A. L. Luis

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%, χ2P<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, (χ2P>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.

Long-term results of the treatment of total colonic aganglionosis with two different techniques.

AIM Aim of this study was to assess the long-term results of the treatment of total colonic aganglionosis (TCA) with 2 different techniques in terms of growth, continence and quality of life (QOL). PATIENTS AND METHODS Forty-one patients treated for TCA between 1972 and 2007 were reviewed retrospectively with special attention paid to the length of aganglionosis, complications, growth, continence and QOL. Until 1992, patients underwent subtotal colonic resection and side-to-side ileosigmoid anastomosis (modified Martin). Since 1992, straight ileo-anal pull-through was preferred. At the end of follow-up (median 18 years, range 1-35), the height and weight, continence and QOL (scoring feeding habits, school/work performance, family life and professional development) were assessed by clinical visit or phone interview. RESULTS Twenty-eight patients were male and 13 female. Six had total intestinal aganglionosis and were excluded from this review together with the 2 who died before definitive treatment. The 33/41 persons in whom aganglionosis had involved less than 50 cm above the ileocecal valve and who had been considered suitable for the reestablishment of transanal fecal flow were included. Eighteen children underwent a modified Martin and 15 straight ileo-anal pull-through. Postoperative intestinal obstruction occurred in 4 cases, prolapse and prolonged TPN requirement in 2, and wound disruption and fistula in 1. Thirteen patients (39 %) had postoperative enterocolitis. Two children died after operation (1 wound disruption with sepsis and 1 pneumonia). Out of 31 survivors, 57 % and 53 % were > p50 with regard to height and weight whereas only 15 % and 19 % were <p3 respectively. Only half the patients had more than 3 bowel movements per day and the median Wildhaber continence score (normal = 14) was 11 (range 6-14). Both types of operations resulted in comparable defecation and continence patterns. QOL was rated as good in all cases but one. All patients but 2 attended high school, 8 attend university, 4 are employed and 1 is married and has 2 daughters. Social life is normal except for 1 patient who perceives his disease as a burden. CONCLUSIONS Patients with TCA amenable to reestablishment of the transanal fecal flow can have adequate growth, normal feeding, reasonably good continence and satisfactory QOL. However, complications and enterocolitis are frequent. A modified Martins procedure was performed as well as straight ileo-anal pull-through with little influence on the long-term outcome.

Tumor carcinoide bronquial y apendicular

Antecedentes El tumor carcinoide es una rara neoplasia neuroendocrina con localizaciones diversas, capaz de producir sustancias vasoactivas y asociada ocasionalmente al sindrome carcinoide (enrojecimiento, diarrea y sibilancias). Pacientes y metodos Revisamos las historias de los pacientes con diagnostico de tumor carcinoide en nuestro servicio entre 1966 y 2003. Resultados Diez pacientes (5 ninos y 5 ninas con una edad media de 8 anos) fueron diagnosticados de tumor carcinoide. Ocho tenian carcinoides apendiculares de los que cuatro presentaron sintomas de apendicitis aguda. De estos ocho, siete eran menores de 2 cm y estaban localizados en la punta del apendice. El paciente restante presentaba un tumor carcinoide de 3,5 cm en la base apendicular por lo que fue reintervenido para realizar una reseccion ileocecal. Dos pacientes de 4 y 6 anos presentaron un tumor carcinoide bronquial asociado a sintomatologia respiratoria cronica y se llego en ambos al diagnostico acertado tras un ano de seguimiento. Se realizo neumonectomia en un caso y reseccion bronquial con anastomosis traqueobronquial en el otro. Ningun paciente de la serie presento sintomatologia carcinoide. Se realizaron controles bioquimicos, radiologicos y fibrobroncoscopicos en los bronquiales. Todos se encuentran actualmente libres de enfermedad. Conclusiones El tumor carcinoide apendicular no se asocia a sintomatologia caracteristica de apendicitis aguda en la mitad de los pacientes. Encontramos que el sindrome carcinoide asociado al tumor carcinoide es excepcional en la edad pediatrica. En la mayor parte de los tumores carcinoides apendiculares la apendicectomia simple es suficiente con un pronostico excelente. El tumor carcinoide bronquial es diagnosticado generalmente de manera tardia por lo que debemos considerar este diagnostico en pacientes con sintomatologia respiratoria cronica.

Parafollicular C-cells of the thyroid are decreased in patients with congenital diaphragmatic hernia.

BACKGROUND Parathyroid and thymic anomalies related to embryonic neural crest dysfunction have been demonstrated in rats with congenital diaphragmatic hernia (CDH). These rats, like infants with CDH, have conotruncal, facial, and other neurocristal defects. The present study examines whether parafollicular C-cells (CC) of the thyroid, whose embryogenesis is related to that of the parathyroids and thymus, might also be abnormal in babies with CDH. MATERIAL AND METHODS Autopsy sections of the thyroids of 12 babies dead from CDH and of 11 controls were stained with anti-calcitonin antibodies. Calcitonin-stained areas and the histological distribution of CC within the thyroid gland were assessed. Mann-Whitney tests were used for comparison, with p<0.05 considered significant. RESULTS The proportion of stained surface to total thyroid surface was significantly smaller in CDH babies than in controls (0.035±0.030% vs. 0.072±0.052%, p<0.05). A normal central CC location was demonstrated in both groups. CONCLUSIONS Parafollicular thyroid C-cells are deficient in patients with CDH. These findings further support the involvement of neural crest dysregulation in the pathogenesis of CDH and the pertinence of using this experimental model to investigate the human condition. The clinical effects of this anomaly are unknown and probably irrelevant, but they are currently under scrutiny.

Eosinophilic Esophagitis (Europe)

Eosinophilic esophagitis (EE) is a chronic inflammatory condition of the esophagus in which eosinophils densely infiltrate the mucosa causing local effects including fibrosis of the lamina propria. Its mechanism is poorly known, but it involves an allergic background with increased expression of the chemoattractant eotaxin-3 and of IL-5, IL-3, and IL-13. EE causes abdominal pain, vomiting, heartburn, dysphagia, and food bolus impaction. Esophagoscopy shows trachealization, longitudinal furrows, and white exudates. Biopsy demonstrates ≥15 eosinophils per hpf. There is manometric and impedance evidence of dysmotility. Exclusion of food allergens, elemental diets, systemic and topic corticosteroids, and leukotriene inhibitors alleviates the symptoms, but recurrence is frequent. EE does not involve long-term risks of either Barrett’s esophagus or adenocarcinoma.