[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.

[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 anterior urethral reconstruction with the 'BAES flap' in a spinal cord-injured patient.

STUDY DESIGN: A case report of urethral destruction in a spinal cord injured (SCI) patient. OBJECTIVES: To report the reconstruction of the whole anterior urethra in one-stage using an epilated scrotal flap. SETTING: Institut Guttmann, Hospital de Neurorehabilitació, Barcelona, Spain. METHODS: A one-stage tubular substitution urethroplasty based on a bi-axial epilated scrotal flap design ('BAES flap') was performed successfully. RESULTS: Long-term follow-up of 6 years has confirmed the excellent adaptation of the flap to its urethral function. CONCLUSION: The bipedicle epilated scrotal flap can effectively resolve this challenging urethral pathology.

[Treatment of caustic stenosis of the esophagus with self-expanding devices]. / Tratamiento de las estenosis cáusticas del esófago mediante dispositivos de autoexpansión.

Caustic stenosis is a serious problem in children due to its complicated resolution and implications in important areas like nutrition, as well as the child's tolerance to the measures taken to correct them. After dealing extensively with this problem over the last twenty five years, always from a conservative approach using traditional methods like dilatations, we believe we have found a technique that brings together all the necessary conditions to achieve a favorable and definitive solution. Our ideal goal to achieve a device that provides a well-tolerated permanent esophageal expansion during the scarring process, also permitting normal swallowing, seems to have been reached through the use of the new generation of silicone stents. The authors present their experience in the first seven cases of caustic stenosis treatment through the placement of silicone stents, describing a precise placement technique while establishing a standard protocol for the use of these devices.

[Bile duct atresia: outline for a solution]. / Atresia de vías biliares: boceto para una solución.

UNLABELLED: Biliary atresia continues to be a serious and relatively rare disease (1/50,000 newborns) and whose long-term prognosis has changed drastically since the appearance of liver transplant (LT) as a therapeutic weapon. The combination of two factors, early diagnosis and correct application of Kasai's surgical technique, is essential to obtain acceptable results and sufficient biliary drainage allowing the children to overcome the critical 7 kg barrier and place them in the lesser morbi-mortality range in relation to a possible LT. But we must keep in mind that despite its critics, Kasai's technique can guarantee, both in our own experience and in the literature, ten years survival percentages over 50% with correct hepatic function, as well as clinical normality and a quality of life clearly superior to first years post-LT. We present the evolution of a group of 20 patients affected with biliary atresia, diagnosed in our center since 1985, the year when pediatric LT began to be used as a therapeutic procedure in this country. We valued the age of intervention, technique, immediate and long-term results and the evolution and necessity of LT. All 20 patients were analyzed individually, and they currently have an age range from 2-14 years and were all operated by Kasai's technique. We classified the patients as having good, regular or poor results with regards to biliary flow, normalization of billirubin levels and clinical evolution. Sixteen patients presented biliary flow of such an extent that 14 of them, classified as good, completely normalized the billirubin levels. Two others, presently aged 14 and 8 years respectively, present average levels of 2.5-5.5 mg/100 ml and are classified as regular in a situation of advisable transplant, although with an acceptable hepatic function. Only one case, the first in the poor group, did not initially present biliary elimination and died at age six months while on the waiting list. Three other cases in the same group presented insufficient biliary elimination and were transplanted with 7, 11 and 12.5 kg, respectively. The second died in the first year post-transplant. CONCLUSIONS: In our opinion, action in biliary atresia must be early and based on the correct application of Kasai's technique, seeking to achieve a biliar flow that eliminates or distances the patient as far as possible from the necessity of a future LT. Three lines come together to obtain this target: an early diagnosis, a correct application of Kasai's technique, and an implication in the follow-up and treatment of these children by the hepatic transplant groups. All this advises us, as is done in other countries, to create reference centers for the study of neonatal cholestasis where an accumulated experience of a relatively rare pathology can be taken advantage of.

[Hydraulic prosthesis for regulating the sling tension in the treatment of urinary incontinence in women]. / Prótesis hidráulica para regular la tensión del "sling" en el tratamiento de la incontinencia urinaria en la mujer.

OBJECTIVE: To improve the results of corrective surgery for severe urinary incontinence in the female (sphincteric lesion) utilizing a new prosthesis that allows adjusting the degree of tension of the band or urethrocervical sling. METHODS: We have added the use of a subcutaneous hydraulic device to the classical pubovaginal sling procedure. The device can be gradually filled by percutaneous punction to adjust the urethral closure pressure. RESULTS: All patients had previously undergone different procedures and had grade III stress urinary incontinence. With the hydraulic device, the success rate (complete continence) was 95.5% (21/22 patients) and the complication rate was 9% (2 cases; one developed infection and the other chronic urinary retention). CONCLUSIONS: The hydraulic device described herein is easy to place, low-cost and carries a minimum morbidity. Its use is indicated in patients who have previously undergone surgery, with a sphincteric lesion and who require precise adjustment of the urethrocervical sling. The degree of tautness can be adjusted repeatedly according to the clinical course of a patient without requiring reoperation.

[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.

Achalasia and Down's syndrome: coincidental association or something else?

Achalasia is an uncommon esophageal motor disorder. It has been associated with other diseases such as Parkinson's disease and depressive disorders, but coincidence of achalasia and Down's syndrome is rare. We report five cases of achalasia in Down's syndrome patients seen in our institution. Two of the five cases were diagnosed at pediatric age. Respiratory symptoms and growth retardation were the main clinical manifestations in pediatric patients, whereas adult patients mainly complained of dysphagia. Taking into account the prevalence rate of both disorders, the association seems higher than that expected by chance. The possible etiopathogenic implications of this association, as well as its clinical relevance, are discussed.

[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.

Infantile hypertrophic pyloric stenosis and achalasia: NO-related or non-related conditions?

BACKGROUND: The regulated ability of sphincters to relax allows adequate control of digestive transit. Relaxation of the lower esophageal sphincter (LES) is essential for esophageal emptying and, similarly, pyloric relaxation permits gastric emptying. When the relaxatory response of these sphincters is impaired, luminal transit is altered, as occurs in achalasia and hypertrophic pyloric stenosis. Nitric oxide (NO) has been identified as the main inhibitory neurotransmitter in both sphincteric regions. Moreover, the absence of NO synthase in the LES and the pylorus has been implicated in the pathogenesis of infantile hypertrophic pyloric stenosis (IHPS) and achalasia, respectively. CASE REPORT: We present the case of a 12-year-old boy diagnosed with these two different conditions attributed to NO absence: IHPS and achalasia. CONCLUSION: To our knowledge this is the first time that such an association has been reported. Whether IHPS and achalasia have been associated in this patient by chance or because they share common pathophysiological mechanisms remains speculative, but is a tantalizing dilemma.