Ectopic recurrence of craniopharyngioma in the posterior fossa: Case report and review of the literature / Recurrencia ectópica de craneofaringioma en fosa posterior: presentación de casos y revisión de la literatura

Craniopharyngiomas are benign epithelial tumors which may very occasionally recur in ectopic locations. We present two cases of ectopic recurrence, both in the posterior fossa, and provide a review of the literature with basic statistics. Two patients admitted to our institution with posterior fossa lesions underwent gross total resection. Pathological studies showed adamantinomatous craniopharyngiomas (ACP). Both patients had a prior history of suprasellar tumor surgery. We also reviewed the related data and undertook a basic statistical analysis. We found 67 cases of ectopic recurrent craniopharyngioma (including the present cases): 51 cases were adamantinomatous (76%), 6 papillary (9%) and 10 unknown (15%). 18 cases occurred in the posterior fossa, all of them diagnosed as the ACP subtype. The intervals until recurrence were 15.15 years for posterior fossa recurrences and 5.75 years for supratentorial cases. Student t test showed significant differences in time to recurrence (p 0.002). Gross total resection was performed in 53 cases (79%), subtotal resection + radiotherapy in 3 (5%) and 11 (16%) cases were treated with other options. Ectopic recurrence is a rare but possible event. Those in the posterior fossa may appear later than in the supratentorial space. ACP is likely to be the most common subtype in these cases, possibly due to its more aggressive behavior compared to the papillary subtype. Long term follow-up should be performed to detect ectopic recurrences. Ectopic recurrences are often surgically accessible and gross total resection should be achieved. (AU)

Los craneofaringiomas son tumores epiteliales benignos que pueden recurrir ocasionalmente en localizaciones ectópicas. Presentamos 2 casos de recurrencias ectópicas, ambos en fosa posterior, y realizamos una revisión de la literatura con análisis estadístico básico. Dos pacientes ingresaron en nuestro servicio con lesiones en fosa posterior en quienes se indicó resección quirúrgica. El diagnóstico histopatológico fue de craneofaringioma adamantinomatoso en ambos casos. Ambos pacientes fueron intervenidos años atrás por un tumor supraselar. Se hallaron 67 casos descritos de recurrencia ectópica de craneofaringioma (incluyendo el presente estudio): 51 fueron adamantinomatoso (76%), 6 papilar (9%) y 10 desconocidos (15%). Dieciocho casos ocurrieron en la fosa posterior, todos con diagnóstico de craneofaringioma adamantinomatoso. El intervalo hasta la recurrencia fue de 15,15 años para aquellas en la fosa posterior y de 5,75 años para recurrencias supratentoriales. El test de Student mostró diferencias estadísticamente significativas en el tiempo de recurrencia (p 0,002). En 53 casos se obtuvo una resección completa (79%), resección subtotal + radioterapia en 3 (5%) y 11 casos se usaron otras modalidades de tratamiento (16%). La recurrencia ectópica es un evento raro, pero posible. Aquellas en la fosa posterior pueden aparecer más tarde que a nivel supratentorial. Los craneofaringiomas adamantinomatosos son los subtipos más probables en esos casos, posiblemente debido a su comportamiento más agresivo comparado con los papilares. Un seguimiento a largo plazo debe llevarse a cabo para detectar dichas recurrencias ectópicas, ya que estas suelen ser quirúrgicamente accesibles, y debe obtenerse una resección completa. (AU)

Ectopic recurrence of craniopharyngioma in the posterior fossa: Case report and review of the literature.

Craniopharyngiomas are benign epithelial tumors which may very occasionally recur in ectopic locations. We present two cases of ectopic recurrence, both in the posterior fossa, and provide a review of the literature with basic statistics. Two patients admitted to our institution with posterior fossa lesions underwent gross total resection. Pathological studies showed adamantinomatous craniopharyngiomas (ACP). Both patients had a prior history of suprasellar tumor surgery. We also reviewed the related data and undertook a basic statistical analysis. We found 67 cases of ectopic recurrent craniopharyngioma (including the present cases): 51 cases were adamantinomatous (76%), 6 papillary (9%) and 10 unknown (15%). 18 cases occurred in the posterior fossa, all of them diagnosed as the ACP subtype. The intervals until recurrence were 15.15 years for posterior fossa recurrences and 5.75 years for supratentorial cases. Student t test showed significant differences in time to recurrence (p 0.002). Gross total resection was performed in 53 cases (79%), subtotal resection+radiotherapy in 3 (5%) and 11 (16%) cases were treated with other options. Ectopic recurrence is a rare but possible event. Those in the posterior fossa may appear later than in the supratentorial space. ACP is likely to be the most common subtype in these cases, possibly due to its more aggressive behavior compared to the papillary subtype. Long term follow-up should be performed to detect ectopic recurrences. Ectopic recurrences are often surgically accessible and gross total resection should be achieved.

Conventional posterior cranial vault expansion: indications and results-review of the literature.

In the late 1980s, craniofacial surgery units reported suboptimal cosmetic results, cranial volume restriction, and intracranial hypertension after anterior cranial vault remodeling in bilateral coronal synostosis associated with severe brachyturricephaly. A possible explanation was a severe associated growth restriction of the posterior calvaria with radiological synostosis at the lambda sutures. "Conventional" or "fixed" posterior cranial vault expansion techniques were developed to address these limitations, sometimes as the first surgical step in a two-staged protocol of total calvarial reconstruction, combined with suboccipital decompression in cases of symptomatic cerebellar tonsillar herniation or, more easily, to resolve the characteristic occipital flattening of lambdoid synostosis. Various surgical approaches have been described; however, the indications for and timing of surgical treatment and postoperative evaluation of results still remain controversial. Although more invasive, conventional posterior cranial vault expansion has proven to be safe and offers a remodeled and protective bony vault immediately after surgery, but the underlying cranial base malformation remained untreated, with implications in the postoperative growth of the facial skeleton. Overcorrection, rigid stabilization, and grafting are also concerns to be addressed.

Quality of life in school-age children with shunt implantation due to neonatal posthemorrhagic hydrocephalus.

PURPOSE: To assess the functional outcome in school-age children shunted in the neonatal period due to post-hemorrhagic hydrocephalus (PHH), using the HOQ-Spanish version (HOQ-sv), and to analyze predictors of quality of life in this group. METHODS: A cross-sectional study was performed between 2015 and 2018. Parents of pediatric patients with PHH attending our neurosurgery outpatient clinic were invited to complete the HOQ-sv and to enroll in the study. Clinical variables regarding the patients' neonatal course and surgical outcome were recorded. A descriptive analysis was done, and independent variables related to the HOQ scores were studied in univariate and multivariate analyses with regression trees. RESULTS: The study comprised a total of 52 patients. The mean overall HOQ score was 0.67 (on a scale from 0 [worse] to 1 [best]). The quality of life for the PHH children at school age was related to perinatal factors (gestational age at birth, time until shunt surgery, length of hospitalization at the time of shunt implantation, and comorbidity), shunt complications (symptomatic overdrainage, number of shunt revisions, and shunt revisions related to infection during the first year after treatment), and clinical background (seizures, spasticity, Gross Motor Function Classification System level or visual impairment). CONCLUSION: HOQ dimension scores in school-age children shunted due to PHH in our center were similar to those of referral centers for other etiologies of pediatric hydrocephalus. Future goals should be the prevention of complications related to worse outcomes at the time of diagnosis and to try to improve shunt performance later.