Thoracic lymphangiomatosis with massive chylothorax after a tumor biopsy and with disseminated intravenous coagulation--lymphoscintigraphy, an alternative minimally invasive imaging technique: report of a case.

Thoracic lymphangiomatosis in childhood is a rare disorder that frequently has poor response to medical therapy as well as a poor prognosis. This disease is often misdiagnosed, and a definitive diagnosis is usually delayed because of the rarity and slow course of the disease. No previous reports have so far described the usefulness of lymphoscintigraphy in the diagnosis of lymphangiomatosis, although some authors have reported the efficacy of lymphoscintigraphy for evaluating chylothorax. A 6-year-old boy presented with a diffuse mediastinal mass and received an open mediastinal biopsy for a definitive diagnosis, which led to the occurrence of massive chylothorax postoperatively. A diagnosis of lymphangiomatosis was finally made based on the lymphoscintigraphic findings demonstrating an obstruction of the thoracic duct. This report describes a rare case of thoracic lymphangiomatosis diagnosed at autopsy, and suggests that the minimally invasive technique of lymphoscintigraphy should be employed to obtain a definitive diagnosis at an early stage.

A case of subcapsular rupture of liver in a neonate associated with hemophilia A.

We describe an extremely rare case of subcapsular rupture of the liver associated with hemophilia A in a neonate. Although, the neonate was in good condition after birth, at 13 hours, he became pale and his abdomen distended. At 17 hours after birth, an emergency laparotomy was performed. The subcapsular rupture of the left side of the liver was found. The ruptured area was closed with a continuous suture, and additional mattress sutures with pledget provided satisfactory hemostasis. On the second day after the operation, relaparotomy was done because recurrent bleeding was suspected. There was no bleeding from the sutured liver. Oozing was found at the suture line on the abdominal wall. An additional postoperative observation was a decrease of factor VIII less than 1%, and hemophilia A was confirmed.

A new fistulectomy method for the second pharyngeal arch remnants.

PURPOSE: We present a new fistulectomy method for the second pharyngeal arch remnants. MATERIALS AND METHODS: Between 1991 and 2003, 4 patients have been treated with a new fistulectomy method. SURGICAL PROCEDURE: Under general anesthesia with nasotracheal intubation, the neck and mouth are prepared as one operative field. A nylon thread is inserted into the cervical opening. On the oral site of the nylon thread, a small gauze ball is tied and gently pulled from the neck site. At both opening sites of the fistula, a very small incision around the nylon thread is performed. Using the nylon thread as a guide, a fistulectomy is carried out. RESULTS: In all 4 patients, no complications have occurred during and after the fistulectomy. No recurrences were seen during 15 months to 9 years. CONCLUSIONS: This is a simple and useful procedure for the treatment of second pharyngeal arch remnants. It produces an excellent cosmetic result compared with the standard method because only one small incision is necessary.

The fungal DNA examination is useful as a sensitive parameter for the initiation and the quit of antifungal therapy in immunocompromised pediatric patients after surgery.

The purpose of this study is whether the fungal deoxyribonucleic acid (DNA) examination is useful as a sensitive parameter for pediatric surgical patients with mycosis. The eleven episodes of five cases (4 cases; progressive liver disease after biliary atresia operation, 1 case; short bowel syndrome and long term total parenteral nutrition with megacystis microcolon intestinal hypoperistalsis syndrome) with mycosis were divided into two groups according to the difference of therapeutic protocols. The sensitivity of fungal DNA examination, serum Candida antigen level, plasma beta-D glucan level, and blood culture were evaluated at the onset of infection and at the quit of antifungal medication under the protocols respectively. The duration of medication and the medication free interval in two groups were compared. The 6 episodes (3 cases) were diagnosed and treated under the protocol not including fungal DNA examination, while the 5 episodes (2 cases) under the protocol including fungal DNA examination. The occurrence rate was not significant. The sensitivity of fungal DNA examination was complete, but others were not. Using the fungal DNA examination, the duration of medication became significantly short. We conclude that the fungal DNA examination could be a sensitive parameter not only to start but to quit antifungal medication in pediatric patients with mycosis.

Efficacy of n-3 polyunsaturated fatty acid enriched enteral nutrient solution in relieving oxidative stress in patients with severe psychophysiologic disorders.

It is not unusual for patients with severe motor and intellectual disabilities to present with eating and swallowing disorders, and such patients often require long term enteral nutrition. These patients tend to receive all their nutrients in the form of a single nutrient solution that is administered over a long period, and there are concerns about the impact of the composition of these nutrient formulas on patient health. Therefore, it is very important that adequate care be taken when selecting a nutrient formula for patients of this type. In the present study we administered two types of enteral nutrient solutions and examined the effect of the differences in nutrient composition on the degree of oxidative stress experienced by human patients. Subjects were 5 patients (mean age: 27.2 years; male/female ratio: 4:1) with severe motor and intellectual disabilities who were incapable of oral feeding, and who were receiving long term enteral nutrition. The subjects were administered a standard elemental diet for 2 months, and this was followed by administration of an n-3 polyunsaturated fatty acid enriched enteral nutrient solution for three months. Results showed that the n-3 polyunsaturated fatty acid enriched diet improved serum concentrations of trace elements, protein synthesis, and the balance between n-6 polyunsaturated fatty acids and n-3 polyunsaturated fatty acids, which are essential fatty acids. At the same time oxidative stress was reduced, and serum IgE levels declined significantly. Patients with severe psychophysiological disorders often suffer from repeated bouts of pneumonia due to immune system suppression, and there are reports that such patients are subject to increased allergic diathesis. The present results indicated that the in vivo membrane damage induced by oxidative stress may be closely related to the onset of these disorders. The results also suggested that in addition to attaining an adequate understanding of the effect of each of the various nutrients in enteral nutrient formulas, it will also be important to consider not only trace elements but also fatty acid composition in the nutritional management of patients with such disorders.

Clinical significance of plasma diamine oxidase activity in pediatric patients: influence of nutritional therapy and chemotherapy.

The aims of this study were to determine the normal values of plasma diamine oxidase (pDAO) activity in children and to examine the influence of several factors (nutritional management, dietary fiber, and chemotherapy) on pDAO activity. The activity of pDAO was measured in 138 healthy children with minor surgical conditions such as inguinal hernia or undescended testis. In order to define normal values patients were subdivided into 5 groups according to age. Next, changes in pDAO activity under different nutritional conditions were studied in 14 patients with adhesive ileus. The influence of chemotherapeutic drugs on pDAO activity was also studied in 19 neuroblastoma patients. I. The normal values of pDAO activity at year < 1, 1 < or = years < 3, 3 < or = years < 6.6 < or = years < 12, 12 < or = years were 6.65 +/- 1.75, 7.70 +/- 2.29, 6.53 +/- 1.68, 5.85 +/- 1.87, 5.06 +/- 1.84 units/l, respectively. II. The pDAO activities in patients with ileus were 4.73 +/- 1.02 (total parenteral nutrition), 6.84 +/- 1.18 (enteral, nutrition), 7.62 +/- 0.67 (soluble dietary fiber added enteral nutrition) and 8.82 +/- 1.26 units/l (oral feeding). The difference in pDAO activity at enteral or oral feeding vs. total parenteral nutrition was significant (p < .0001). III. The pDAO activity decreased significantly and remained low during the first 4 days after cyclophosphamide administration in neuroblastoma patients. The preadministration of dietary fiber inhibited the influence of cyclophosphamide. Plasma DAO activity was greatly influenced by nutritional management and administration of dietary fiber and/or cyclophosphamide. Plasma DAO activity may be a sensitive marker of intestinal function in children.

The elongation of distal esophageal pouch by mechanical bougienage for a year resulted in a tension-free anastomosis in a patient with long gap esophageal atresia: a case report.

Despite the numerous approaches described for the management of neonates with "long gap" esophageal atresia, controversy still exists as to the preferred method. Delayed primary anastomosis is probably the most frequently adopted practice but often the native esophagus is abandoned. We report a case of a 2.98 kg newborn with pure esophageal atresia. Although the elongation of the distal esophageal pouch by mechanical bougienage was initiated at 1 year and 8 months, a successful tension-free anastomosis with minimum dissection of the lower esophagus was performed at 2 years and 7 months. Her postoperative quality of life has been quite excellent. This report emphasizes that a tension-free anastomosis without operating on the lower esophagus and stomach is essential for the treatment of long-gap esophageal atresia.