Long-term sequelae and quality of life after childhood-onset craniopharyngioma: Results of a Spanish multicenter study.

PURPOSE: We aim to describe the characteristics of patients with childhood-onset craniopharyngioma and to analyze factors that impair quality of life (QoL) in this population. METHODS: Multicenter national study including patients treated between 2008 and 2022, from 2 to 25 years of age diagnosed with craniopharyngioma. QoL was assessed once during patient's follow-up by age-adapted versions of Pediatric Quality of Life Inventory (PedsQL) questionnaire. RESULTS: Sixty-six patients were included. Median age at diagnosis was 5 years (interquartile range [IQR]: 3-8), while median follow-up was 7.4 years (IQR: 2.8-9.7). Most craniopharyngioma were suprasellar (93.9%), and 59.7% had hypothalamic involvement (HI). All patients underwent surgery, 44.4% received radiotherapy, and 23.6% intracystic therapy. Most frequent long-term complications were visual deficit (72.7%) and endocrine impairment (94.5%). Patients exhibited hypothyroidism requiring hormone replacement (92.4%), hypocortisolism (80.3%), diabetes insipidus (86.4%), and/or growth hormone therapy (50%). When parents evaluated QoL, PedsQL median score was 53.8 points out of 100 (IQR: 41-71.6). Higher scores were noted when patients assessed their own QoL (median score 64.8 [IQR: 57.3-81.8]), observing statistically significant differences (p = .019). QoL was impaired by repeated surgeries (r = -.44; p = .014), HI (median score 51.5 [IQR: 39-63.8] vs. 76.4 [59-84.8]; p = .001), radiotherapy (median score 51.9 [IQR: 38.1-61.3] vs. 63.8 [IQR: 49-82.5]; p = .02) and longer follow-up (r = -.3; p = .01). CONCLUSION: In our study, most patients had significant comorbidities and low overall QoL scores, which was mainly affected by repeated surgery, radiation, and hypothalamic involvement. This reflects the need for further research and intensified studies of systemic therapy/alternate strategies to broaden the standard-of-care options, so that treatment-related sequalae can be avoided.

Clinical Manifestations and Treatment of Vogt-Koyanagi-Harada Disease during Pregnancy and after Birth: A Case Report.

PURPOSE: To describe the clinical manifestations of Vogt-Koyanagi-Harada (VKH) disease during pregnancy and after birth and the therapeutic challenge of treating patients with this condition. METHODS: We describe the clinical manifestations of this disease, as well as the diagnostic tests and treatments performed. RESULTS: The patient was referred for evaluation due to a persistent headache. Examination revealed bilateral anterior uveitis, papillitis and yellowish-white choroidal lesions in both eyes. A tentative diagnosis of VKH disease was made. A multimodal imaging study was performed at the time of presentation and throughout the disease course. The patient was initially treated with intravenous corticosteroids, and subsequently, oral corticosteroids and cyclosporine were administered. Clinical manifestations increased in severity after childbirth. CONCLUSIONS: The clinical course of VKH disease can be modified by pregnancy. While clinical manifestations during gestation may be mild, these may be exacerbated after birth. Treatment with corticosteroids and cyclosporine can be effective.

Ocular Involvement in a Patient with Intravascular Large B-Cell Lymphoma: A Diagnostic Challenge. Clinical Case Report and Literature Review.

PURPOSE: To describe the ophthalmic findings and diagnosis of a case of intravascular large B-cell lymphoma. METHODS: Clinical case observational report. CLINICAL CASE: A Spanish 51-year-old man referred to our hospital with a diagnosis of panuveitis. The patient presented with blurred vision, photophobia, fever, and weight loss. Ocular examination revealed anterior uveitis, vitritis, and multiple round and oval creamy spots on the posterior pole. Fluorescein angiography, optical coherence tomography (OCT), and angio-OCT were used for the ocular examination. The diagnosis of lymphoma was formulated on the basis of a random normal skin biopsy, which showed significant CD20 cellularity within the vessels and extensive CD3 expression. CONCLUSIONS: Intravascular lymphoma is a rare form of extranodal diffuse large B-cell lymphoma, often with delayed diagnosis because of the nonspecific symptoms. Hence, random skin biopsy could be useful in the diagnosis.

Clostridium Septicum Endogenous Endophthalmitis as the Initial Manifestation of Colorectal Carcinoma: Clinical Case Report and Literature Review.

PURPOSE: To describe the presentation and clinical course of a case of endogenous endophthalmitis caused by Clostridium septicum, an anaerobic and gram positive bacteria. METHODS: Observational report of a clinical case presented in Spain. CLINICAL CASE: A 61-year-old male patient was referred to our hospital with a diagnosis of endophthalmitis of his left eye since ten days prior to presentation, and a history of poor response to medical treatment. Evisceration was performed, and C. septicum was isolated from a sample of the vitreous humor. Fourteen days after surgery, the patient returned with orbital cellulitis, and exenteration was performed. Forty-two days after initial presentation, colonoscopy revealed an adenocarcinoma of the proximal colon. Surgical resection of the tumor was performed, and clinical recovery was achieved. CONCLUSIONS: iSystemic C. septicum infection without a traumatic cause has been associated with malignancy, although an initial presentation of endophthalmitis has rarely been reported. In cases of clostridial endogenous endophthalmitis, investigation for colorectal carcinoma is indicated.