Hemodynamic failure as an indication to urgent liver transplantation in infants with giant hepatic hemangiomas or vascular malformations--report of four cases.

The aim of this study was to present acute hemodynamic failure as a rare indication for liver transplantation in neonates and infants with liver hemangiomatosis. We report four patients aged one to six months with giant liver hemangiomas, with huge arterio-venous shunting within these malformations. In three, many skin hemangiomas were found. All children developed right ventricular failure. In two, a trial of pharmacological reduction was attempted with corticosteroids and cyclophosphamide. In one patient, the arterio-venous fistulas were embolized without any improvement in hemodynamic status. Two children underwent rescue hepatic artery surgical ligation, which did not prevent heart and then multiorgan failure including liver failure. After unsuccessful conventional therapy, all infants were considered for urgent liver transplantation; in three cases, it was performed with a living-related donor, and in one case with a deceased donor. All patients are alive and well with the follow-up between nine and 37 months after transplantation. Liver transplantation should be considered as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency when conventional therapy is unsuccessful and multiorgan failure develops.

Non-resectable hepatic tumors in children - role of liver transplantation.

BACKGROUND: There is a group of children with primary hepatic tumors which can not be resected by conventional partial liver resection. Total hepatectomy followed by liver transplantation may be the only solution in such cases. Authors reviewed own experience with the liver transplantation for unresectable tumors in children and assessed the possible indications and role of transplantation in these patients. MATERIAL AND METHODS: Liver transplantation was performed in 17 children with unresectable hepatic tumors out of total number of 350 children transplanted. Hepatocarcinoma was present in 8 children, hepatoblastoma in 6 and benign giant hemangioma in 3. There was no other option for the treatment which would lead to the oncological cure of children with malignant tumors. All patients with giant hemangiomas were infants transplanted urgently due to circulatory and then multiorgan failure. RESULTS: Survival within whole group is 75.5% (13 of 17 pts), 3 children died of malignant tumor recurrence, one of other causes. All 3 children with benign tumors are alive and well. Actual follow-up is from 3 months to 7 years. CONCLUSIONS: Liver transplantation should be considered as option in the treatment of all children with unresectable hepatic tumors. With the careful and individual patient selection significant chances for survival can be achieved in this group of patients which would otherwise not survive with the conventional treatment.

[Perioperative antibiotic therapy in newborns according to CDC criteria - preliminary report]. / Antybiotykoterapia okolooperacyjna u noworodków a uwzglednieniem klasyfikacji CDC - doniesienie wstepne.

The aim of the study was elaboration of optimal perioperative antibiotic therapy in neonates according to CDC criteria (Center for Disease Control and Prevention). Classification in 4 groups of infections was carried out in relation to contamination of the surgical wound: clean, clean-contamination, contaminated and dirty. Based on the authors' own experience, CDC criteria were adapted to the contamination of surgical wounds in neonatal surgery. Antibiotic therapy for each of the four groups was also proposed except for the newborns with birth weight below 1500 g. For the neonates with extreme low birth weight (<1500 g) individual treatment based on other risk factors of infection, abnormal bacterial colonization and antibiotics received up to now have to be taken into consideration. Criteria for local and general infection were also defined. We consider that our report concerning rules of antibiotic therapy in neonates requiring surgical treatment will contribute to rational antibiotic administration in this group of patients. Verification of this report should be performed, based on a multicentre study.

[Tumours in newborns and infants up to three months of life. One institution experience]. / Guzy u noworodków i malych niemowlat. Doswiadczenia wlasne.

INTRODUCTION: Newborns and infants up to three months of life are a specific group of population in paediatric oncology due to immaturity of tissues and organs and rarity of neoplastic diseases in this group of patients (pts). There are no strict therapeutic procedures established for these children. THE AIM of our study was to examine distribution of tumours in newborns and infants up to 3 months of age treated in our institution and to present our own experience in the treatment of these patients. MATERIAL AND METHODS: Medical records of 71 pts (37 boys and 34 girls) treated from 1996 to 2004 were reviewed. Distribution of tumour types in newborns and babies from 1 to 3 months of age was analyzed separately. Due to similar growth pattern, response to treatment and it's side effects in newborns and small infants, treatment results were evaluated for the whole group. RESULTS: there were 50 newborns. The most common diagnosis in this group was germ cell tumours (GCT) which constituted 60% of all tumours, amongst them 52% were mature teratomas (MT). The second most common was neuroblastoma (NBL) 22%. There were also 3 cases of soft tissue sarcomas (STS), 2 central nervous system tumours (CNS), 2 retinoblastoma (RB), 2 hepatoblastoma (HB). In the group of 21 babies aged 1-3 months NBL was the commonest (37%) followed by RB, CNS tumours (14% of each) HB and MT (10% of each) and Wilms tumour (WT) and immature teratoma (IT) each 5%. Surgery alone was performed in 48 pts. It concerned pts with MT--28, IT--3 pts, yolk sac tumour (YST)--1 pt and malignant tumours (stage I and II): 8-NBL, 2-CNS tumours, 2 STS, 3-HB, 1-WT. Forty two pts from this group are alive. Six pts died: 2 from surgical complications, 1 from sepsis, 1 of congenital heart defect and 1 from unknown reason at the age of 18 months. It was a patient with severe infantile cerebral palsy. One pt died of disease--relapse of yolk sac tumour, 2 years 4 months after surgery of MT. Eleven pts underwent combined treatment of chemotherapy and surgery: 5 with stage III and IV NBL, 6 with other tumours. One pt with STS at the age of 1 yr 6 months was the tumour bed irradiated after surgery for microscopic tumour residual. Four pts are alive, 4 with NBL and 5 with other tumours. Two pts died from disease: one with NBL and one with IT. Chemotherapy alone was administered to 7 pts in whom local advancement of disease enabled surgery and to pts with RBL. Three out of 7 pts are alive all with RBL. Four pts died: 3 from disease, 1 from infectious complications. Four pts with NBL (2 stage IV and 2 stage IVS) were treated with irradiation to the liver only. Two pts (st. IV) died and 2 (st. IVS) pts are alive. One pt relapsed at age of 2 yrs 3 mths, probably at the primary site which was not visualized at primary diagnosis. One pt, critically ill, died before any treatment. Fifty six out of 70 pts (80%) are alive with a follow up from 1 year to 9 yrs 11 months (median- 4 yrs 4 months). Fourteen pts died (20%), 8 from disease and 6 of other reasons. CONCLUSIONS: 1. GCT and neuroblastoma are the most common tumours in newborns and infants up to 3 months of age. 2. Newborns and small infants with advanced neoplastic disease, similarly to older children can be cured with chemotherapy. 3. Individual approach is warranted in newborns and small infants and treatment should be carried out in specialized centres. 4. All patients who completed treatment of any tumour type should be followed up by a pediatric oncologist.