Will CSF Diversion in Patients with Idiopathic Intracranial Hypertension (IIH) Lead to Long-Lasting Shunt Dependency?

Long-term shunt dependency rates in patients treated for IIH with CSF diversion have not been established. We therefore present our experience with 5 children shunted for IIH during the years 1984–2000 with very long-time follow-up. Three out of these patients have experienced late or very late episodes of severe shunt failure during the second or third decade after initial shunt treatment. They were all boys and may not be representative for IIH patients as a whole. Three of them appear, however, to be permanently shunt dependent, indicating that long-term shunt-dependency in children treated for IIH with CSF diversion may be more common than previously expected

Fatal Sunt Failure 40 years after Implantation of a Lumbouretral Shunt in a Hydrocephalic Infant

Long-term results in hydrocephalic children treated with CSF diversion to the ureter have not been reported. We present our experience with seven children shunted with lumbo-uretral shunts during the years 1957-1959. They presented as infants (2-9 months old) with severe head enlargement (46 – 61 cm), all of them with very tense heads and sunset signs. Six of the patients died within two years. The last patient grew up and had been in full-time work for 23 years, when he unexpectedly died from acute shunt failure. He had not been at regular follow-up since his early years and had never been to a CT until his dramatic shunt failure. Although shunt failure may occur after decades with good function of the shunt, the topic is troublesome since very long-time follow-up together with patient awareness may seem appropriate to avoid or reduce the risk of future shunt failure

Choroid plexus tumors in children: Long-term follow-up of consecutive single-institutional series of 59 patients treated over a period of 8 decades (1939-2020)

Objective To present long-term follow-up of a consecutive single-institutional series of patients treated for choroid plexus tumors over 8 decades. Methods From 1939 to 2020, 59 children were treated for choroid plexus tumors. Median age at diagnosis was 1.7 years. Results Gross total resection was achieved in 51 patients (86%). Ten patients (17%) underwent >1 resection. During the first 4 decades of the study (1939–1979), 14 patients with plexus papillomas were treated. Operative mortality was 50%, with 6 of the remaining 7 patients experiencing excellent survival with follow-up periods of 41–81 years. In the last 4 decades (1980–2020), 38 patients had low-grade tumors, and all were alive at the latest follow-up (range, 0.5–39 years). Observed 5-year survival in this subgroup was 100% (n = 30), as was observed 10-year survival (n = 26). One of 7 (14%) patients with atypical choroid plexus papilloma and 3 of 31 patients (10%) with choroid plexus papilloma underwent a second resection owing to recurrent tumor. At last follow-up, 47 patients (80%) were alive; 45 (96%) had a Barthel Index score of 100 and 2 had a Barthel Index score of 50. Today 25 patients are adults (20–59 years old); 17 work full-time, 4 work part-time, and 4 are unable to work. Conclusions Low-grade choroid plexus tumors can be cured with gross total resection alone, with excellent long-term survival and functionality. The vast majority of survivors live independently as adults and work full-time. Recurrences are uncommon (8.7%), appear within the first few years after primary surgery, and can be treated with repeat resections

Outcomes in adulthood after neurosurgical treatment of brain tumors in the first 3 years of life: long-term follow-up of a single consecutive institutional series of 97 patients

Abstract Background Long-term outcome for children who underwent surgery for brain tumors in the first 3 years of life is not well-known. Methods We performed a retrospective study on surgical morbidity, mortality rate, academic achievement, and work participation in children below 3 years of age who underwent primary tumor resection for a brain tumor in the period from 1973 to 1998. Gross motor function and activities of daily life were scored according to the Barthel Index. Long-term survivors were defined as with a survival from primary diagnosis of 20 years or more. Findings Ninety-seven consecutive children were included. No patient was lost to follow-up. Gross total resection was achieved in 67 children during the primary procedure, 25 had subtotal resections, and 5 had only partial resection. The 20-year survival figures for the 46 children with high-grade tumors was 33%, and the corresponding figures for 51 patients treated for low-grade tumors was 82%. Five of the 57 20-year-survivors died 21, 29, 30, 30, and 41 years, respectively, following primary surgery. Fifty of the 52 long-term survivors had a Barthel Index (BI) of 100, while the remaining two had a BI of 40. Twelve patients were long-term survivors after treatment for HG tumors (26%), while 40 of the 51 patients treated for LG tumors (78%) were alive. Thirty-two of the 52 long-term survivors were in full-time work and 29 of them after treatment for LG tumors. Another 10 were in part-time work, while the last 10 individuals had no working capacity. Conclusion Survival is better for patients with low-grade tumors compared with those with high-grade tumors. The functional level of long-term survivors is affected by adjuvant therapy and radiotherapy in particular. Neurosurgical intervention in itself is safe and plausible for pediatric brain tumor patients below 3 years of age. However, there should be a focus on potential late affects, and survivors should be followed by knowledgeable clinical staff for the neoplastic disease as well as for potential side effects. In this consecutive series, a 33% 20-year survival for children treated for HG tumors and 82% for patients with LG tumors was observed. The patients with LG tumors who had been treated with surgical resection without any adjuvant therapy showed a good clinical outcome as adults, and two-thirds of them were in full-time work

Outcome After Treatment of Spinal Ependymoma in Children and Adolescents: Long-Term Follow-up of a Single Consecutive Institutional Series of 33 Patients Treated Over Eight Decades

Background Long-term outcomes for pediatric patients treated for spinal ependymoma are unknown. Methods We performed a retrospective analysis of outcome data from 33 children and young adults (0–22 years) who were operated on for a spinal ependymoma at our institution during the last 8 decades (1938–2019). Results Nineteen patients are alive, with follow-up period up to 60 years. Twelve of them are tumor-free, and 7 are alive with disease. Fourteen patients are dead, 9 of them due to recurrent and/or progressive disease 1–56 years (median: 11 years) after the initial surgery. Four of the deceased patients were treated before 1948, 3 of them with excellent long-term survival for 62–66 years after the initial surgery. Tumor recurrence was observed in half of the patients, both local at the site of the primary tumor resection as well as widespread intraspinal presentations. Recurrences were observed within months but also occurred after up to 20 years after initial treatment. After the implementation of magnetic resonance imaging in 1987, details of recurrent disease became more easily demonstrated. Repeated resections were performed when the symptomatic spinal disease was in progress (n = 11). Furthermore, 2 patients have intracranial tumor manifestations, 1 of them underwent resection of a suprasellar tumor in 1991. Four deceased patients experienced aggressive extraspinal progressive disease requiring multiple surgeries, including pulmonary metastasis in 1 of them. Conclusions Pediatric spinal ependymomas can be treated with favorable results and functional outcome may be good even after more than half a century of follow-up. Nevertheless, unexpected and late recurrences may occur, and life-long follow-up is therefore recommended