Progress in pediatric surgery最新文献

Nesidioblastosis is a life-threatening form of hypoglycemia that starts during the neonatal period in most cases and is caused by hyperinsulinism. Its diagnostic criteria are an extremely high demand for carbohydrates (more than 15 g/kg/day), an inadequately high plasma insulin level, and an inhibited production of ketone bodies. This acute, life-threatening hypoglycemia requires immediate intensive-care treatment. The most important aim of continuous therapy is the prevention of irreversible brain damage. This cannot be reliably avoided by conservative treatment (increased carbohydrate supply, diazoxide administration). Therefore, surgical treatment consisting in subtotal pancreatectomy is becoming increasingly important. The reduction of hormone-producing tissue resolves hyperinsulinism and apparently enables the onset of physiological regulatory mechanisms. Surgical strategy and results in 12 children who underwent surgery for nesidioblastosis are described.

Magnetic resonance imaging of the trachea was performed in 21 children with congenital or acquired narrowing of the trachea or main bronchi. Diagnosis included aortic arch anomalies, innominate artery compression, pulmonary artery compression and tracheomalacia. All patients were examined after bronchoscopy. The demonstration of the trachea and the surrounding tissue and vessels on MR images enables the cause of tracheal compression and the degree and location of collapse to be evaluated. MRI is a modality well suited to characterizing tracheal narrowing without employing ionizing radiation or intravenous contrast medium. All MRI examinations were carried out with the patient under general anaesthesia so as not to risk pulmonary deterioration during sedation. In the cases presented MRI is the diagnostic step of choice after tracheobronchoscopy and broadens the diagnostic potential in extrinsic tracheal or bronchial stenosis in paediatric patients.

The authors report on the complex follow-up of 60 patients operated on for hyperthyroidism in childhood, on average 13.7 years after surgery. In 16.7% of the patients manifest hypothyroidism, in 45% subclinical hypothyroidism was found; 30% of the patients were euthyroid, and manifest hyperthyroidism recurred in 8.3%. Autonomous adenomas were enucleated in two children and three young adults. Severe disorders in thyroid function developed especially after the surgery of diffuse toxic goiters accompanied by ophthalmopathy. The disorders of humoral and cellular immunity were detected most frequently in recurrent manifest hyperthyroidism. There was no case where ophthalmopathy progressed after the operation. In the offspring of the operated patients the incidence of hyperthyroidism was not increased in childhood. The authors call attention to the importance of postoperative follow-up and hormone treatment.

Hypoglycemia with hyperinsulinism persisted in a newborn weighing 6410 g despite treatment with high doses of diazoxide and glucagon, as well as infusions of glucose and somatostatin. A subtotal pancreatectomy was performed after nesidioblastosis had been diagnosed on the basis of the laboratory findings. Due to the persistence of therapy-resistant hypoglycemia, a total pancreatectomy preserving the duodenum and the bile duct was done 6 weeks later. With insulin and pancreatic enzyme substitution the now 6-year, 9-month-old child has shown normal, age, appropriate development.

From 1970 to 1989, 121 children with mediastinal masses of various sorts were seen in the Department of Pathology, Royal Children's Hospital, Melbourne. The series is considered representative of the true incidence of these conditions in the state of Victoria, which had an average paediatric population during the time of this series of 900,000 children. The commonest cause of a mediastinal mass was NHL (36 cases). This was followed by HD (24 cases), then neuroblastoma and ganglioneuroma (16 and 9 cases respectively), duplication cysts (10 cases), teratomas (7 cases), neurofibroma (4 cases) and lymphangioma (3 cases). A great variety of rare conditions made up the remainder of the series and included mediastinal abscess, thymic cyst, pericardial cyst, accessory lobe of lung, plasma cell granuloma, fibromatosis, paravertebral Ewing's tumour, carcinoid tumour and neurofibrosarcoma. Presentation of the children with NHL was often acute with respiratory distress, while the child with HD was usually older and symptoms were more often systemic than local. The surgeon's role in diagnosis of these most frequently encountered mediastinal masses can be crucial and biopsy when indicated must be carried out with great care to produce material that is adequate for diagnosis and for the performance of cell marker studies and chromosome analysis. Neuroblastoma (NBL) and ganglioneuroma (GN) together were the third largest group. Children with neuroblastoma were usually young; 15 of the 18 cases were less than 2 years old. One-third of the infants with neuroblastoma presented with paraplegia and one-third with respiratory symptoms including wheeze, stridor and respiratory difficulty. Three children had Horner's syndrome. Prognosis of children with thoracic neuroblastoma is very good and contrasts with the poor outlook for those with abdominal neuroblastoma. Stage at presentation is probably the most important single prognostic variable. Ganglioneuroma presents at a later age than neuroblastoma and symptoms may be present for a long time or may be completely absent. Catecholamines, usually raised in neuroblastoma, are mostly normal in ganglioneuroma. Duplication cysts were the next most frequent group. Symptoms can often be acute and life threatening, although in three of our ten cases the cyst was an incidental finding on chest X-ray. However, only three of our patients had a normal respiratory examination. Teratomas were usually large and more often benign than malignant. Excision is the mandatory treatment and is usually curative. Although teratomas in young infants are often cellular and composed of many immature tissue types, their behaviour is benign.(ABSTRACT TRUNCATED AT 400 WORDS)

Although thoracic afflictions may be traced to the prehistoric age, successful thoracic surgery was a development of the late nineteenth and early twentieth centuries. From 1543 to 1661, Vesalius, Servetus, Harvey and Malpighi established the dynamic anatomy of the pulmonary system. Boyle, Hooke, Black and Lavoisier elaborated upon the respiratory gases between 1660 and 1794. Although the drainage of empyema had been known since Hippocratic times, the underwater seal drainage bottle to prevent pneumothorax was not invented until 1872. At the turn of the century, Sauerbruch delayed the development of thoracic surgery for 30 years by opposing the use of positive pressure ventilation, which had not only been known to the ancients but also in clinical practice since the mid-1800s. Once positive pressure ventilation was established, routine successful thoracic surgery still had to await the development of safe blood transfusion by Landsteiner (1900, 1940) and the availability of antibiotics discovered by Chain, Florey and Waksman in the 1940s. Techniques and instrumentation were then developed for the safe and routine surgery of specific thoracic organs.

Between 1974 and 1985, 12 children and adolescents aged 10-18 years were operated on for immunogenic hyperthyroidism resistant to medical treatment. Bilateral, subtotal strumectomies were carried out, leaving a remnant of 2-3 g of thyroid tissue in place. There were no immediate postoperative complications. Hyperthyroidism recurred in two instances. During the same time, 26 children and adolescents up to 18 years of age underwent surgery for autonomous adenoma. Enucleation is the method of choice in adenoma, but is not always possible.

From 1971 to 1988, 45 girls aged 1 week to 17 years were treated for a total of 46 solid and cystic tumors of the ovaries. Pathohistological examination revealed epithelial tumors in eight cases, a tumor originating from the ovarian stroma in one case, germinal tumors in 17 cases, 15 functional ovarian cysts, and five paraovarian cysts. The stroma tumor and four of the 17 germinal tumors were malignant. Surgical treatment for solid tumors consisted generally of a unilateral salpingo-oophorectomy, but in operations for cystic tumors as well, vital ovarian tissue could only rarely be preserved. Functional ovarian cysts were excised if they were larger than 5 cm. Subsequent to excision of malignant tumors, chemotherapy with cisplatin, vincristine and bleomycin was performed. On follow-up, all patients with benign lesions were well. One of the girls with malignancies died and another is undergoing chemotherapy for tumor recurrence in the contralateral ovary.

Parathyroid surgery in children is uncommon. Spontaneously occurring cases of hyperparathyroidism are almost always due to single-gland disease: however, on exploration all four parathyroid glands should be identified. Most of the other instances in which the surgeon needs to perform a parathyroidectomy on an infant or a child will be situations were multiple-gland disease is the rule rather than the exception. Therefore, the surgeon must have in his mind a well developed logical approach to the management of children with parathyroid disorders on the basis of multiple glandular disease. We believe that the technique of parathyroid autotransplantation very satisfactorily addresses the surgical needs of children with familial hyperparathyroid states, including the multiple endocrine neoplasias. We believe that it is mandatory treatment in patients presenting with neonatal primary hyperparathyroidism and is also the procedure of choice in children with secondary and tertiary hyperparathyroidism. The workup and diagnosis of parathyroid disorders should be familiar to the surgeon who undertakes neck exploration on children, and the entity of familial hypocalciuric hypercalcemia should be looked for, as these patients have a strong likelihood of not benefiting from parathyroidectomy.

A series of 18 children suffering from persistent idiopathic neonatal hypoglycemia (PINH) is reported. Medical and surgical managements are described in detail. All patients subjected to surgery had failed medical treatment. These patients were divided into two groups: 1) 85% pancreatectomy leaving the uncinate process in situ, and 2) 95% pancreatectomy leaving a small rim of pancreatic tissue along the duodenum and the common bile duct. The spleen was preserved in all cases. Two out of 5 children of group 1 required further resection of the pancreas for persistent hypoglycemia and were converted to 95% pancreatectomy. Since 1981 95% pancreatectomy was exclusively employed. Only one patient required insulin for 3 weeks postoperatively. Histopathology and immunohistochemistry revealed islet cell dysplasia and islet cell nuclear hypertrophy in the majority of cases, 35% of the patients had focal adenomatosis. Better control of hypoglycemia is achieved by primary 95% pancreatectomy and, thus, 95% pancreatectomy is recommended as the initial procedure in the treatment of PINH.