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C Marhuenda

Publications and source records attributed to C Marhuenda.

At least 19 recordsLinked to original sources

[New surgical strategy in gastroschisis: treatment simplification according to its physiopathology].

INTRODUCTION: Gastroschisis (GS) is a congenital abdominal wall defect that permits bowel exposure to amniotic fluid (AF). Intestinal damage is related to the chemical action of AF and constriction. After birth at term, a thickened intestinal wall with inflammation and, in some cases, intestinal atresias were observed. Surgical repair and intestinal reubication may be difficult, and thus staged silo repair could be necessary. These patients require a long hospital stay owing to bowel damage causing severe intestinal hypoperistalsis and poor absorptive capacity. Total parenteral nutrition (TPN) is required for a long period. OBJECTIVE: The aim of this prospective study is to evaluate the benefits of a preterm delivery to avoid bowel damage and its post-natal consequences. PATIENTS AND METHODS: Six cases of prenatally-diagnosed GS have been treated following a new strategy since July 2002. A preterm Cesarean section (c-section) delivery was programmed at 34-35 weeks of gestational age (GA). Some hours after birth, at bedside in the NICU, bowel reduction through the defect hole was performed under general anesthesia. This preterm group (PT) was compared the past 6 cases at term (AT) from January 1998 to July 2002. Macroscopic appearance, atresia existence, surgical technique, silo requirement, neonatal outcome, TPN and hospital stay were analyzed. RESULTS: All six cases AT (mean GA: 36.3 weeks) presented bowel inflammation and thickened wall. Only 2/6 cases allowed the intestine to be housed in a primary closure after laparotomy. 4/6 cases required staged silo repair. 1 patient presented intestinal atresia and other had perforations who died at 17 days of life from intestinal sepsis. Mean postoperative intubation period was 16.2 days. Mean TPN was 41.2 days and mean hospital stay 69.8 days. PT group was monitored by prenatal sonography seeking bowel sonolucency. After programmed PT c-section delivery (mean GA: 34.8 weeks) in all 6 cases, bowel loops presented normal appearance and intestinal thickening was absent, except in one case. No prematurity-related respiratory complications were observed. Easy bowel reduction without abdominal compression was performed in all cases. 1/6 cases required surgical release of occlusive intestinal adherence. Mean postoperative intubation period was 0.4 days (9.6 hours). Oral feeding was started at 6 days. Mean TPN was 13.4 days and mean hospital stay 28.6 days. CONCLUSIONS: The third trimester is a critical period for fetal bowel development. Intestinal damage rises with increasing exposure time to amniotic fluid. This strategy of preterm delivery for the treatment of GS avoids intestinal damage, prevents "peel" and intestinal atresia, renders surgical reduction easier, reduces the hypoperistalsis, need for TPN and hospital stay. Multidisciplinary coordination between obstetricians, neonatologists and pediatric surgeons is required.

Digestive System Surgical Procedures↗

Lung transplantation in young infants with interstitial pneumonia.

OBJECTIVES: We describe our experience with infants suffering from interstitial pneumonia referred for lung transplantation. METHODS: From April 1998 to December 2000, three infants were admitted to our lung transplantation program: a 9-month-old girl (patient 1) suffering from surfactant protein C deficiency who had high oxygen requirements (fraction of inspired oxygen: 70% to 90%), and two boys, ages 2 (patient 2) and 9 months (patient 3), who were ventilator-dependent due to chronic pneumonitis of infancy. RESULTS: Patients were transplanted at the age of 5 months (patient 2) and 13 months (patients 1 and 3) at 87 to 105 days after being accepted for lung transplantation. All cases underwent a sequential double lung transplant on cardiopulmonary bypass. The immunosuppressive regime included tacrolimus, prednisone, and azathioprine. Patients 2 and 3 also received basiliximab. Two cases suffered a mild rejection episode that responded to high-dose steroids. Patient 2 was ventilator-dependent for 8 months after transplant, owing to severe bronchomalacia and left main bronchus stenosis. Bronchial stenosis resolved after pneumatic dilatation and endobronchial stenting. This patient also presented with a pulmonary artery anastomosis stricture that required percutaneous balloon dilatation. All three patients are at home, carrying out normal activities for their age, with no respiratory symptoms after a period of 8 to 29 months of follow-up. CONCLUSIONS: Interstitial pneumonia of infancy is a rare disease with a bad prognosis and no specific treatment; therefore, lung transplantation represents a good therapeutic option for these infants.

Female↗

Surgical approach to funnel-shaped congenital tracheal stenosis.

PURPOSE: The aim of this study was to evaluate the results obtained by 2 different techniques of tracheoplasty in the treatment of long-segment, funnel-shaped congenital tracheal stenosis (CTS) in 2 tertiary paediatric hospitals. METHODS: The clinical records of patients with long segment congenital tracheal stenosis during the period January 1990 to June 1999 were reviewed retrospectively. Age, gender, symptoms, imaging (x-ray, computed tomography, magnetic resonance imaging, or bronchography), endoscopic findings, associated anomalies, treatment, intubation time, postoperative treatment, intensive care unit stay, complications, hospital stay, evolution, and follow-up time were analyzed. Short congenital tracheal stenosis treated by resection and end-to-end anastomosis are not included. Results are given as mean +/- SEM. RESULTS: Ten (7 boys, 4 girls) of 14 patients with CTS had a funnel-shaped CTS. Mean age at treatment was 9.7 +/- 4.8 months (range, 3 days to 4.7 years). All of the patients presented with CTS affecting the distal third of the trachea and 4 of them extension to 1 main stem bronchus. They were treated by 2 different types of tracheoplasty: anterior costal cartilage graft (ACGT, 6 patients) and slide tracheoplasty (ST, 4 patients). All the patients treated by ACGT failed: 4 died and 2 required further surgery for restenosis. Causes of death were acute respiratory failure during surgery (2 cases) and anastomotic dehiscence (2 cases). The patients treated with ST are asymptomatic and doing well. Mean postoperative intubation time of the ACGT group was 35 +/- 25 days (10 to 60 days), whereas in the ST group it was 14 +/- 12 days (0 to 51 days). Mean hospital stays were 292 +/- 271 days (21 to 563 days) and 24 +/- 13 days (7 to 63 days), respectively. Mean follow-up time is 28 +/- 14 months (3 to 94 months). CONCLUSIONS: Surgery of long-segment congenital tracheal stenosis has a high failure and complication rate with vital implications in prognosis. Treatment should be done in a multidisciplinary basis by a highly trained and motivated team. Slide tracheoplasty seems to be the better option, although further multicentre studies should be conducted.

Child, Preschool↗

[Treatment of caustic stenosis of the esophagus with self-expanding devices].

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.

Adolescent↗

[Nineteen years experience with posterior sagittal anorectoplasty as a treatment of anorectal malformation].

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.

Adolescent↗

[Bile duct atresia: outline for a solution].

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.

Adolescent↗

Conservative treatment of caustic esophageal injuries in children: 20 years of experience.

The authors present their experience in the medical treatment of 1, 296 caustic esophageal injuries in children over the last 20 years in two study groups, one comprising the period up to 1989 and the other 1990 to 1996, comparing the different treatments used in each group. The treatment was based fundamentally on dilatations with anterograde mercury bougies, Savary bougies, or retrograde thread-guided bougies with gastrostomy. Pneumatic balloons or stenting procedures have also been employed in the last 3 years. Early fiberendoscopy was used systematically in the second group, which provides a more accurate evaluation of the esophageal lesions. Antibiotic coverage was done systematically during the first 10 days in all serious cases, while steroids were employed routinely only in the last 3 years. The results were similar in both groups, with a dilatation average of 32 in the first and 30 in the second group and an initial dilatation interval of 3 to 4 weeks in both. Using updated exploration and dilatation techniques, we drastically reduced the number of gastrostomies needed for retrograde thread-guided dilatations from 51 in the first group to 5 in the second, consequently improving the patients' life quality. There was no mortality and only five esophageal perforations, which did not require surgical treatment.

Anti-Bacterial Agents↗

[Intraesophageal stent in the prevention of stenosis caused by caustic ingestion].

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.

Age Factors↗

[Laparoscopy for the undescended testis].

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.

Child↗

[Pulmonary biopsy by thoracoscopy: techniques and results in 9 patients].

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).

Adolescent↗

[The usefulness of manometry in the determination of the morphology of the anal canal].

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.

Adolescent↗

[Transjugular portosystemic shunt. An alternative in recurrent hemorrhage in portal hypertension in children].

The transjugular portosystemic bypass is a new technique for treating portal hypertension of an intrahepatic nature by inserting a multipurpose catheter through the jugular vein and vena cava as far as the suprahepatic vein, with the aid of a puncture device it is fed via the hepatic parenchyma into a portal branch. The passageway thus created is distended with a balloon to permit the insertion of an extensible metal mesh prosthesis or "stent" to maintain a permeable connection. Our experiment was conducted on a paediatric patient suffering from mucoviscidosis with severe pulmonary and hepatic involvement and recurrent bleeding in an uncontrollable position. After inserting the bypass, portal pressure dropped sharply and the bleeding stopped, the patient being discharged on the sixth day. Two months after the bypass, the coronary vein of the stomach, the seat of the gastric varicose veins, was selectively occluded through the stent via the femoral vein. Six months after follow-up, the digestive bleeding has not recurred.

Adolescent↗

[Our experience in the management of portal cavernoma in children].

The authors present 14 patients affected with portal cavernoma, diagnosed and controlled over the last 12 years. The splenomegaly (60%) and the digestive hemorrhages (40%) were the initial study causes. The cavernoma appeared idiopathic in 10 patients or secondary either to a catheterization of umbilical vein (3 cases) or onfalitis (1 case). The first clinical manifestation oscillated between the ages of 3 months to nine years. The diagnosis was performed in the initial cases (4 cases) using splenoportography (3 cases) and superior mesenteric arteriography (SMA) in the other. In the remaining cases the ultrasonography together with the endoscopic evidence of the varies backed up the diagnosis. Before the intervention, a SMA was performed in all cases. The surgical decision was taken with regards to the persistence of the digestive bleeding after the start of medical treatment with propranolol, cimetidine, and sucralfate, along with the existence of dangerous varices. Eight patients were operated on, two by the Warren technique, four by mesocaval jugular graft interposition (MJI) and two by azigo portal disconnection (APD). The shunts initially controlled the bleeding in all cases. There was one rebleeding instance at eleven months in a patient operated on by MJI, and in another by APD. Four patients continue medical treatment without problems, and two are free of treatment. Our experience with varies sclerosis is limited to two cases, although since the beginning of the treatment, one year ago, neither of them has bled.

Angiography↗

[Nitric oxide and Hirschsprung's disease: a causal relation biochemically, immunohistochemically and functionally demonstrated].

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)

Arginine↗

[Considerations regarding the treatment of non-aganglionic congenital intestinal neuropathies].

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.

Anastomosis, Surgical↗

[Prognostic factors in congenital diaphragmatic hernia. Can they modify our therapeutic approach?].

Mortality rate from congenital diaphragmatic hernia (CDH) is still high despite current therapeutic advances. For some time now we have been looking for prognostic parameters which be enable us to determine, in the prenatal and postnatal period, the patients who will die from their pulmonary hypoplasia in spite of treatment and those who will survive. The authors review 94 newborns with CDH diagnosed during the first 24 hours of life. The following prognostic parameters were studied: preoperative arterial gases, position of stomach, right or left sided hernia, liver in thorax and presence of postoperative pneumothorax. All these factors were related to mortality. The arterial gases were the most sensitive factor. Eighty per cent of patients with poor gasometric prognosis died. Thoracic stomach is also a poor prognostic parameter with a 76.7% mortality rate, however we think this does not justify prenatal surgical measures. The remaining parameters were not significant with regard to mortality.

Blood Gas Analysis↗

[Acute gastric volvulus in a patient with asplenic syndrome].

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.

Acute Disease↗

[Anorectal manometry in Hirschsprung disease].

To show the absence of inhibitory reflex of the anus (R.I.A.) by ano-rectal manometry is an important sign in the diagnosis of Hirschsprung's disease. Between 1989 and 1991, a series of 199 patients was studied. The patients were divided into two groups: 0-1 month and 1 month to 50 years. Both groups were subdivided into cases with or without clinical and radiological suspicion of aganglionism. In this series, R.I.A. was absent in 88 patients and present in 111. Histologic study and follow-up confirmed the existence of aganglionism in 86 patients, hyperganglionism in 1, anal stenosis in 8, cystic fibrosis in 4 and 1 coeliac disease; the rest were megarecta to a greater or lesser degree. There was 1 false negative, signifying a percentage of error of 0.5%.

Adolescent↗