[Peri-operation treatment for patients with hemophilia A in children with intracranial malignant tumor: a report of 2 cases].

Hemophilia A is aninherited bleeding disorder, lack of coagulation factor VIII (FVIII), and if combined with intracranial malignant tumor, the operation risk is very high. Department of Pediatric Surgery in Peking University First Hospital used coagulation factor replacement therapy, succeeded in the operation of 2 cases of intracranial malignant tumor with hemophilia A in children, with no abnormal bleeding events. The establishment of the multi subject cooperation group before operation, good preoperative preparation, enough alternative factors, and close postoperative monitoring, are the key to the successful treatment.

[Children primary intestinal inflammatory myofibroblastic tumor: a case report].

Children intestinal inflammatory myofibroblastic tumor (IMT) is rare, and its clinical differential is very difficult to the malignant tumor. Its diagnosis depends on pathology, and operation is the most effective therapy method. We preset here a case of a 5-year-old girl who was admitted to the hospital for intestinal obstruction. A malignant tumor in the intestinal wall showed on the enhanced CT. Exploratory laparotory found the tumor and then it was resected. Postoperative pathology and immunohistochemistry proved that it was an intestinal IMT. Three months' follow-up found no tumor recurrence and metastasis.

Laparoscopic cholecystocholangiography for diagnosis of prolonged jaundice in infants, experience of 144 cases.

PURPOSE: Cholangiography is often crucial for establishing the definitive diagnosis in infants with prolonged jaundice. Here, we describe our protocol of using the two-hole laparoscopic technique and discuss its benefits. METHODS: 144 consecutive patients with suspected biliary atresia were included in this retrospective study. A 5-mm umbilical port is introduced for a 30 degrees laparoscope. An additional 5-mm trocar was inserted at right subcostal incision. A liver biopsy was performed first if needed. The fundus of gallbladder was then exteriorized through the incision of the working port after pneumoperitoneum was released and a catheter is inserted into the gallbladder for cholangiography. RESULTS: The average duration of operation was 34 min (range 20-55 min). Laparoscopic cholangiography failed in 21 cases (14.6%) where atrophic gallbladder was found and BA was confirmed by subsequent laparotomy. For the remaining 123 cases, biliary atresia were diagnosed in 88 (71.5%), biliary hypoplasia in 14 (11.4%), and cholestasis in 21 (17.1%), respectively. There was no bleeding or any other complications intraoperatively. CONCLUSION: The technique of laparoscopic cholecystocholangiography is simple, safe and efficient. It can provide an accurate diagnosis. Furthermore, for patients without biliary atresia, unnecessary laparotomy can be avoided.

Hypothalamic Hamartoma, Gray Matter Heterotopia, and Polymicrogyria in a Boy: Case Report and Literature Review.

BACKGROUND: Hypothalamic hamartomas (HHs) are rare, and it is even rarer when combined with gray matter heterotopia (GMH) and polymicrogyria (PMG). CASE DESCRIPTION: A 5-year-old boy with HH, GMH, and PMG was retrospectively evaluated. The clinical data, including the symptoms, examinations, diagnosis, and treatment, were collected. The patient had a chief complaint of gelastic seizures and intellectual deficiency. Brain magnetic resonance imaging showed HH, paraventricular nodular heterotopia, and PMG. Video electroencephalographs were normal. The patient underwent resection of the HH via transcallosal transseptal interforniceal approach. Seizures disappeared immediately after complete resection of HH, and the intellectual development improved. CONCLUSIONS: In this extremely rare case, resection of the HH eliminated the symptoms. Nonetheless, we still need to be cautious about the possible epilepsy that may be caused by GMH and PMG.