forces, that even in rest conditions as in voluntary contraction, they keep coaptation of the anal canal, and this will act as a continent closure system in the most distal part of the G.I. tract. The study is with the normal parameters obtained in 14 individuals, considered as normal, getting the mean +/- sd pressure of the anal canal convey in mm Hg, from de anal canal profile in rest as in voluntary contraction, and the maximum variability that could exist between the six profile waves, that are obtained in the same individual to develop an image of the anal canal. This valves will allow the author's to get to the bottom of fecal incontinence derivative from anorectal malformations, defining the pressure valves of muscular hypoplasia or surgical outcomes of the malformations.

[Acute gastric volvulus in a patient with asplenic syndrome]. / Vólvulo gástrico agudo en un paciente con síndrome de asplenia.

A 18 month-old boy with asplenic syndrome was admitted to our hospital with a twelve hours history of mucous vomiting, abdominal pain and a round epigastric mass. It was diagnosed as having a gastric volvulus and an emergency laparotomy was performed. The stomach was situated at the right side and a mesenterioaxial volvulus was found, with cyanosis of the antrum. The gastrophrenic and gastrosplenic ligaments were absent. The clinical and radiological characteristics of asplenic syndrome are reviewed. The pediatric surgeons must be aware of the digestive malformations of the asplenic syndrome, because some of that like malfixation of the stomach could be the cause of an acute abdomen.

[Nitric oxide and Hirschsprung's disease: a causal relation biochemically, immunohistochemically and functionally demonstrated]. / Oxido nítrico y enfermedad de Hirschsprung: relación causal demostrada bioquímica, inmunohistoquímica y funcionalmente.

UNLABELLED: Hirschsprung's disease may be due to impaired nonadrenergic-noncholinergic inhibitory input in the aganglionic segment of the colon. It has been suggested that nitric oxide (NO) might be the lacking neurotransmitter. Thus, our specific aims were to determine in ganglionic and aganglionic segments: 1. The activity of the NO synthetase (NO-S); 2. The location of this enzyme; and 3. The "in vitro" basal motor activity of the muscle strips and their responses to an NO donor and to an NO antagonist. METHODS: NO synthetase activity was quantified in samples of tissue from both aganglionic and ganglionic segments obtained during surgery in 6 patients with Hirschsprung's disease by the transformation of 14C-L-arginine into 14C-L-citrulline in tissue homogenates. Immunohistochemical staining of the tissues was performed using a polyclonal antibody raised against a peptide sequence of rat brain NO synthetase. Furthermore, in 2 patients we measured "in vitro" the tonic response of muscle strips to an exogenous NO donor (sodium nitroprusside) and to an NO antagonist (L-NAME). RESULTS: NOS activity was undetectable in every aganglionic segment whereas it was present in all ganglionic segments (0.49 +/- 0.09 pmol citrulina/mg.min; mean +/- SE). Immunohistochemically, NO-S was absent in the myenteric plexus of aganglionic segments and it was present in ganglionic segments. "In vitro" basal motor activity of ganglionic segments was normal, with presence of low-frequency contractions (LFC) and summation contraction (SC); in aganglionic segments SC were absent. Sodium nitroprusside induced a marked relaxation (90% from basal) in muscle strips, both aganglionic and ganglionic, precontracted with bethanocol.(ABSTRACT TRUNCATED AT 250 WORDS)

[Considerations regarding the treatment of non-aganglionic congenital intestinal neuropathies]. / Consideraciones al tratamiento de las neuropatías intestinales congénitas no agangliónicas.

Hyperganglionosis or neuronal intestinal dysplasias (NID) and hypoganglionosis (HO) are intestinal diseases of difficult diagnosis and treatment and diverse evolution, despite identical histologic findings. The aim of this study was to discuss the therapeutic problems derived from the patients differing clinical course. Retrospective review of 14 patients with regard to diagnosis, manometry and histology (hematoxylin-eosin, acetylcholinesterase activity, immunohistochemistry and Smith's silver stain) was done. Six patients presented intestinal occlusion or sub-occlusion from the first months of life with impeded oral feeding. Ileostomy was performed in 5 and total colectomy with anastomosis in 1. All patients required parenteral nutrition; cisapride was added in 2. Three died from sepsis (3 NID). Of the 3 survivors, 2 have ileostomies (2 NID) and the other ileo-rectal anastomosis (NID). Of the remaining patients, two presented aganglionism and the finding of proximal hyperganglionism occurred post-surgery. Surgery was repeated in one patient. The remaining 6 (1 HO, 5 NID) were diagnosed between 3 and 10 years of age because of constipation. Four are under treatment with cisapride and 2 required partial colic resection. No relationship can be established between histologic findings and clinical manifestations. In chronic clinical courses, treatment with cisapride and cleaning enemas should be tried first. Acute clinical pictures (occlusion-sub occlusion) should be treated by decompressive ileostomy. Partial colic resection may lead to new intestinal failure.

[Intestinal dysmotility-pseudo-obstruction]. / Dismotilidad intestinal-pseudoobstrucción.

Chronic intestinal pseudo-obstruction is defined as a syndrome related to any process which affects intestinal regulation and propulsion. Its origin may be muscular, neurogenic or hormonal, excluding Hirschsprung's disease or any known mechanical obstruction. Between 1989 and 1991, 11 patients with intestinal pseudo-obstruction were studied at our centre, and included nine hyperganglionisms B, and two hypoganglionisms) and two visceral myopathies (Berdon's syndrome). Diagnosis was established in all cases by histologic study. The techniques of haematoxylin-eosin, acetylcholinesterase, enolase, protein S-100 and Smith were used in neuropathies and haematoxylin-eosin and Masson's trichromic in myopathies. Intestinal motility was studied by ano-rectal and gastrointestinal manometry in seven and three cases respectively. Gastrointestinal manometry and radiology permitted differentiation of localized and diffuse forms of involvement. Medical treatment consisted of total parenteral nutrition when oral feeding was impossible, and in five cases, cisapride was given, with good results in four. Derivative surgery was performed in cases of diffuse involvement, and resection with anastomosis in those of localized forms. We conclude that: 1. Diagnosis is established according to histologic criteria. 2. Complementary examinations should be directed towards distinguishing localized from diffuse involvement. 3. Cisapride was effective in the treatment of neuropathies in the majority of cases.

[Intraesophageal stent in the prevention of stenosis caused by caustic ingestion]. / Tutor intraesofágico para la prevención de estenosis en las ingestas de cáusticos.

Esophageal stenosis is a frequent consequence of lye ingestion, and their treatment is of longstanding and complex matter. The search of new solutions for this problem is challenging and always attractive if its associated with less aggressiveness to the patient. The use of intraesophageal stents is not new in the treatment of stenosis, but it was anecdotal, until histocompatible material came out in the market. The stent is designed in the OR, having the same length as the stenosis, previously observed by endoscopy, its made out of a silicone tube 30 or 36 French, mounted over an ng tube 16, all this is fixed in the ends of the silicone tube, that way won't slip over the ng tube. It is placed with a laryngoscope assistant, the proximal end of the ng tube comes out the nasal nares. Once the location of the tube is checked, the stent is left in place for six weeks. Our experience, with seven patients, has shown excellent tolerance to the stent; at the third post procedure day the patients were eating soft diet by mouth, we believe that the esophageal spasm had resolved by this day. Once the stent was withdraw, five cases were free of esophageal lesions, in the other two small areas of bleeding were visualized. In the follow-up the five cases that were free of lesions, had a normal esophageal diameter, in the other two, one had a moderate restenosis and the other case was severe.

[The usefulness and evaluation of postanorectoplasty sagittal posterior biofeedback]. / Utilidad y evaluación del biofeedback postanorrectoplastia sagital posterior.

Posterior sagittal anorectoplasty (ARPSP) was described at first of the 80-90 decade like a new surgical technique for the anorectal malformation treatment and derived fecal incontinence. We have evaluated 35 of 67 ARPSP using biofeedback techniques (BFB) before surgery as restore system to striate muscle. Manometric values of the canal anal profile (PCA), postoperative external sphincter pressure and postoperative, first and last BFB voluntary contraction pressure shows clearly the differences between surgical intervention and BFB outcome. The author's opinion is that BFB is the indispensable therapy in the ARPSP postoperative to lend the patients get optimal fecal continence.

[Extrahepatic cholestasis of nonatretic origin. New diagnostic and therapeutic possibilities]. / Colestasis extrahepatica de origen no atresiante. Nuevas posibilidades diagnosticas y terapeuticas.

Nonatretic cholestatic disorders exhibit differential features in the pediatric age group, at times not too obvious but always discernible. These characteristic differences, with the aid of increasingly more precise exploratory techniques, make a rapid and sound diagnosis possible. At present, echography, transparietohepatic cholangiography and endoscopic retrograde cholangiography allow us, within a short time, to reach a diagnosis of disorders that not long ago were only roughly profiled and on occasion were left undiagnosed. At the Children's Hospital Valle de Hebrón, treatment has been afforded, in the last seven years, to seven patients with a choledochal cyst, four with a long common biliopancreatic duct, one with a choleperitoneum due to spontaneous perforation of a choledochal cyst and one patient with congenital stenosis of the common hepatic duct. In all cases where echography was performed, an intra- or extrahepatic bile duct dilatation could be demonstrated, or otherwise the diagnosis of a choledochal cyst was established. Furthermore, four transhepatic and three retrograde cholangiographic examinations were carried out, via a fiber duodenoscopy, in those cases that failed to show, on echography, the existence of a choledochal cyst.