The frequency and management of CBD stones in a Pediatric Series

Figure 2: Gadolinium-enhanced axial T1-weighted MRI showing pachymeningitis with atrophy of the left optic nerve.

A set of laboratory tests was performed to including autoimmune antibodies: anti-nuclear antibodies, antibodies against extractable nuclear antigens, anti-double- and single-stranded DNA; anti-neutrophil cytoplasmic antibodies, anti-cyclic citrullinated peptide antibodies, anti-MOG, and anti-aquaporin-4 (AQP4), all proved negative.

The blood levels of vitamin B12, B9, thyroid-stimulating hormone, and the angiotensin-converting enzyme (ACE) were normal.

Lumbar puncture revealed an opening pressure of 20 cmH2o, elevated protein of 1.3g/L, normal glucose levels and normal WCC in the cerebrospinal fluid (CSF), with a sterile culture.

In March 2022, the patient presents a progressive onset of visual loss in the right eye. Ophthalmic examination showed a visual acuity at 2/10 without papillitis. A brain MRI found pachymeningitis with atrophy of the left optic nerve (Figure 2).

A CT scan of the chest showed the presence of a nodular shadow with some cavitation involving the middle lobe of the left lung in favor of vascular malformation. Salivary gland biopsy showed sialadenitis. A blood patch for suspicion of CSF hypotension was performed as a therapeutic test but without clinical benefit.

In May 2022, a brain and whole-body FDG-PET was performed and showed the presence of moderately hypermetabolic bilateral hilar lymph nodes, and the progression of left frontal pachymeningitis associated with moderate hypermetabolism affecting bilateral internal temporal regions.

The patient was started on intravenous methylprednisolone (1 g/day) for 3 days, without ocular improvement and with poor tolerance of his diabetes

The patient was re-hospitalized for a follow-up evaluation in January 2023.

A whole-body Positron emission tomography (PET) imaging with F-18-labelled 2-fluoro 2-deoxy-D glucose (F-18 FDG) showed a hypermetabolic nodule of the walls of the trachea, the right main bronchus, the subcarinal, the pulmonary hiles, and the nasal septum. A cerebral F-18 FDG PET showed intense hypermetabolism of the right optic nerve, associated with marked encephalic hypermetabolism, predominantly in the temporal and supratentorial regions (Figure 3 and 4).

Laboratory test showed Erythrocyte sedimentation rate (ESR) of 65 mm/h (normal range: 0-20 mm/h), C-reactive protein (CRP) of 45 mg/L (normal range: <10 mg/L), and a white blood cell count of 14,000 cells/µL (normal range: 4,000-11,000 cells/µL) with neutrophil predominance. Lumbar puncture revealed an elevated opening pressure, elevated protein, and normal glucose levels in the cerebrospinal fluid (CSF), with a sterile culture.

Autoimmune and infectious tests were re-performed, and all proved negative including the Anti-neutrophil cytoplasmic antibodies directed to proteinase3 (c-ANCA) but not the Perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA) which was positive.

So, the patient was diagnosed with granulomatosis with angiitis.