Risk factors for renal injury in patients with meningomyelocele

PATIENTS AND METHODS: Thirty operated patients of myelodysplasia were clinically evaluated for the age at presentation, the extent of lesion and neurological deficit. Urological assessment was done with urine cultures, serum creatinine, radiological (ultrasound of kidney, ureters and bladder, voiding cystourethrogram) and urodynamic (water cystometry) parameters. An objective scoring for bladder (Galloway, et al.) was applied. Dimercapto-succinic acid (DMSA) scan was done in all the patients for evidence of renal scars. The results of above investigations were correlated with presence or absence of renal scars (renal injury) on DMSA scan. None of the patients had received any prior bladder care. RESULTS: Twenty one patients had no renal scars and 9 patients had evidence of renal scarring. Patients with renal scars were older at presentation, they had greater degree of hydroureteronephrosis (P < or = 0.001) and vesicoureteric reflux (P < or = 0.005). The incidence of high leak pressures (>25 cm of water, P < or = 0.05), unacceptable bladder volumes (maximum cystometric capacity < 60% for age, P < or = 0.005) and high risk Galloway's score (> 5, P < or = 0.05) was high in patients with associated renal scarring as compared to their nonscarred counterparts. Three of these patients had serum creatinine >1 mg/dl (P < or = 0.005). The incidence of urinary complaints and positive urine cultures was also higher in these patients (NS). CONCLUSION: Increasing age, evidence of hydroureteronephrosis and vesicoureteric reflux, high leak pressures, low bladder volume and high combined Galloway score (>5) define a high risk bladder in our population and predispose to renal injury in patients of myelodysplasia. Early referral for bladder risk assessment and management of all myelodysplasia patients is recommended.     

The problem of treatment abandonment in children from developing countries with cancer

BACKGROUND: There is an inequality gap between the outcome of childhood cancer in resource, rich and limited countries. Abandonment of treatment is one of the reasons for this. PROCEDURE: We searched the medical literature for evidence on abandonment, its causes, and any preventative interventions. RESULTS: Abandonment is a very real problem all across the developing world. Cancers associated with poorer prognosis seem to have higher abandonment rates. It is also related to the socio-economic and educational status of parents, travel time to treatment centers, and affordable, locally available treatment. CONCLUSIONS: Twinning between institutions, which includes several preventative interventions, has clearly been shown to work.     

Familial calcific band-shaped keratopathy: report of two new cases with early recurrence

We report two siblings with the rare entity of familial calcific band-shaped keratopathy (BSK). Detailed ophthalmic and systemic investigations failed to reveal any underlying causative pathology. Topical disodium ethylenediamine-tetraacetate (EDTA) was applied for 30 min to all four eyes. In addition the right eye of the younger sibling required a superficial keratectomy. An improvement in corneal clarity was seen in the immediate postoperative period in both siblings. Histopathology of the keratectomy specimen revealed linear extracellular sub-epithelial granular calcium deposits. However, an early recurrence was noted in all four eyes at four weeks postoperatively. We report the second instance in the English literature of this entity. Band-shaped keratopathy presenting without an obvious etiology merits a complete systemic and ophthalmic workup. Patients with familial idiopathic BSK could be cases with poor prognosis for treatment with EDTA due to an early recurrence of the disease.     

In vivo microscopy of Best's Vitelliform Macular Dystrophy: optical coherence tomography study of combined stage III and IV lesions

A 35-year-old man presented with decreased vision in both eyes. Fundus biomicroscopy of the right eye revealed a pseudohypopyon along with an area of hyperpigmentation and scarring at the macula (combined stage III and IVb Best's Vitelliform Macular Dystrophy [BVMD]). The left eye showed the presence of a pseudohypopyon along with macular atrophy (combined stage III and IVa). On optical coherence tomography both eyes had a neurosensory retinal detachment with an optically clear subretinal zone, an accumulation of material between neurosensory retina and the retinal pigment epithelium corresponding to the pseudohypopyon and subfoveal photoreceptor atrophy. We document on optical coherence tomography the transition of BVMD from stage III to IV (in the form of the combined stage). These new findings may be considered unique for this stage of BVMD, and would help in a further understanding of the disease as it passes through its various stages.     

Congenital infiltrating lipomatosis of the face with exophytic temporomandibular joint ankylosis: a case report and review of the literature

Congenital infiltrating lipomatosis of the face (CIL-F) is a rare lipomatous lesion with diffuse fatty infiltration of tissues and hyperplasia of underlying bone. We report clinical and CT findings in an unusual case of CIL-F presenting with progressive hemifacial asymmetry, manifesting as severely restricted mouth opening owing to exophytic temporomandibular joint ankylosis. The role of imaging in diagnosis is presented with a review of the literature. Differential diagnosis of CIL-F and its exclusion as a cause of hemifacial hyperplasia are also discussed.     

Andersson lesion: are we misdiagnosing it? A retrospective study of clinico-radiological features and outcome of short segment fixation

This study reviews the presentation, etiology, imaging characteristics and reasons for missed diagnosis of Andersson lesion (AL) and analyzes the surgical results of short segment fixation in the thoracolumbar region. This is a retrospective single center study. Fourteen patients (15 lesions) who were operated for AL were analyzed. The study was designed in two parts. The first part consisted of analysis of clinical and radiological features (MRI and radiographs) to highlight, whether definitive characteristics exist. The second part consisted of analysis of outcome of short segment fixation as measured by VAS, Frankel score, AsQoL index, and union, with assessment of complications. The follow-up was 42.33 ± 19.29 months (13 males and 1 female) with a mean age of 61.13 ± 19.74 years. There was predisposing trauma in five patients. There was a delay in presentation of the patients by 5.86 ± 2.50 months. There was misdiagnosis in all the cases, at primary orthopedic level (ten cases were put on anti-tuberculous treatment due to its MRI resemblance to infection) and all but one case at radiologist level. Radiographs and MRI had characteristic features in all cases, and MRI could detect posterior element affection in 14 lesions as against only 8 posterior lesions detected in radiographs. In all patients, there was a patient’s delay and/or physician’s delay to arrive at a diagnosis. Spinal fusion was seen in all the cases. Outcome measures of VAS, Frankel score, and AsQoL index showed significant improvement (P < 0.002). No major complications occurred. There is a lack of awareness of AL leading to misdiagnosis. Definite clinico-radiological features do exist in AL and short segment fixation is effective.     

Paroxysmal complete atrioventricular block in pediatric heart transplant recipients following cardiac catheterization: A case series

Late‐onset paroxysmal AVB has been described as a rare complication after HT and has been associated with AR or CAV. We describe 4 pediatric HT recipients who developed paroxysmal AVB hours after routine cardiac catheterization in the absence of AR, CAV, or underlying conduction system disease. Four pediatric HT recipients who were >1 year post‐transplant had episodes of paroxysmal AVB hours after surveillance cardiac catheterization with EMB. Telemetry demonstrated high‐grade block, ranging from 2:1 AVB to complete AVB without ventricular escape for several seconds. None of the patients had significant AR or rapidly progressive CAV. Supplemental testing did not reveal underlying conduction system disease. Three of the 4 patients received permanent pacemakers, although subsequent interrogations showed minimal ventricular pacing. These pediatric HT recipients had paroxysmal AVB hours after cardiac catheterization in the absence of significant AR, CAV, or underlying conduction system disease. Subsequent pacemaker interrogations showed minimal ventricular pacing, suggesting these were isolated episodes. These cases suggest that mechanisms in addition to AR and CAV may cause paroxysmal AVB in pediatric HT recipients, warranting further investigation.