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The management of children receiving small bowel grafts involves potentially life-threatening complications that affect their nutritional status. The aim of this paper was to define these factors and their influence on nutritional outcome.Patients with intestinal failure (IF) who received an isolated small bowel transplantation (SBT) or small bowel/liver transplantation (SBLT) at our hospital during the last 6 years were reviewed for weight Z-score, biochemical nutritional parameters, total parenteral nutrition (TPN) weaning, catheter-related sepsis, rejection and steroid treatment.Twenty patients, 11 females and 9 males, received a SBT or a SBLT and survived the postoperative period; in the present study we only included 11 children with follow-up periods longer than 1 year. Seven males and 4 females with a mean age of 4.5 years (range, 1 to 20 years) received 6 SBLT and 5 SBT. Nine (82%) were weaned from TPN to an amino-acid or peptide enteral formula during the first 6 months after surgery. During the first year there was a significant increase in total protein from 5.11 +/- 1.8 mg/dl to 6.1 +/- 1.5 mg/dl (p0.05) and an increase in albumin from 3.8 +/- 0.9 mg/dl to 4.5 +/- 1.1 mg/dl (p0.05). There was an increase in weight Z-score in 9 patients (82%) during the first year. Mean Z-score improved from - 2.6 +/- 1 at transplant to - 1.0 +/- 0.6 (p0.05) after 1 year. Three patients (27.2%) had at least one rejection period, which was treated with steroids alone or in combination. Mean weight Z-score 1 year after surgery was - 0.9 +/- 0.6 for patients without rejection and - 1.24 +/- 0.8 for those with at least one rejection episode treated with steroids (p0.1). Four patients (36%) had at least one catheter-related sepsis episode. Mean weight Z-score 1 year after surgery was - 1.01 +/- 0.6 for patients without catheter-related sepsis and - 1.24 +/- 0.8 for those with at least one catheter-related sepsis episode (p0.1).There was a significant improvement in weight Z-score and biochemical nutritional parameters 1 year after receiving a small bowel graft. No influence of steroids or catheter-related sepsis on children's nutritional status was noted 1 year after surgery, although this point will need further evaluation.

Antecedentes: El tumor carcinoide es una rara neoplasia neuroendocrina con localizaciones diversas, capaz de producir sustancias vasoactivas y asociada ocasionalmente al síndrome carcinoide (enrojecimiento, diarrea y sibilancias). Pacientes y métodos: Revisamos las historias de los pacientes con diagnóstico de tumor carcinoide en nuestro servicio entre 1966 y 2003. Resultados: Diez pacientes (5 niños y 5 niñas con una edad media de 8 años) fueron diagnosticados de tumor carcinoide. Ocho tenían carcinoides apendiculares de los que cuatro presentaron síntomas de apendicitis aguda. De estos ocho, siete eran menores de 2 cm y estaban localizados en la punta del apéndice. El paciente restante presentaba un tumor carcinoide de 3,5 cm en la base apendicular por lo que fue reintervenido para realizar una resección ileocecal. Dos pacientes de 4 y 6 años presentaron un tumor carcinoide bronquial asociado a sintomatología respiratoria crónica y se llegó en ambos al diagnóstico acertado tras un año de seguimiento. Se realizó neumonectomía en un caso y resección bronquial con anastomosis traqueobronquial en el otro. Ningún paciente de la serie presentó sintomatología carcinoide. Se realizaron controles bioquímicos, radiológicos y fibrobroncoscópicos en los bronquiales. Todos se encuentran actualmente libres de enfermedad. Conclusiones: El tumor carcinoide apendicular no se asocia a sintomatología característica de apendicitis aguda en la mitad de los pacientes. Encontramos que el síndrome carcinoide asociado al tumor carcinoide es excepcional en la edad pediátrica. En la mayor parte de los tumores carcinoides apendiculares la apendicectomía simple es suficiente con un pronóstico excelente. El tumor carcinoide bronquial es diagnosticado generalmente de manera tardía por lo que debemos considerar este diagnóstico en pacientes con sintomatología respiratoria crónica. : Background: Carcinoid tumor (CT) is an unusual neoplasm observed in several locations and associated with the production of vasoactive substances and occasionally with carcinoid syndrome (flushing, diarrhea, wheezing). Patients and methods: A retrospective review of the medial records of all children with a diagnosis of CT treated in our service between 1966 and 2003 was performed. Results: Ten patients (5 boys and 5 girls with a mean age of 8 years) were diagnosed with CT. Eight had CT of the appendix, of which 4 showed the typical clinical presentation of acute appendicitis. Seven of these tumors were localized at the tip of the appendix and measured 2 cm or less. In one patient, the tumor was located at the cecum and measured 3.5 cm. In this patient, reoperation with ileocecal resection was performed. Two patients (4 and 6 years old) had bronchial CT associated with chronic respiratory manifestations. An accurate diagnosis was made after a 1-year follow-up. Pneumonectomy and tracheobronchial sleeve resection were carried out. None of the patients showed symptoms of carcinoid syndrome. All the patients underwent biochemical and radiological studies and fiberoptic bronchoscopy during follow-up. All the patients are currently disease-free. Conclusions: Typical symptoms of acute appendicitis were not observed in half of patients with CT of the appendix. CT associated with carcinoid syndrome is exceptional in pediatric patients. In most CT of the appendix, simple appendicectomy was associated with an excellent prognosis. Diagnosis of bronchial TC tends to be delayed and consequently CT should be considered in the differential diagnosis of children with respiratory symptoms unre-sponsive to standard medical treatment.

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 P0.05) 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 P0.1). 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.

We present the case of a newborn with sternal cleft (SC) and presternal enterogenous cyst operated on during the neonatal period. SC is an uncommon congenital malformation of the thoracic wall which can occur as an isolated form or in association with other malformations. To our knowledge, the presence of SC and enterogenous cyst has not been described to date. Early surgical repair of SC gives good aesthetic and functional results and is usually the preferred approach.

This retrospective study reviews a series of teratomas of the neck and mediastinum aiming at defining the features of these particular locations. We recorded prenatal diagnosis, perinatal management, clinical and radiologic features, pathology, surgical strategies and results in cervical and mediastinal teratomas treated over the last 10 years. During this period we treated 66 children with teratoma of which 11 (6 male and 5 female) had cervicomediastinal locations. Five babies had cervical teratomas extended into the anterior mediastinum in two cases. Prenatal diagnosis was made in three (two with polyhydramnios). Four babies were born by C-section and only one had a successful EXIT procedure. The diagnosis was confirmed by imaging and increased AFP. Surgical treatment involved total tumor removal and in one case subsequent removal of lymph node metastases. All children survived except one in whom airway could not be cleared at birth. Two children bear mild hypothyroidism. During the same period six patients aged 0-17 years were treated for mediastinal teratoma. Only one was prenatally diagnosed and only two had some dyspnea. Removal was performed either by median sternotomy, thoracotomy, or thoracoscopy. They all survive and are free of disease. Teratomas of the neck may cause fetal disease and unmanageable neonatal airway obstruction. Prenatal diagnosis and planned multidisciplinary management are mandatory at birth. In contrast, only some mediastinal tumors cause respiratory embarrassment. Although benign, these tumors are sometimes immature and may metastasize to regional lymph nodes. Total surgical removal is curative. Thyroid insufficiency may be present at birth in cervical teratomas and may be aggravated by surgery.

Background/Purpose: In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has been the usual approach for esophageal perforation (EP) in children. This report aims to assess whether this strategy stands the passage of time. Methods: We reviewed retrospectively the charts of 17 patients aged 5.3 ± 0.9 years (mean ± SD) treated at our institution for EP between 1991 and 2001. Results: Nineteen episodes of EP were caused by stricture dilation in 9 cases, foreign body extraction in 3, and blunt trauma and sclerosis of varices in 2 cases each. The remaining child had multiple gastrointestinal perforations in the course of chemotherapy for leukemia. Vigorous treatment, consisting of nasopharyngeal aspiration, wide spectrum antibiotics, prompt drainage of effusions and either parenteral or infraesophageal nutritition, was implemented immediately after diagnosis. Perforations were closed without direct surgery in 18 of 19 episodes (16 of 17 children). One or more pleural drains were inserted in 12 cases, and pericardial drainage was required once. Seven gastrostomies, 2 jejunostomies, and one esophagostomy were performed. Several major abdominal operations were necessary to repair concomitant lesions in a child who sustained severe blunt abdominal trauma and in the patient with leukemic perforations. All patients survived, and all recovered esophageal function. However, 2 with intractable lye strictures ultimately required esophageal replacement. The only patient in whom a direct approach for esophageal necrosis due to variceal endosclerosis was unavoidable, lost her organ and had a retrosternal colonic interposition after a successful portosystemic shunt. Excluding patients with other concomitant lesions and the patient who underwent surgery, median length of stay was 11 days (range, 6 to 47). Conclusions: Prompt and aggressive nonoperative treatment of esophageal perforations in children allows survival with conservation of the organ in most cases and remains, in the authors' hands, the first therapeutic choice at this age. J Pediatr Surg 38:685-689. © 2003 Elsevier Inc. All rights reserved.

Aim The aim of this case report is to emphasize the importance of an early diagnosis of Gardner's syndrome through the detection of lesions appearing in the oral and maxillofacial area as well as to present two cases of the disease. Background Gardner's syndrome is an autosomal dominant disease characterized by gastrointestinal polyps that develop in the colon as well as in the stomach and upper intestine, along with multiple osteomas, skin, and soft tissue tumors. Cutaneous findings may include desmoid tumors, epidermoid cysts, and other benign tumors. Early diagnosis and therapy of the disease are critical because polyps have a 100% risk of undergoing malignant transformation. Craniomaxillofacial manifestations (osteoma formation, tooth impaction, diffuse opacities in the skull, mandible and maxilla, scalp tumors) usually precede polyposis. Report Case 1: Gardner's syndrome was diagnosed in a 25-year-old Caucasian man who was referred by his endodontist for evaluation of an uncommon radiographic image in the mandibular molar area. Further investigation revealed a familial adenomatous polyposis (FAP) complicated by adenocarcinoma of the colon. A colectomy and an ileorectal anastomosis were performed. Case 2: A 12-year-old Caucasian girl, who is a niece of the patient described in Case 1, presented with progressive difficulty with mouth opening but no complaints of digestive problems. Radiographic examination revealed multiple radiopacities in the maxilla, mandible left temporomandibular joint, and in the left mandibular angle. Multiple impacted teeth were present. A colonoscopy was suggested, but the patient's parents decided to continue the investigation and treatment with their own physician in their home town. Summary Since an early diagnosis is essential and general dental practitioners may be the first healthcare professionals to suspect the diagnosis, it is important for them to be familiar with the features of Gardner's syndrome. Citation de Oliveira Ribas M, Martins WD, de Sousa MH, de Aguiar Koubik ACG, Ávila LFC, Zanferrari FL, Martins G. Oral and Maxillofacial Manifestations of Familial Adenomatous Polyposis (Gardner's syndrome): A Report of Two Cases. J Contemp Dent Pract 2009 January; (10)1:082-090.