SummaryBackgroundIn neonatal encephalopathy, the clinical manifestations of injury can only be reliably assessed several years after an intervention, complicating early prognostication and rendering trials of promising neuroprotectants slow and expensive. We aimed to determine the accuracy of thalamic proton magnetic resonance (MR) spectroscopy (MRS) biomarkers as early predictors of the neurodevelopmental abnormalities observed years after neonatal encephalopathy.MethodsWe did a prospective multicentre cohort study across eight neonatal intensive care units in the UK and USA, recruiting term and near-term neonates who received therapeutic hypothermia for neonatal encephalopathy. We excluded infants with life-threatening congenital malformations, syndromic disorders, neurometabolic diseases, or any alternative diagnoses for encephalopathy that were apparent within 6 h of birth. We obtained T1-weighted, T2-weighted, and diffusion-weighted MRI and thalamic proton MRS 4–14 days after birth. Clinical neurodevelopmental tests were done 18–24 months later. The primary outcome was the association between MR biomarkers and an adverse neurodevelopmental outcome, defined as death or moderate or severe disability, measured using a multivariable prognostic model. We used receiver operating characteristic (ROC) curves to examine the prognostic accuracy of the individual biomarkers. This trial is registered with ClinicalTrials.gov, number NCT01309711.FindingsBetween Jan 29, 2013, and June 25, 2016, we recruited 223 infants who all underwent MRI and MRS at a median age of 7 days (IQR 5–10), with 190 (85%) followed up for neurological examination at a median age of 23 months (20–25). Of those followed up, 31 (16%) had moderate or severe disability, including one death. Multiple logistic regression analysis could not be done because thalamic N-acetylaspartate (NAA) concentration alone accurately predicted an adverse neurodevelopmental outcome (area under the curve [AUC] of 0·99 [95% CI 0·94–1·00]; sensitivity 100% [74–100]; specificity 97% [90–100]; n=82); the models would not converge when any additional variable was examined. The AUC (95% CI) of clinical examination at 6 h (n=190) and at discharge (n=167) were 0·72 (0·65–0·78) and 0·60 (0·53–0·68), respectively, and the AUC of abnormal amplitude integrated EEG at 6 h (n=169) was 0·73 (0·65–0·79). On conventional MRI (n=190), cortical injury had an AUC of 0·67 (0·60–0·73), basal ganglia or thalamic injury had an AUC of 0·81 (0·75–0·87), and abnormal signal in the posterior limb of internal capsule (PLIC) had an AUC of 0·82 (0·76–0·87). Fractional anisotropy of PLIC (n=65) had an AUC of 0·82 (0·76–0·87). MRS metabolite peak-area ratios (n=160) of NAA–creatine (<1·29) had an AUC of 0·79 (0·72–0·85), of NAA–choline had an AUC of 0·74 (0·66–0·80), and of lactate–NAA (>0·22) had an AUC of 0·94 (0·89–0·97).InterpretationThalamic proton MRS measures acquired soon after birth in neonatal encephalopathy had the highest accuracy to predict neurdevelopment 2 years later. These methods could...

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Undifferentiated embryonal sarcoma is the uncommon malignant mesenchymal tumor of the liver seen in children and young adults. Radiologic findings reflect the solid to cystic appearances observed in the gross specimens. The discrepancy in the appearances of the mass between the ultrasound scan and computed tomography scan is the hall mark of this tumor. Ultrasonography typically show a large mass which is hyperechoic or isoechoic (solid). Computed tomography shows cystic component of the tumor. This unique radiologic feature will help in the diagnosis of the tumour.

Correspondence to Palaniappan Sashikumar, docsashi@rediffmail.com DESCRIPTIONA 3-day-old female baby born by normal delivery following an uncomplicated pregnancy presented with blisters on her legs. These blisters were present since birth and were diagnosed as staphylococcal skin infection and treated with oral flucloxacillin and the baby was discharged home. She was referred to our hospital on day 3 as the rash was getting worse. The baby was feeding well and showed no signs of sepsis. The baby was screened for sepsis and changed intravenous antibiotics. The inflammatory markers, blood culture and skin swab were normal.The rash was characterised by linear vesicles, pustules and bullous lesion with erythema along the lines of Blaschko (figure 1). The rash followed a particular pattern, which is pathognomic for incontinentia pigmenti.Incontinentia pigmenti is a rare, X-linked, dominantly inherited, disorder of skin pigmentation that often is associated with ocular, dental and central nervous system abnormalities. 1 The incidence is 1 case per 40 000. It usually affects females, as the male fetus does not survive. It results from deletion of the NEMO gene and half of these are new mutations. The skin manifestation is evident soon after birth, as an erythematous eruption with linear vesiculation, followed by a verrucous stage.2 After a few months the verrucous growth drops off and leaves hyperpigmented areas. The management involves multidisciplinary team involvement with regular follow-up.

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