Lipoblastoma of the neck

Lipoblastoma and lipoblastomatosis are benign tumours arising from embryonal fat cells. These rare tumours essentially occur in infancy and early childhood. The males are affected more than females often in the ratio of 3∶1. These tumours usually arise in extremities. The involvement of neck is rare with only 10 cases reported so far in English literature. We herein report lipoblastoma of the neck in a 3 year old girl.     

Radiological–pathological correlation in lipoblastoma and lipoblastomatosis

Background Lipoblastoma and lipoblastomatosis are uncommon benign mesenchymal lesions that predominantly occur in infancy and early childhood.Objective To evaluate the imaging and histological features of lipoblastoma and lipoblastomatosis.Materials and methods Retrospective review of the radiological and pathological findings in children with lipoblastoma and lipoblastomatosis treated at a single centre between 1997 and 2004.Results Eight children (median age 18 months) had undergone imaging and surgery at our institution. An infiltrative growth pattern was identified at imaging in two children with lipoblastomatosis, and a well-defined mass in six children with lipoblastoma. In all patients, imaging showed a lesion composed mostly, but not entirely, of fat. There were no recurrences at follow-up of between 1 and 91 months.Conclusion In infancy and early childhood, the identification of a tumour composed mostly of fat should suggest the diagnosis of lipoblastoma or lipoblastomatosis.     

Giant abdomino-pelvic adipose tumors of childhood

Adipose tumors are rare in pediatric age and are commonly represented by benign forms: lipoma and lipoblastoma/lipoblastomatosis. Generally localized in subcutaneous tissue of the trunk and extremities, they can exceptionally occur as giant intra-peritoneal or pelvic masses. These tumors could reach considerable size prior to diagnosis since they are asymptomatic. The authors report a case series of three giant abdomino-pelvic adipose tumors.     

Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors

Background: Intracranial atypical teratoid/rhabdoid tumors (AT/RT) are rare and extremely aggressive neoplasms seen primarily in childhood. Imaging features are often considered non-specific. However, correct diagnosis of AT/RT is important because these tumors have a markedly different clinical prognosis and require more aggressive therapy.Objective: To determine the imaging features of AT/RT.Materials and methods: We retrospectively analyzed imaging findings in 11 patients with primary intracranial AT/RT presenting over a period of 5 years. CT (n=11), MR (n=7), clinical (n=11) and pathological (n=11) features were evaluated. FISH analysis showing monosomy of chromosome 22 (absence of bcr 22q11 locus) was available for three patients. Immunohistochemical staining for INI-1 (BAF47) was performed on all tumors. Results: There were 11 patients, 6 boys and 5 girls. The age of presentation varied from 1 month to 15 years (average age 3 years 8 months). Six tumors were located in the posterior fossa and five in the supratentorial compartment. The tumors showed a hyperdense solid component (64%) that showed moderate to marked enhancement with contrast medium. On MR imaging, the predominant signal pattern was isointensity on T1-weighted images (57%) and T2 shortening with heterogeneity on T2-weighted images (86%). All tumors were large in size (average 4.2×3.7 cm), and there was a tendency for calcification (36%), hemorrhage (46%), necrosis (46%) and perifocal edema (100%). There was also a high tendency for subarachnoid dissemination, with five patients (46%) demonstrating brain and/or spinal metastasis. At follow-up (n=7), six patients showed local recurrence. At the time of recurrence, all these patients showed extensive leptomeningeal spread of the disease in both intracranial and intraspinal compartments. Conclusion: There are no specific imaging features for intracranial AT/RT. But a high tendency toward large size, a hyperdense solid component on CT scan with calcification, hemorrhage, necrosis and subarachnoid spread suggest that this tumor should be considered in the differential diagnosis of large pediatric intracranial tumors.     

Chest wall lipoblastomatosis in a 2-year-old girl: a case report and literature review

Lipoblastoma is an uncommon tumor of adipose tissue that usually occurs in infancy and early childhood. They predominantly locate in the extremities, but are less frequently found in the head and neck region, trunk and various other locations. The most common sign is a rapidly growing mass. In spite of their potential for local invasion and rapid growth, they are benign tumors and have no distant metastasis. Although more than 180 cases of lipoblastoma distributed over various parts of the body have been reported, only nine cases were located in the thoracic wall. We present the case of a two-year-old girl with lipoblastomatosis of the chest wall that was identified by histologic examination. In this article, the English literature on this disorder is briefly reviewed.     

Hepatic lobectomies in children: experience of a center in the light of changing management of malignant liver tumors

Hepatic resection is the main treatment modality for hepatic tumors in childhood. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood. Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively. Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients. Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7±0.3 cycles of chemotherapy before the operation. Right lobectomy (n=12), left lobectomy (n=5), extended left lobectomy (n=4), and extended right lobectomy (n=3) and right lobectomy with enucleation of two masses from left lobe (n=1) were performed. Intraoperative blood transfusion of 30.7±6.0 ml/kg body weight was necessary. Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n=5), hepatocellular carcinoma (n=4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n=1), hemangioma (n=1), cyst adenoma (n=1), and metastasis of cellular mesoblastic nephroma (n=1). Patients were observed in the intensive care unit for 3.4±0.3 days. Postoperative complications were sepsis (n=1), disseminated intravascular coagulation (n=2), fever (n=3), jaundice (n=3), intraabdominal abscess (n=3), ileus (n=2), and subdiaphragmatic abscess with pleural effusion (n=1). Hepatic lobectomy is a major operation, which is feasible yielding curative results in children. Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control. An erratum to this article can be found at     

Lipoblastoma and lipoblastomatosis in children.

Lipoblastoma and lipoblastomatosis are rare mesenchymal benign tumors of embryonal white fat. They are found primarily in children less than 5 years of age. This tumor presents in two forms: a localized well-circumscribed lesion (lipoblastoma), or a multicentric type (lipoblastomatosis). The authors presents three cases, two of them with a buttock lesion, the last with a paravertebral one. It is recommended a complete but conservative excision of the tumor because there is a natural tendency to involution, although in the first year of life a local reexcision for recurrence is sometimes described.     

Extrarenal rhabdoid tumours outside the central nervous system in infancy

Background  Malignant rhabdoid tumours (RT) are increasingly recognized in young children, probably as a consequence of advances in accurate histological diagnosis rather than a true increase in frequency. Although typically presenting as renal tumours in infancy, extrarenal tumours outside the central nervous system (CNS) in children less than 12 months of age are now well recognized, but previous literature on their imaging features is very limited. Objective  To demonstrate the imaging features of extrarenal RTs outside the CNS. Materials and methods  A retrospective database review was made from 1989 to 2007 of patients diagnosed with extrarenal RT in infancy, i.e. below 12 months of age. Results  There were nine patients (six boys and three girls). The age at presentation varied from 1 to 11 months (average 6 months). Tumours were located in the thorax/mediastinum (n=3), liver (n=3), neck (n=1), shoulder (n=1) and axilla (n=1). The imaging modalities used included US (n=8), CT (n=7) and MRI (n=6). Bone scan was positive in one patient, while metastases at the time of diagnosis occurred in four patients. On MRI the tumours tended to show nonspecific hypointensity on T1-W images and heterogeneous hyperintensity on T2-W images, with heterogeneous enhancement. Conclusion  This is the largest radiological series of extrarenal RTs outside the CNS in infancy. In our series no imaging features were found specific to the diagnosis. A tendency towards large size and mediastinal/paravertebral location were noted. A hypodense solid component on CT and a heterogeneous hyperintensity on T2-W MR images suggest that this tumour should be considered in the routine differential diagnosis of soft-tissue tumours in infancy, in addition to rhabdomyosarcoma.     

The results of surgical treatment of chest wall tumors in childhood

Chest wall tumors (CWT) are rarely seen in childhood and surgery constitutes a complementary part of the therapy. The early and late results of CWT resection and chest wall reconstruction were evaluated retrospectively. The children who underwent chest wall resection for CWT between January 1990 and November 2003 were evaluated retrospectively. Seventeen children (male/female=12/5, mean age: 7.58 years) underwent chest wall resection for CWT. Fifteen patients underwent initial biopsy (tru-cut, n=8 or open biopsy, n=7) and two underwent initial resection. The diagnosis was malignant tumor in 12 (70%) and benign in 5 (30%). They were Ewing’s sarcoma (ES) (n=4), primitive neuroectodermal tumor (PNET) (n=3), Askin’s tumor (n=1), rhabdomyosarcoma (RMS) (n=2), neuroblastoma (n=2), osteochondroma (n=1), aneurysmal bone cyst (n=2) and hamartoma (n=2). Preoperative chemotherapy was given to most patients with malignant tumor. All patients had only local tumor at the time of resection. Thoracotomy was performed in all patients. All tumor tissues with the affected rib/ribs were resected en bloc with the adjacent tissues. The number of resected ribs was 1 (n=6), 2 (n=7) and 3 (n=4). Chest wall defects were repaired primarily (n=8) or with grafts (n=9). Dura (n=4), Neuro-patch (n=3) and Goretex (n=2) were used for closure. Wound infection and pleural fistula occurred in one patient. Patients with benign tumor were free of complaints or complications during follow up. All patients with malignant tumor received postoperative chemotherapy. Local recurrence did not occur in all patients. Five patients developed distant metastasis and two died. Scoliosis was encountered in one patient during follow-up. Since most of the CWT are malignant and not initially suitable for surgical excision, the management includes tissue diagnosis either by tru-cut or open biopsy. Determination of malignant condition should be followed by an intensive chemotherapy. Chest wall resection is planned to control local disease. Chest wall reconstruction may be needed for large defects following resection of CWT. Prosthetic materials can be used safely. Early complications of the surgery are limited. The patients should be closely followed up for late complications such as scoliosis, restrictive pulmonary disease and for the development of metastasis, which is a part of natural course of malignant CWT in children.     

A European phase II study of recombinant human granulocyte colony-stimulating factor (lenograstim) in the treatment of severe chronic neutropenia in children

We conducted a multicentre, open-label prospective study to evaluate the efficacy and tolerability of lenograstim (human-identical glycosylated rHuG-CSF) in the prevention of infectious episodes of severe chronic neutropenia in 19 patients. The median follow up period was 54.6 months. Lenograstim was administered subcutaneously at a starting dosage of 5 μg/kg per day. Neutrophil recovery was achieved in all patients at induction dosages of 5 (n = 15), 10 (n = 2), 15 (n = 1) or 20 μg/kg per day (n = 1) and occurred at a median 7 days after therapy initiation. Alternate-day administration of double-dose lenograstim was feasible in 7 of 17 patients. Lenograstim treatment significantly (P= 0.012) reduced the incidence of treated infections and hospitalization for infection compared with the pre study period and significantly (P<0.001) improved perceived health and disease-related symptoms. One patient discontinued treatment because of adverse events (pustulosis) initially related to lenograstim therapy but not confirmed. One patient withdrew by personal choice and was therefore only treated occasionally. One patient committed suicide after 45 months because of social difficulties. One patient was lost during follow up, and three patients presented with a spontaneous neutrophil recovery after 9, 15 and 27 months, respectively. Moderate and transient side-effects related to lenograstim were observed (thrombocytopenia, n = 2; splenomegaly, n = 2; moderate anaemia (without transfusion requirement), n = 5; bone pain, n = 2; increased of alkaline phosphatase, n = 5). Conclusion Lenograstim produced a sustained neutro phil recovery in patients with severe chronic␣neutropenia, reduced the incidence and severity of infection, and improved quality of life. Received: 22 October 1996 / Accepted: 25 February 1997     

Diagnosis of vascular rings and slings using an interleaved 3D double-slab FISP MR angiography technique

Background: Congenital upper airway obstruction and dysphagia may be caused by vascular rings and slings. Often, invasive and radiation-dependent diagnostic procedures are needed to clarify the diagnosis. Objective: To evaluate the diagnostic utility of high-resolution, free-breathing three-dimensional double-slab fast imaging with steady precession magnetic resonance angiography (3D FISP MRA) in infants and children with respiratory upper airway obstruction and/or dysphagia for detection or exclusion of vascular rings and slings. Materials and methods: Eleven patients (median age 1.3 years; range 5.1 months to 15.8 years) were investigated prospectively with 3D FISP MRA and spin-echo techniques. Additional diagnostic data were available from surgery (n=7), cardiac catheterization (n=5), CT (n=2), barium swallow (n=3) and bronchoscopy/oesophagoscopy (n=4). Results: In one case, diagnosis was missed with low-resolution spin-echo sequences, but high-resolution 3D FISP MRA revealed a double aortic arch. 3D FISP MRA accurately found (n=8) or excluded (n=3) vascular rings or slings in all patients. Using a five-level grading system for 3D FISP MRA image quality (1=non-diagnostic; 5=excellent), the mean grade was 4.3±0.7 with no significant grade difference between two independent observers (P=0.81). Conclusions: High-resolution 3D FISP MRA accurately defined or excluded vascular rings and slings in patients with respiratory symptoms and/or dysphagia. This technique may provide a non-invasive, radiation-free alternative without contrast agents for diagnosis of vascular rings and slings in free-breathing infants and children.Electronic supplementary material is available for this article at     

Hyperechoic lesions in the basal ganglia: An incidental sonographic finding in neonates and infants

Cerebral ultrasound (US) imaging was performed as a screening procedure in approximately 3,600 neonates and infants over a period of 18 months. Hyperechoic lesions in the basal ganglia and thalamic region were detected incidentally in 15 of these patients. Clinical diagnoses included cytomegalovirus infection, asphyxia, rotavirus infection, prematurity, amniotic infection, dysmorphic stigmata, hyperbilirubinemia, congenital heart disease, and diabetic fetopathia. Lesions showed a single punctate (n=5), multiple punctate (n=8), or stripe-like pattern (n=2), with no disease-specific distribution. Computed tomography performed in two of the 15 patients was normal. Lesions resolved within four to seven months in four of eleven cases who had follow-up studies, whereas echogenicities persisted in the remaining seven patients over a period of observation ranging between one to 15 months. Our results indicate that hyperechoic lesions in the basal ganglia and thalamic region may be associated with congenital infections and asphyxia, but could indicate some other unknown pathology. No correlation was found between the morphology of foci and both clinical diagnosis and results of follow-up studies.     

Whole-body magnetic resonance imaging: a useful additional sequence in paediatric imaging

Background: Whole-body MR (WBMR) imaging allows the acquisition of images of the entire body in a matter of minutes. Its use has primarily been in the evaluation of possible metastases in the setting of a known primary tumour. Objective: To document the value of WBMR imaging in ten children in whom this was added as an additional sequence when the primary diagnosis had not yet been made. Materials and methods: Ten children, age range 4 months–15 years (mean 7 years 4 months) had WBMR imaging after initial MR showed an abnormality that raised the possibility of systemic disease. Initial scanning was of the brain (n=1), spine (n=2), retroperitoneum (n=4), hips (n=1), femur (n=1) and wrist (n=1). Results: Abnormalities were detected in eight patients. Two patients had acute lymphoblastic leukaemia, and another had an anaplastic lymphoma, unsuspected prior to the WBMR. Two patients had a previously undiagnosed neuroblastoma with bone marrow metastases. Two patients had Langerhans cell histiocytosis. Another had multiple bone lesions due to cystic angiomatosis. Conclusions: WBMR imaging may be a useful additional sequence in children in whom a systemic and especially a bone marrow abnormality is suspected.Presented at 40th annual meeting of ESPR, Genoa, June 2003     

Chest radiographic features of thoracic metastatic disease in adolescents with colon cancer

This study describes chest radiographic features of thoracic metastatic disease (TMD) in patients referred for colon cancer to a pediatric oncology hospital. The study group was comprised of 9 patients (7 males, 2 females, age 13–19 years) with serial chest radiographs demonstrating TMD from colon cancer. All patients had a chest radiograph performed within the 2 months prior to death. The median interval from diagnosis of colon cancer to appearance of radiographic TMD was 3 months. Four of nine patients had TMD at presentation, eight of nine patients within 2 years of diagnosis. All abnormalities progressed on serial radiographs. The median interval from appearance of radiographic abnormalities to death was 2 months. Radiographic findings included pleural effusions (n=6), lymphadenopathy (n=5), lymphangitic carcinomatosis (n=4), solitary pulmonary nodule (n=2), and lobar atelectasis (n=1). Five patients with pleural effusions initially had right-sided effusions. Radiographic TMD in adolescents with colon cancer usually occurs within 2 years of diagnosis. Once TMD manifests, the prognosis is dismal. Findings include pleural effusions, lymphadenopathy, lymphangitic carcinomatosis, solitary pulmonary nodules, and lobar atelectasis. We suggest that metastatic colon carcinoma should be included in the differential diagnosis for lymphangitic spread of tumor in adolescents.Supported in part by National Cancer Institute Cancer Center Support (Core) grant P30CA21765, CA 23099, and by the American Lebanese Syrian Associated Charities (ALSAC)     

The clinical outcome after inferior vena cava thrombosis in early infancy

The clinical outcome after inferior vena cava thrombosis in early infancy is unknown. We report the clinical long-term follow-up of 12 patients presenting inferior vena cava thrombosis within their first months of life (gestational age: 24–41 weeks; follow-up: 7±3 years). Accompanying renal venous thrombosis occurred in 9, and adrenal bleeding in 4 patients. A central venous catheter was related to the thrombosis in only four patients. Heterozygous factor V Leiden mutation was found in two of the eight infants without central venous catheter. Thrombolysis was performed in seven and effective in three infants; one infant required surgical thrombectomy. In three of eight infants with ineffective or with no therapy, spontaneous recanalization occurred during follow-up. No patient died of the thrombosis. Although no long-term anticoagulatory prophylaxis was performed, none of the children with persisting occlusion (n=5) or stenosis (n=1) of the inferior vena cava developed symptomatic thrombo-embolic complications. However, extensive internal collaterals (n=6), visible varicosis (n=5), pain in the legs (n=3) and persisting renal disease (n=3) with arterial hypertension (n=2) were observed during follow-up. Conclusion Inferior vena cava thrombosis of early infancy frequently persists and may cause considerable long-term morbidity. New strategies for early and long-term therapy are necessary. Received: 6 April 1998 / Accepted in revised form: 24 July 1998     

Cerebral venous sinus thrombosis in infancy and childhood: role of genetic and acquired risk factors of thrombophilia

Over a 3 year period the R506Q mutation in the factor V (FV) FV:Q⁵⁰⁶ gene, FV, factor XII (FXII), prothrombin, protein C, protein S, antithrombin, heparin cofactor II, anticardiolipin antibodies and lipoprotein (a) (Lp(a)) were measured in 32 infants and children with sinus thrombosis. Heterozygous FV:Q⁵⁰⁶ (n= 5), homozygous FV:Q⁵⁰⁶ (n= 2), homozygous FXII deficiency (n= 1), protein C deficiency type I (n= 5), protein C deficiency type II (n=1), antithrombin deficiency type I (n = 1) increased Lp (a) (n=5), activated protein C-resistance without mutation in the FV gene (n= 2), and increased anticardiolipin IgG antibodies (n= 2) were diagnosed in the children investigated. In a further two patients we found combinations of increased Lp(a) with moderate hyperhomocystinaemia and heterozygous plasminogen deficiency with heterozygous FXII deficiency. In addition, increased anticardiolipin IgG antibodies were found in combination with heterozygous FV:Q⁵⁰⁶ (n= 1) and protein C type I deficiency (n= 2) respectively. Out of 32 patients with venous sinus thrombosis, 3 showed additional peripheral venous vascular occlusion. Contributing factors were present in 31 out of 32 patients investigated. Family members of 10 affected children had suffered from venous thrombo-embolism prior to the study. Conclusion Our data suggest that additional contributing factors may promote manifestation of cerebral venous sinus thrombosis in infants and children with an inherited prothrombotic state. Further prospective studies are required to evaluate their potential role as “triggering” agents. Received: 5 August 1997 / Accepted revised form: 15 December 1997     

Clinical,hormonal and imaging findings in 27 children with central diabetes insipidus

Clinical, auxological, biological and neuroradiological characteristics of 27 children with central diabetes insipidus (CDI) were retrospectively analysed. Median age at diagnosis was 8.6 years (range: 0.3–16.1 years). Final aetiologies were postsurgical infundibulo-hypophyseal impairment (n=7), cerebral tumour (n=8), Langerhans cell histiocytosis (n=3), septo-optic dysplasia (n=1), ectrodactyly ectodermal dysplasia clefting syndrome (n=1), and idiopathic (n=7). In the non-postsurgical CDI patients, major cumulative and often subtle presenting manifestations were: polyuria (n=20), polydipsia (n=19), fatigue (n=11), nycturia (n=10), growth retardation (n=9), and headache (n=9). An associated antehypophyseal insufficiency, mainly somatotropic, was documented in 11 children. All patients except one who initially had a cerebral tomography, underwent magnetic resonance imaging revealing the lack of the physiological posterior pituitary hyperintense signal. One third of the idiopathic patients initially had a thickened pituitary stalk. All patients with idiopathic CDI were intensively followed up with 3-monthly physical examination, antehypophyseal evaluation, search for tumour markers, and cerebral MRI every 6 months. In one of them the pituitary stalk had normalized after 4.3 years. In one patient Langerhans cell histiocytosis was diagnosed after 7 months of follow-up, and in another patient a malignant teratoma was found after 2.4 years of follow-up. Conclusion: CDI may be the early sign of an evolving cerebral process. The association of polyuria-polydipsia should incite a complete endocrine evaluation and a meticulous MRI evaluation of the hypothalamo-hypophyseal region. A rigorous clinical and neuroradiologic follow-up is mandatory to rule out an evolving cerebral process and to detect associated antehypophyseal insufficiencies.     

Completion Angiography After Cardiac Surgery for Congenital Heart Disease: Complementing the Intraoperative Imaging Modalities

Residual structural pathology after cardiac surgery for congenital heart disease may complicate postoperative recovery. Completion angiograms obtained in the operating room may facilitate early detection and therapy of residual structural abnormalities. Our objective here is to report our institutional experience performing completion angiograms after cardiopulmonary bypass surgery. Between October 2007 and August 2008, 31 patients underwent completion angiograms after 32 cardiac surgical procedures. The median age was 7.5 months (range, 50 days to 31.2 years) and the median weight was 6.5 kg (range, 3.1–153 kg). Type of procedure, angiographic findings, and therapeutic decision were retrospectively reviewed. Procedures (proc) evaluated through completion angiography included comprehensive stage II or Glenn (n = 13), aortic arch reconstruction/conduit (n = 3), repair/palliation of tetralogy of Fallot or pulmonary atresia with ventricular septal defect (n = 4), PVR or conduit replacement (n = 5), and others (n = 7). Unexpected pathology was identified in 18 of 32 (56.3%) proc, which included left pulmonary artery (LPA) stenoses (n = 15), right pulmonary artery (RPA) stenoses (n = 11), and stenosis impairing coronary blood flow (after DKS; n = 1). In 9 of 32 (28.1%) proc, findings may have led to a change in therapeutic management. This included surgical revision (n = 1), ‘Hybrid’ therapy in the same setting (n = 2: LPA stent, 1; RPA balloon, 1), early catheterization within 3 months (n = 4), and change in medical management (n = 2: change in anticoagulation, 1; early CT, 1). Complications related to completion angiography were seen in only a single procedure (LPA staining). In conclusion, completion angiograms using a dedicated Hybrid cardiac operating suite may aid in early diagnosis and therapy of postsurgical abnormalities. They complement other methods of intraoperative imaging and may reduce the potential need for early surgical or transcatheter reintervention.     

Surgical complications and outcome of paediatric liver transplantation: the Singapore experience

The purpose of this study was to analyse the early and late results of paediatric liver transplantation (LT), with particular reference to complications that required surgical intervention. The charts of all children who underwent LT between 1990 and 2002 were reviewed retrospectively. Results were analysed with a minimum follow up of 9 months. Thirty-five children have undergone 38 LTs; 22 received grafts from their parents, 16 received cadaveric organs and three children had retransplantation. The ages of the children ranged from 12 to 168 months. Biliary atresia was the most frequent indication for transplant (n=27). Twenty-seven children had complications that required surgical or radiological interventional procedures. Vascular complications included hepatic artery thrombosis (n=2), hepatic vein (HV) thrombosis (n=1), and the majority being portal vein thrombosis (n=6). Bile leaks were observed in eight children. Other complications included intestinal perforation (n=2), intra-abdominal abscesses (n=1), wound dehiscence (n=2), post-operative bleed (n=2), intestinal obstruction (n=2), ventral hernia (n=1), and multiple abdominal wound sinuses (n=1). Three children underwent retransplantation, two for hepatic artery thrombosis with multiple episodes of cholangitis and intrahepatic biliomas and the third was done for hepatic vein thrombosis. Patient and graft survival at 1 year is 81.5 and 74.2%, respectively. Paediatric LT is associated with significant morbidity, the main complications being vascular and biliary. The article was presented at the 8th Congress of the Asian Society of Transplanatation (8th CAST) in September 2003 held in Kuala Lumpur, Malaysia.     

CENTRAL NERVOUS SYSTEM (CNS) TUMORS IN THE FIRST SIX MONTHS OF LIFE: The Childrens Hospital Los Angeles Experience, 1979–2005

Background: The authors report the experience at the Children's Hospital Los Angeles with brain tumors diagnosed before 6 months of age, describing the characteristics of the patients, their tumors, treatment strategies, and prognostic factors. Methods: Thirty-three children who were identified between 1979 and 2005 were included. Twelve were female (36%). There were 11 gliomas, 9 choroid plexus tumors, 8 medulloblastomas and supratentorial primitive neuroectodermal tumors (PNET), 2 atypical teratoid/rhabdoid tumors (ATRT), and 1 each of ependymoma, craniopharyngioma, and immature teratoma. Locations of primary tumors included 21 supratentorial (64%) and 7 posterior fossa, and 5 tumors involved both compartments. The treatment strategies included 5 patients with biopsy only, 18 less than gross total resections (<GTRx), and 9 GTRx. Fourteen children (42%) received chemotherapy. Three patients (9%) received irradiation, 1 at initial diagnosis and 2 at relapse. Nine patients (27%) demonstrated metastases, 6 at diagnosis and 3 at relapse. Results: The Kaplan Meier analysis of event-free survival (EFS) and overall survival (OS) for all patients is 21 ± 9% and 35 ± 9% at 5 years. For the glioma patients, the 4-year OS is 48 ± 17%, while the 5-year OS for the medulloblastoma/PNET/ATRT patients is 12 ± 11% (p = .39). The 5-year OS for children achieving a GTRx is 64 ± 21% and for those with <GTRx is 27 ± 10% (p = .08).