New-Onset Vision Impairment in Children: Magnetic Resonance Imaging Findings by Age Groups.

INTRODUCTION: Non-traumatic visual impairment is rare in the pediatric population, but early diagnosis and treatment of the cause is crucial to prevent long-term consequences affecting children's neurocognitive development. The authors aim to determine the most common causes of non-traumatic visual impairment in pediatric patients according to age groups by magnetic resonance imaging (MRI). METHODS: Images of patients who underwent contrast-enhanced cranial and orbital MRI for new-onset visual impairment between June 2019 and June 2022 were retrospectively reviewed. MRI findings were categorized as tumors, idiopathic intracranial hypertension, demyelinating disorders, infections, isolated optic neuritis, and others. The patients were grouped according to age as preschoolers, schoolchildren, and adolescents. Demographic features of patients and MRI findings were collected and compared among age groups. RESULTS: One hundred seventeen of the 238 patients had pathologic MRI findings. The most common pathologies were tumors (26.4%), idiopathic intracranial hypertension (24.7%), demyelinating disorders (18.8%), infections (11.1%), and isolated optic neuritis (7.6%). Tumors (69.2%) in preschool children, idiopathic intracranial hypertension (36.3%) in schoolchildren, and demyelinating disorders (32.7%) in adolescents were the most common cause of vision impairment by age group. CONCLUSION: Children with acute vision impairment could have severe pathologies. Tumors in preschool children, idiopathic intracranial hypertension in schoolchildren, and demyelinating disorders in adolescents were the most common causes of new-onset vision impairment detected with MRI. Because of the difficulty of performing optimal ophthalmologic and neurologic examinations, especially in young children, cranial and orbital MRI should be considered to detect life-threatening pathologies.

Corpus callosum thickness: A predictive factor for the first drug efficiency of self-limited epilepsy with centrotemporal spikes (selects)?

OBJECTIVE: To investigate the existence of a possible linkage between the thickness of corpus callosum (CC) regions and the first antiepileptic drug response in patients with Selects. MATERIALS AND METHODS: CC thickness of 68 patients with Selects and 42 healthy controls between 4 and 12 years of age were measured using brain magnetic resonance imaging (MRI). Clinical and EEG features of newly diagnosed Selects patients were recorded. Patients were divided into two groups: good-response (patients without seizures within 24 weeks) and poor-response (patients with ≥ 1 seizure within 24 weeks). Thickness of CC was compared between patients (good-response and poor-response groups).and healthy controls. RESULTS: The thicknesses of genu and isthmus were significantly reduced in the Selects group than healthy controls. Isthmus and splenium were significantly thinner in poor responders than those in the good-response group (p = 0.005 and p < 0.001, respectively). The total number of seizures was negatively correlated with the thickness of the body, isthmus, and splenium (p < 0.001). There was no significant difference in CC thickness of the children with and without electrical status epilepticus in sleep (ESES). The thickness of the isthmus and splenium were significantly thinner in patients receiving ≥ 2 antiepileptic drugs (p = 0.002 and p = 0.001, respectively). CONCLUSIONS: Our study highlights the notable differences in areas of CC in Selects patients. These changes may help uncover the underlying cause of seizure recurrence and antiepileptic drug (AED) response. Different thinner parts of CC may be a protective mechanism to prevent seizure spread to other brain regions. CC thickness can be used as a new radiologic biomarker for predicting first AED response and seizure recurrence in Selects patients.

IgG4-related disease and ANCA positive vasculitis in childhood: a case-based review.

Autoimmune pancreatitis (AIP) type 1 is an IgG4-related disease (IgG4-RD), characterized by inflammatory pseudotumors and histologically by dense lymphoplasmacytic infiltrates rich in IgG4 positive plasma cells, storiform fibrosis, and obliterative phlebitis. Although quite rare, IgG4-RD was found to be associated with medium or small vessel vasculitides. A new overlap syndrome between IgG4-RD and ANCA-associated vasculitis (AAV) has recently been described in the adult population. Here we present a 16-year-old adolescent girl admitted with abdominal pain, episcleritis, palpable purpura, salivary gland enlargement, and bloody diarrhea. Laboratory investigations revealed findings of glomerulonephritis. Abdominal imaging surprisingly revealed a focal mass in the pancreatic tail, while the c-ANCA level was found to be quite high as well as serum IgG4 level. Biopsy of the pancreatic mass showed lymphoplasmacytic IgG4 positive cells infiltrating the pancreas with storiform fibrosis compatible with IgG4-related AIP. The renal biopsy that was done simultaneously showed necrotizing granulomatous vasculitis indicating AAV. Renal biopsy showed IgG4 positive plasma cells very rarely by immunohistochemical examination, which does not indicate any significance for IgG4-RD. Our diagnosis was IgG4-related AIP and AAV overlap syndrome, which has not been reported in the pediatric populations yet. IgG4-RD should be investigated in patients with ANCA-associated vasculitis who shows atypical organ involvement. We searched the Pubmed/Medline and Google Scholar databases to identify clinical findings, treatment, and outcome of the patients with IgG4-related AIP and AAV.

Nutcracker syndrome.

The nutcracker phenomenon [left renal vein (LRV) entrapment syndrome] refers to compression of the LRV most commonly between abdominal aorta and superior mesenteric artery. Term of nutcracker syndrome (NCS) is used for patients with clinical symptoms associated with nutcracker anatomy. LRV entrapment divided into 2 types: anterior and posterior. Posterior and right-sided NCSs are rare conditions. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Symptoms include hematuria, orthostatic proteinuria, flank pain, abdominal pain, varicocele, dyspareunia, dysmenorrhea, fatigue and orthostatic intolerance. Existence of the clinical features constitutes a basis for the diagnosis. Several imaging methods such as Doppler ultrasonography, computed tomography angiography, magnetic resonance angiography and retrograde venography are used to diagnose NCS. The management of NCS depends upon the clinical presentation and the severity of the LRV hypertension. The treatment options are ranged from surveillance to nephrectomy. Treatment decision should be based on the severity of symptoms and their expected reversibility with regard to patient's age and the stage of the syndrome.

From a branchial fistula to a branchiootorenal syndrome: a case report and review of the literature.

Branchial abnormalities constitute 32% to 45% of all neck pathologies in children. They may be a part of branchiootorenal (BOR) syndrome, which is characterized by branchial arch anomalies, preauricular pits, hearing impairment, and renal malformations. Typically, the management of a branchial fistula does not necessarily require an extensive diagnostic workup. However, in patients with a branchial fistula associated with external ear anomalies on physical examination and/or a history of hearing loss and a similar history and findings in other family members, an additional workup should be performed to eliminate the possibility of BOR syndrome. The aim of this report is to make pediatric surgeons aware of the BOR syndrome in patients presenting with branchial arch anomalies.