Bilateral intracranial calcifications with bilateral facial cutaneous naevus: Sturge Weber syndrome

Sturge Weber syndrome also known as mother spot disease belongs to a group of disorders called phakomatoses (neurocutaneous syndromes involving the central nervous and cutaneous systems). It is a congenital disorder of the vasculature of the meninges, brain, face and eyes. Clinically, it usually presents with seizures and other neurological complications, including mental retardation, contralateral hemiparesis and glaucoma. Its incidence is estimated at one case in 20 000–50 000 persons with equal frequency in boys and girls. We present a case of 18-months-old boy was brought by parents with history of seizures since birth resulting in fall 1 day back. Physical examination showed bilateral port-wine stain on face. CT head examination revealed bilateral intracranial calcification. This is a highly unusual presentation of Sturge Weber syndrome.

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Bilateral large subconjunctival haemorrhages unmasking immune thrombocytopenic purpura during retinopathy of prematurity screening

Although thrombocytopenia is known to be associated with pathogenesis of retinopathy of prematurity (ROP), immune thrombocytopenic purpura (ITP) is rare in infancy and not reported to occur with ROP. A preterm infant with aggressive posterior ROP developed bilateral massive subconjunctival haemorrhage after scleral indentation during screening. On evaluation, the infant was found to have severe ITP. Following intravenous transfusion of platelets and immunoglobulin, platelet counts improved and subconjunctival haemorrhage resolved over time. This case highlights the unusual presentation of ITP and also discusses the association of thrombocytopenia with ROP. Ophthalmologists should get prompt haematological work-up of such occurrences.

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Why in the age of CT scans and MRIs is a brain tumour mistaken for a psychiatric illness?

The first author of this paper operated on two patients with brain tumour, who had been undergoing long-term treatment for depression. In the age of CT scans and MRIs, why are there still cases in which a brain neoplasia is mistaken for a psychiatric condition with consequent serious delays in diagnosis? In this article, we have highlighted what in our experience are three noticeable obstacles in achieving the right diagnosis.

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Oxaliplatin-induced posterior reversible encephalopathy syndrome (PRES)

Posterior reversible encephalopathy syndrome (PRES), first introduced in 1996, is a neurotoxic state characterised by seizures, headache, vision change, paresis, nausea and altered mental status. Risk factors include hypertension, eclampsia/pre-eclampsia, infection/sepsis and cancer chemotherapy. Although exposure to toxic agents is a common occurrence in patients who develop PRES, oxaliplatin has rarely been associated with it, with only 10 cases reported worldwide. We present the case of an oxaliplatin-induced PRES in a 23-year-old male patient who was started on oxaliplatin/capecitabine as adjuvant chemotherapy for anal canal adenocarcinoma. The patient developed symptoms of headache, slurred speech and left-sided facial weakness on the ninth day after the first dose of oxaliplatin that lasted for 6–8 hours. The patient experienced another episode next day with similar symptoms that lasted for 8 hours. Oxaliplatin was withheld and the patient was discharged on capecitabine only. The patient had no new episodes since discharge on follow-up.

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Management of extensive external apical root resorption leading to root perforation

Resorption is both a physiological and pathological process which results in loss of hard tissues of the tooth, i.e , cementum and dentine and the surrounding bone. External resorption is much more common than internal resorption and can occur when tooth is luxated or avulsed. If remained unchecked, resorption can eventually lead to loss of the tooth. Timely management of the affected tooth can slow down the resorptive lesion and increase the prognosis of the survival of the tooth. This case report describes the surgical management of extensive external root resorption leading to perforation of apical one-third of the root area which was managed through root canal along with periapical surgery and bone grafting. A 6-month follow-up showed arrest of the resorptive defect and progressive healing as evident on the cone-beam computed tomography.

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Aspiration of Nasopore nasal packing

We present a case of postoperative Nasopore aspiration in an otherwise fit and well 11-year-old. An endoscopic adenoidectomy had been performed without incident and Nasopore packing placed into each nasal cavity. Immediately after extubation, there was marked hypoxia, tachypnoea and high clinical suspicion of pack aspiration. The patient returned to theatre for emergency rigid bronchoscopy and retrieval of nasal packing.

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Refractory or relapsed aggressive B-cell lymphoma failing (R)-CHOP: An analysis of patients treated on the RICOVER-60 trial

Abstract

Background

The prognosis of elderly patients with aggressive B-NHL after first lymphoma-related treatment failure (TF-L) is not well described.

Methods

We analysed patient characteristics including presence of MYC rearrangements and MYC-expression (IHC) at diagnosis and modalities of salvage therapy and their impact on the prognosis of patients between 61 to 80 years who had been treated on the RICOVER-60 trial.

Results

TF-L occurred in 301 of 1222 (24.6%) patients; 297 patients could be analyzed. Prognosis was extremely poor in patients with primary progressive disease or early relapse (≤12 months) with median survivals of 3.3 and 6.4 months. Survival after TF-L was significantly lower in patients pretreated with R-CHOP compared to CHOP (23.0% vs. 36.4% at 2 years, p = 0.016). In patients with MYC translocation at diagnosis Rituximab reduced the risk of TF-L from 58.8% to 26.3%. Survival after TF-L was significant longer for patients after CHOP without MYC translocations (31.8% vs. 0% at 2 years, p < 0.001) or negative MYC-IHC (41.0% vs. 16.8% at 2 years, p = 0.017) but not after R-CHOP. 224 patients (75.4%) received salvage therapy. Rituximab was part of salvage therapy in 57.4% and improved 2-year survival rate from 20.7% to 46.8% (p < 0.001). The benefit of R was significant after first-line CHOP (2-year-OS 49.6% vs. 19.1%, p < 0.001) as well as after R-CHOP (2-year-OS 33.1% and 22.5%, p = 0.034). For patients pretreated with R-CHOP long-term survival was below 15% regardless of the treatment chosen.

Conclusion

MYC rearrangement and IHC are adverse prognostic factors after TF-L for CHOP treated patients, rituximab as part of first line therapy reduced the effects of MYC-break. Rituximab improves results of any type of salvage therapy, however survival after progression/relapse of aggressive B-cell lymphoma in elderly patients pretreated with (R)-CHOP is poor regardless of treatment chosen.

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