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Biomedical subjects

A L Luis

Publications and source records attributed to A L Luis.

13 recordsLinked to original sources
PubMed2006

Liver transplantation for malignant tumours in children.

OBJECTIVE: The object of this study was to analyse our results with liver transplantation (LTX) for primitive malignant unresectable liver tumours in children and to discuss the controversial indications, based on our experience. METHODS/PATIENTS: We report on 12 patients, aged 6 months to 14 years, with hepatic malignant tumours: 11 with hepatoblastoma and 1 with fibrolamellar hepatocelullar carcinoma without cirrhosis. LTX was the primary treatment in 10 patients (PRETEXT IV or any grade, if there was extension to the retrohepatic vena cava, 3 hepatic veins or portal vein) and a rescue therapy after recurrence for 1 and for persistence of unresectable macroscopic residuals in 2 patients. One of the patients who underwent a LTX as primary therapy had lung metastases previously resolved with chemotherapy. We used entire liver (n = 5), left lateral segment from cadaveric donor (n = 3), living related donor (n = 3; 2 segments II-III and 1 right lobe) and left lateral segment from split liver (n = 1). All children received chemotherapy prior and post transplantation following the SIOPEL protocol. We analysed procedure tolerance, survival, recurrence rate, disease-free period and risk factors for adverse evolution. RESULTS: All patients overcame LTX and no early graft loss was recorded. Two cases died because of tumour relapse, 1 after primary LTX and 1 after rescue LTX (survival rate of both groups, 90% and 50%). Graft and patient survival rates at 1 year, 3 years, 5 years and 14 years were 91%, 91%, 82% and 82% respectively. The boy who presented with lung metastases developed new ones one year after LTX that were removed and he is currently free of disease. The disease-free period has a probability at 1, 3 and 5 years of 91%, 75% and 75%, respectively. Tumour tissue persistence was the only risk factor for an adverse clinical course in our series. CONCLUSIONS: LTX is a reasonable therapeutic approach for unresectable malignant liver tumours, providing outcomes comparable to those for resectable tumours. Results obtained with LTX are better when it is used as a primary treatment than when used as a rescue procedure. Proper staging and early referral to centres with enough expertise optimise the results. LTX for patients with lung metastases could be a controversial option. Living related-donor transplantation is an excellent alternative to avoid disease progression while on the waiting list for cadaveric donors.

Adolescent↗
PubMed2006

Non stenotic food impaction due to eosinophilic esophagitis: a potential surgical emergency.

AIM: Eosinophilic esophagitis (EoE) is an emergent condition in which a mucosal infiltrate of > 20 eosinophils per high power microscopic field is accompanied by motor disturbances that may cause food impaction in the absence of esophageal stricture. We report a series of such cases to point out the potential involvement of pediatric surgeons in diagnosis and treatment. Furthermore, data on the motor function of the esophagus investigated manometrically is included. MATERIAL AND METHODS: Thirteen patients with EoE were referred to our emergency room for acute food bolus impaction. Their median age at diagnosis was 12 years (range 7.6-14.4). History of allergy, endoscopy with biopsy and esophageal function (24-h combined ambulatory manometry with simultaneous pH-metry) were investigated. RESULTS: In 7 patients emergency endoscopic extraction of the impacted bolus was necessary. Allergic tests were positive in eight patients. The pH probe showed gastroesophageal reflux in two cases. Upon endoscopy, typical features of EoE (esophageal trachealization and whitish papular exudates) were found. Ambulatory 24-h manometry revealed abnormal motility of the distal esophagus with strikingly high amplitudes (> 150 mmHg) and long duration (> 7 sec) of the waves, particularly during the night. Six patients responded rapidly to steroids and/or antiallergic treatment. The remaining patients had a good outcome with dietary treatment alone. CONCLUSIONS: EoE is an emergent condition that may involve the pediatric surgeon in both the diagnosis and treatment. Typical endoscopic findings and biopsy are required for proper diagnosis. Ambulatory manometry reveals a marked propulsive dysfunction that explains impaction. This dysfunction is reversible, since the symptoms usually disappear with steroids or antiallergic treatment.

Adolescent↗
PubMed2005

Impact of preoperative diagnosis of congenital heart disease on the treatment of esophageal atresia.

Congenital heart disease (CHD) has a major impact on the survival of babies with esophageal atresia (EA). The present study assesses whether early diagnosis influences the management strategies in a large series of EA. Cases of EA treated between 1982 and 2002 were retrospectively divided into groups according to the presence or absence of CHD and to whether this was diagnosed or not prior to tracheo-esophageal fistula repair. Patients were also staged according to Spitz's classification in which major congenital heart disease (MCHD) are those associated with cyanosis and/or heart failure requiring surgery. Comparisons between groups of patients were made by standard statistical tests. Among 195 babies with EA (99 boys and 96 girls), 82 (42%) had CHD (31 boys, 39% and 51 girls, 61%, chi2 P < 0.05) and 43 out of these 82 (52%) had MCHD. Six children died without treatment. CHD was diagnosed prenatally in six cases and before EA repair in 26 cases. The diagnoses were missed in 12 instances (in five it was a MCHD). Regardless of preoperative diagnosis of right aortic arch in 3/6 cases, the oesophagus was approached from the right thorax in all cases and only in one of them the operation was not completed due to hemodynamic instability. There were 145/195 (75%) Spitz group I patients; 44/195 (22%) were group II and 6/195 (3%) group III. The mortality was respectively 9.5, 59 and 83%. We lost 15/113 (13%) babies with EA without CHD, 4/39 (10.2%) with EA and CHD and 26/43 (60%) with EA and MCHD. In children with CHD diagnosed prior to the esophageal operation 8/26 (30.7%) died and among those without diagnoses 3/12 (25%) died, (chi2 P > 0.1). 1. CHD was diagnosed after EA repair in one-third of cases. 2. Preoperative diagnosis of CHD changed the operative indication only in one case. 3. Survival after treatment for EA was not influenced by the moment or the accuracy of the diagnosis of CHD in this series. 4. With only a few exceptions, associated CHD should not change the strategies of EA repair.

Abnormalities, Multiple↗
PubMed2005

[Chest wall distraction in thoracogenic scoliosis].

INTRODUCTION: Combined congenital scoliosis and rib fusion associated with other chest deformities during infancy can lead to a progressive hypoplastic thorax that could be unable to support normal lung growth and respiratory function. Campbell introduced an expansion thoracoplasty technique in which fused ribs are separated and a vertical expandable prosthetic titanium rib is used as a chest wall distraction device to enlarge the affected hemithorax. This technique benefits the underlying lung by improving the thoracic volume and the respiratory function. PATIENTS AND METHODS: Four patients (3 boys and 1 girl) with severe unilateral thoracic deformity with combined scoliosis and rib fusion plus a restrictive respiratory insufficiency are presented herein; 1 of them needed continuous CPAP preoperatively. All had progressive scoliosis and failure to thrive. Preoperative evaluation included three-dimensional CT reconstruction. A thoracic expansion placing an intercostal vertical expandable prosthetic titanium device as a chest wall distractor was performed. Afterwards, distraction control was made every four months. RESULTS: Our 4 patients had a satisfactory outcome. Scoliosis was corrected and there were improvements of volume and function of the thorax. CONCLUSIONS: The placement of an intercostal distractor device improves the thoracic insufficiency syndrome, lengthening and expanding the thoracic cage at the same time. These effects benefit respiratory function and correct scoliosis, allowing an adecuate lung function.

Child↗
PubMed2005

[Cardiac function alterations in pectus excavatum].

BACKGROUND: Cosmetic deformity and psychological repercusion are the main surgical indications in pectus excavatum. However cardiopulmonary function is subclinically abnormal in some patients. The goal of this paper is to demonstrate that heart response during physical activity improves after surgical correction of pectus excavatum. PATIENTS AND METHODS: Fifty five patients were operated upon for pectus excavatum during the last 10 years at our institution. Baseline ecocardiography and isometric effort test (Handgrip) were preoperatively performed in 15 patients (11 males and 4 females) aged from 6 to 15 years (median 9,63). An increase below 12% in cardiac index was considered as positive. In those patients with positive tests, a new test was made 6 months after surgical correction. Pre and postoperative results were compared using non-parametric tests. RESULTS: Baseline cardiac index values were normal in all cases (11). Four patients were not included, 2 because of no collaboration (aged 6 and 7 years) and 2 because difficult ecocardiography interpretation (both operated upon for congenital diaphragmatic hernia). Overall, preoperative test was negative in 6 patients and positive in the remaining 5. Postoperative test was made in these 5 patients, and all of them showed an improvement in cardiac index (medium 40,6%, range 25-70) when compared to preoperative values. There was statistically significant association between test values and patient ages. CONCLUSIONS: Baseline cardiac index values were normal in children with pectus excavatum, albeit 45,5% of them showed a limited response to exercise. Pectus excavatum repair improves these values, so physiopathological indication as well as cosmetic one should be considered specially in children.

Adolescent↗
PubMed2004

[Congenital neuroblastomas].

BACKGROUND: We consider congenital neuroblastomas (CN) those detected in pregnancy or at the very first hours of life. Due to perinatal sonography, its incidence has increased in the last years. We present herein our experience in the treatment of this condition and we try to find out any different clinical pattern from those neuroblastomas diagnosed later in life. METHODS: We review the CN treated in our hospital from 1990 to 2003, analyzing diagnosis, localization, tumor staging, N-myc amplification, treatment and evolution. RESULTS: Among the 107 neural tumors managed during this period (89 neuroblastomas, 18 ganglioneuromas), 8 were congenital neuroblastomas (7 girls, 1 boy). Two patients had prenatal diagnosis and 6 tumours were detected in routine exploration or casual findings upon neonatal examination. Six were abdominal, 1 thoracoabdominal and 1 abdominopelvic with dumbbell invasion. Three tumours were classified like stage 1, 1 stage 2, 1 stage 3, 1 stage 4 and 2 stage 4S. Although most of them had unfavorable histology, we didn't find N-myc amplification in any tumor. All patients were operated upon, with preoperative chemotherapy in 2 of them. Resection was complete in 7 out of the 8 tumors. The patient who presented neurological symptoms at birth recovered neither motility nor bladder function after resection. All of them survive after 60+/-53 months of follow-up. CONCLUSIONS: The outcome in this group of neuroblastomas is better that expected, probably because of its abdominal location. On the contrary in dumbbell neuroblastomas, neurological damage at birth seems to be irreversible.

Female↗
PubMed2004

[Paraneoplasic syndrome in inflammatory pseudotumor of the lung].

BACKGROUND: Inflammatory pseudotumor (IPT) is the most frequent pulmonary mass in childhood. It is histologically benign but locally aggressive. Atelectasis and recurrent airway infections are the most frequent presenting findings. We present two children in whom first clinical signs were paraneoplasic syndromes. MATERIAL AND METHODS: Retrospective study of two cases of IPT treated in our clinic from 1998 to 2002. Age, clinical presentation, preoperative diagnosis, treatment, histological diagnosis and postoperative outcome were reviewed from clinical chart. RESULTS: Case 1: 7 year old male with incidental diagnosis of superior right lobe IPT in routine study because of diabetes. The mass collapsed superior and median lobar arteries and compressed superior cava vein. Right pneumonectomy was necesary to complete removal of the mass. From immediate postoperatory the child became normoglycemic and is free of insulin. Case 2: 11 year old male with rheumatologic clinic consisting in hypertrophic osteoarthropathy, arthralgy and knees liquid lasting for two years. X-ray examination showed mediastinic mass. He underwent complete removal of a pulmonary mass. Few months after the operation the rheumatologic-like symptoms were resolved. CONCLUSIONS: IPT can present with paraneoplasic syndromes, although physiopathology is not well understood. In children with recurrent respiratory infections one diagnosis to take in mind is IPT, even more when clinic is associated with symptoms compatible with paraneoplasic syndrome.

Child↗
PubMed2004

[Midgut malrotation risk in abdominal wall defect].

BACKGROUND: Midgut malrotation has a low incidence (0.5-1% at necropsies) and it is a rare symptomatic condition. Nevertheless it is often associated to clinical situations with a high morbility and mortality and it could complicate them. Our aim is to study the patients treated for abdominal wall defects and who have suffered complications due to non-treated malrotation at first surgery. METHODS: We retrospectively studied the charts of patients diagnosed of abdominal wall defects in our hospital from 1993 to 2002. We reviewed the initial treatment, the associated morbility and any new surgical treatment needed. RESULTS: During this 10-year period, 110 abdominal wall defects were managed: 56 congenital diaphragmatic hernias, 30 onphaloceles and 14 gastroschisis; overall 79 of these 100 patients survived and were included in this study. Eleven patients had symptoms due to bowel malrotation during the follow-up period. In 1 case the clinical picture had an acute onset as a volvulus that required extensive gut resection; another patient presented as a persistent gastrocutaneous fistula after removal of a gastrostomy tube; in the remaining 9, symptoms were always of classical intestinal obstruction. The malrotation was never treated during the initial surgical procedure for abdominal wall defect; later on, Ladd procedure was always the definitive treatment. CONCLUSIONS: Due to local conditions during first abdominal wall surgery that limit the evaluation of the malrotation, we must think about it when we find obstructive symptoms any time during life.

Child, Preschool↗
PubMed2004

[Lipoblastoma: the least well known of adipose tumors].

INTRODUCTION: Lipoblastoma is the least known of adipose tumours. It comes from embryonic adipose tissue and though it is histologically benign, it is locally invasive, implying a high risk of relapse if it is incompletely removed. The pediatric surgeon should be familiar with this tumor since it usually appears in children under three. MATERIAL AND METHODS: A retrospective study of the patients who were operated upon at our institution with a histologic diagnosis of lipoblastoma from 1966 to 2002 taking into account: age, tumor site, cytogenetic studies, first diagnosis, treatment and clinical course. RESULTS: The diagnosis of lipoblastoma was carried out in six patients (three boys and three girls). All were diagnosed before the first year of life and one was present at birth. In only one case the diagnosis was pre-operative. Two tumors were thoracic, two paravertebral with an intrarachidian component without spinal cord involvement (one of them was associated with myelomeningocele and diatomyelia), two in limbs and one perineal. A girl operated for the first time at four months, was latter operated in four opportunities for a relapse. In the last operation the excision was incomplete due to a subclavian involvement; in the last 15 years no new tumor growth was seen. After a follow-up period of 1 to 25 years in the other six patients no relapse was observed. CONCLUSIONS: Despite its low frequency, the diagnosis of lipoblastoma must be considered in children with masses in soft tissue, mainly if they are younger then one year. Because the lipoblastoma is locally invasive, this tumor must be treated before it affects viscera. The resection must be complete, avoiding the risk of relapse, although radical mutilating surgery is not recommended.

Female↗
PubMed2003

[Early esophageal replacement in patients with esophageal atresia].

INTRODUCTION: When primary anastomosis is not feasible in esophageal atresia esophageal replacement is one of the possible options. We report our experience with this approach in patients with long-gap esophageal atresia. MATERIALS AND METHODS: From 1991 to 2002 we treated 50 children with esophageal atresia. Nine required esophageal replacement because of long-gaps. Six were boys and three girls. Six had isolated atresia, and three had regular atresia with TEF. Associated malformations in 8 patients were: cardiovascular (2), anorectal (2), urogenital (5), intestinal duplication (1), and costovertebral (1). The weight at birth ranged from 1.2 to 3.95 kg (median 2.3). Patients with type I atresia had immediate gastrostomy accompanied by esophagostomy in one. Esophageal anastomosis was impossible or failed in 3 patients with type III EA, who had fistula ligation and esophagostomy. Esophageal replacement was performed at a median age of 4.95 months (range 2.3 to 18), with a median weight of 5.33 (range 2.89 to 11.5 kg.). We used gastric pull-up in 2 cases and colonic transposition in 7 with isoperistaltic left transverse colon in retromediastinal-transhiatal (6) or restrosternal (1) position. Two patients had extramucosal piloromyotomy and 4 had piloroplasty. RESULTS: All patients survive and have functional grafts. Postoperative complications were: pneumonia (1), wound dehiscence with evisceration (2) and salivary fístula (2) which closed spontaneously. In the long range, one patient was operated three times for hernia through the enlarged hiatus, one had one episode of intestinal subobstruction successfully managed with nasogastric aspiration and another one had dumping syndrome for several months. After a median follow-up of 3.15 years (range 1.6 to 6.9) all patients eat normal diets per os. CONCLUSIONS: Esophageal replacement for the treatment of infants with long-gap EA has been in our hands as good as any other option with 100% survival and good functional results. The operation can be safely performed in the first months provided that associated malformations are under control. However, like other options, this approach is not devoid of complications.

Esophageal Atresia↗
PubMed2003

[Severe complications in the treatment of vascular anomalies].

BACKGROUND: Success in the treatment of vascular anomalies during infancy depends on an accurate early diagnosis and a correct therapeutic management. Current available resources can be divided into pharmacologic, endovascular, surgical, and laser. Results are variable, and complications should always be kept in mind. METHODS: We reviewed all patients with vascular anomalies who suffered from major complications directly due to the treatment during the past five years. RESULTS: 1. A patient with an upper lip hemangioma following treatment with interferon after no response to steroids. A long-term severe neutropoenia forced to an early surgical excision of the lesion. 2. A patient suffering from an orbitary hemangioma with severe exoftalmus was treated with interferon because of a steroid-resistance. A spastic diplegia due to interferon reverted after treatment was discontinued. 3. In a patient with a Blue-Rubber Bleb Nevus syndrome, extended percutaneous sclerosis was performed. She developed skin necrosis of the left leg and a permanent sciatic nerve paralysis. 4. In a patient with an arteriovenous malformation on the right leg and gluteous, a femoral artery endoprosthesis was placed because of a massive bleeding. She underwent a total excision of the malformation and developed a recurrent ischemia related to a femoral thrombosis. Finally a foot amputation was needed. 5. A patient with a Kaposi-like hemangio-endothelioma who was following a high-dose prolonged steroid therapy died because of a meningoencephalitis related to a severe immunosuppression. CONCLUSIONS: Treatment of vascular anomalies during childhood need an early accurate diagnosis. If a right therapeutic sequence is not performed, useful drugs can turn into inefficient or dangerous. Most treatment complications in these patients can be avoided.

Blood Vessels↗