| Summary: | Objective: To retrospectively review our institutional experience with the
treatment of paediatric craniopharygiomas and assess the evolution in
management and influence on patient outcomes.
Patients and Methods: A retrospective review from January 1995 to
December 2015 of children age <14 treated at a single institution. Data
collected included admission clinical features, endocrine function, surgery
performed, surgical outcome, intracystic therapy and radiotherapy. Long-term
functional outcome was calculated considering hormonal dependence, level of
independence and schooling.
Results: There were 41 patients with a mean age of 84.2 months: 57% were
female. Primary surgical resection was performed in 36 patients: 80.5% had
subtotal resection, 11% had gross total resection and the rest had biopsy
only. Of surgical approaches, 60,7% had pterional craniotomy and 39,2%
supraorbital keyhole craniotomy. No surgical mortalities occurred but 2
patients had new post-operative neurological deficits. Stereotactic placement
of intracystic catheters transitioned to endoscopic. Intracystic treatments
transitioned from Yttrium (1) to Bleomycin (6) to Interferon Alpha (6).
Radiotherapy was given in 30 patients, median dose 54Gy. Final Wen
functional outcome was 21,8% Class I, 32% Class II and 46% Class III. There
were no early deaths in the series but 5 patients died more than 6 years after
diagnosis, mostly due to endocrine crises from poor chronic care.
Conclusion: The findings reflect a multidisciplinary team approach consisting
of maximal safe resection with radiotherapy, intracystic agents and endocrine
support. For a cohort limited to young children, our results are similar in
number and outcomes to other published series. Mortality remains low but
lifelong dependence on endocrine replacement is a significant contributor to
long-term morbidity and mortality. This has important implications for patients
referred from large distances and where primary and secondary follow up
care is poor.
|
|---|
