Canada has come a long way since Dr. C. Henry Kempe first described battered-child syndrome in 19... more Canada has come a long way since Dr. C. Henry Kempe first described battered-child syndrome in 1962. The year 1999 was crucial in Canada’s battle against shaken baby syndrome/abusive head trauma (SBS/AHT), when the first national conference on the topic was held in Saskatoon. This was followed by the issuance of a national statement and multidisciplinary guidelines, recently updated in 2020. Incidence of AHT in Canada is similar to that found in population-based studies from Switzerland and New Zealand. The mainstay of prevention of AHT in Canada is education of parents and caregivers with respect to their response to infant crying. Population-based data for global incidence of AHT are lacking, largely because of social and cultural differences contributing to poor understanding of AHT as a medico-legal entity. India faces a distinct challenge in the battle against female feticide and infanticide.
BackgroundThere are few data in the literature on the characteristics and natural history of intr... more BackgroundThere are few data in the literature on the characteristics and natural history of intracranial arterial infundibular dilatations in children.MethodsAn institutional review board-approved retrospective review was performed of infundibula reported on MR angiography in patients <18 years of age at our tertiary pediatric institute from 1998 to 2016. Clinical data (age, sex, diagnosis, other vascular variants/pathologies) were recorded and images assessed for vessel of origin, infundibulum size and exact location. Ratios of infundibulum:parent artery were assessed at diagnosis and last follow-up. Temporal evolution to aneurysm was evaluated.ResultsWe found 60 intracranial infundibula in 60 children (male:female=27:33; mean age 9.7±5.2 years, range 2–18 years,). Family history of aneurysms was present in 2/60 (3.3%). Syndromic association was found in 14/60 (23.3%), most frequently sickle cell disease (4/14=28.6%). Mean infundibulum size was 2.2±0.5 mm, with mean ratio to pa...
Ultrasound assistance improves success rates and reduces adverse outcomes of lumbar punctures (LP... more Ultrasound assistance improves success rates and reduces adverse outcomes of lumbar punctures (LPs) among adult patients in the emergency room and the operating room, but has not been evaluated in pediatric patients with cancer. Our objectives were (1) to determine whether pediatric oncologists could perform ultrasound‐assisted LPs following a structured teaching curriculum, and (2) to determine the feasibility of recruiting pediatric cancer patients to a clinical trial of this procedure.
Magnetic resonance (MR) imaging is emerging as one of the most important tools in identifying the... more Magnetic resonance (MR) imaging is emerging as one of the most important tools in identifying the etiology of neonatal encephalopathy as well as in predicting long-term outcomes. This makes it imperative to have a broader understanding of normal myelination of the neonatal brain on MR imaging and to be familiar with the spectrum of imaging features in ischemic and non-ischemic neonatal encephalopathy. Hypoxic ischemic injury (HIE) is one of the most common causes of neonatal encephalopathy and imaging appearances are influenced by factors such as the stage of maturation of the neonatal brain and severity as well as duration of ischemic insult. Other common causes of neonatal encephalopathy include infectious diseases, congenital disorders and inborn errors of metabolism.
Cancers arising from germline DNA mismatch-repair or polymerase-proofreading deficiencies (MMRD a... more Cancers arising from germline DNA mismatch-repair or polymerase-proofreading deficiencies (MMRD and PPD) in children harbour the highest mutational and microsatellite insertion/deletion (MS-indel) burden in humans and are lethal due to inherent resistance to chemo-irradiation. Although immune checkpoint inhibitors (ICI) have failed to benefit children in previous studies, we hypothesized that hypermutation caused by MMRD and PPD will improve outcomes following ICI in these patients. ICI treatment of 45 progressive/recurrent tumours from 38 patients revealed durable objective responses in the majority, culminating in 3-year survival of 41.4%. High mutation burden predicted response for ultra-hypermutant cancers (>100 mutations/Mb) enriched for combined MMRD+PPD, while MS-indels predicted response in MMRD tumours with lower mutation burden (10-100 mutations/Mb). Further, both mechanisms were associated with increased immune infiltration even in “immunologically-cold” tumours such a...
Purpose To evaluate the safety and efficacy of long vascular sheaths for transfemoral neuroendova... more Purpose To evaluate the safety and efficacy of long vascular sheaths for transfemoral neuroendovascular procedures in children. Materials and Methods A retrospective evaluation of transfemoral neuroendovascular procedures in children <18 years, using long sheaths was undertaken analyzing procedure type, fluoroscopic times, technical success, access site and systemic complications. Twenty-seven consecutive procedures were included over a 2-year period. Mean age was 8.4 years (standard deviation [SD] 6.3) (range 17.0 months–16.3 years). Results Patients were 44% female and mean weight was 35.0 kg (SD 22.8) (range 9.8–72.2 kg). A third of the procedures were performed in ≤15 kg children. The most common procedure was for embolization (n=13, 48.1%) and the most common indication was dual microcatheter technique (52%). The most common device used was the 5 Fr Cook Shuttle sheath. Mean fluoroscopy time was 61.9 minutes (SD 43.1). Of these procedures, 93% were technically successful. Fe...
Pediatric patients present with a wide variety of head and neck pathologies. Although some lesion... more Pediatric patients present with a wide variety of head and neck pathologies. Although some lesions can be diagnosed on clinical grounds, there are many others that require imaging for diagnosis. Imaging plays an important role in distinguishing among the potential diagnoses, as often, history and clinical assessment is limited in these young patients. Beyond diagnosis, imaging has a crucial role in evaluating the location and extent of a lesion for possible intervention. Understanding the imaging appearance of the myriad pathologies which are commonly encountered and their potential complications facilitates optimal management. In this chapter, we review the various imaging modalities available, benefits and limitations of each modality, and discuss the ten subsets of pediatric head and neck pathologies.
BACKGROUND Given the expanding evidence of clinico-radiological differences between moyamoya dise... more BACKGROUND Given the expanding evidence of clinico-radiological differences between moyamoya disease (MMD) and moyamoya syndrome (MMS), we compared the clinical and radiographic features of childhood MMD and MMS to identify predictors of ischemic event recurrence. METHODS We reviewed a pediatric moyamoya cohort followed between 2003 and 2019. Clinical and radiographic characteristics at diagnosis and follow-up were abstracted. Comparisons between MMD and MMS as well as between MMD and two MMS subgroups (neurofibromatosis [MMS-NF1] and sickle cell disease [MMS-SCD]) were performed. RESULTS A total of 111 patients were identified. Patients with MMD presented commonly with transient ischemic attacks (TIAs) (35 % MMD versus 13% MMS-NF1 versus 9.5% MMS-SCD; P = 0.047). Symptomatic stroke presentation (MMD 37% versus MMS-NF1 4% versus 33%; P = 0.0147) and bilateral disease at diagnosis (MMD 73% versus MMS-NF1 22 % versus MMS-SCD 67%; P = 0.0002) were uncommon in MMS-NF1. TIA recurrence was common in MMD (hazard ratio 2.86; P = 0.001). The ivy sign was absent on neuroimaging in a majority of patients with MMS-SCD (MMD 67% versus MMS-NF1 52% versus MMS-SCD 9.5%; P = 0.0002). Predictors of poor motor outcome included early age at diagnosis (odds ratio [OR] 8.45; P = 0.0014), symptomatic stroke presentation (OR 6.6; P = 0.019), and advanced Suzuki stage (OR 3.59; P = 0.019). CONCLUSIONS Moyamoya exhibits different phenotypes based on underlying etiologies. Frequent TIAs is a common phenotype of MMD and symptomatic stroke presentation a common feature of MMD and MMS-SCD, whereas unilateral disease and low infarct burden are common in MMS-NF1. In addition, absence of ivy sign is a common phenotype in MMS-SCD.
Antithrombotic therapy is currently recommended for stroke prevention in pediatric cardioembolic ... more Antithrombotic therapy is currently recommended for stroke prevention in pediatric cardioembolic stroke where the recurrence risk is high; however, safety concerns remain. The primary objective of this study was to evaluate clinical and radiographic predictors of hemorrhagic transformation and stroke recurrence in children with cardiac disease to ascertain the safety and failure rates for secondary stroke prevention.
BackgroundFailure to appreciate deep venous drainage pathways is a major cause of severe complica... more BackgroundFailure to appreciate deep venous drainage pathways is a major cause of severe complications in the endovascular treatment of vein of Galen aneurysmal malformations (VOGMs).ObjectiveTo report deep venous drainage patterns in patients with VOGM, emphasizing the internal cerebral veins, and to describe the challenges in evaluating these.MethodsPatients with VOGM presenting to our institute between 2000 and 2018 were retrospectively analyzed. Patients with complete and good quality imaging datasets were included in the study. Three neuroradiologists with expertise in the subject independently analyzed the deep venous drainage patterns on multi-sequence MRI and digital subtraction angiography. Follow-up imaging studies were analyzed for alterations in deep venous drainage patterns that occurred following endovascular treatment. Descriptive statistics were used to report findings.ResultsTwenty-three patients had optimal quality MRI imaging and 25 had optimal quality DSA imaging...
Journal of Stroke and Cerebrovascular Diseases, 2020
PURPOSE The purpose of this study was to determine the additional information provided by Magneti... more PURPOSE The purpose of this study was to determine the additional information provided by Magnetic Resonance Angiography (MRA) in pediatric sickle cell disease (SCD) patients with normal Transcranial Doppler imaging (TCDI) examinations. METHODS This cohort study included all pediatric SCD patients over an 18-year period who had no history of stroke and had normal TCDI examinations and subsequently underwent MRA. Routine TCDI inclusive of time-averaged mean of maximum velocities (TAMMV) were assesses and compared with tortuosity on MRA and silent infarct on MRI. RESULTS 86 children (52.3% female; mean age 8.7 ± 3.5years) were included. There were 77 patients (89.5%) with Hb-SS disease and 9(10.4%) with HB-S beta-thalassemia. All patients had normal TAMMV (<170 cm/s) on TCDI. 76/86 (88.3%) patients also had one or more velocity readings <70 cm/s, albeit none in the middle cerebral arteries. Posterior cerebral arteries had the lowest velocities, <70 cm/s in 51.7% (right) and 60.9% (left). Silent MRI infarcts were seen in 27/86 (31.4%) patients. No new lesions were identified on follow-up MRI. Although mild vascular tortuosity was appreciated in 31/86 (36.0%) of the patients, there were no steno-occlusive lesions in the circle of Willis. CONCLUSIONS TCDI and MRA are routinely performed for non-invasively evaluating intracranial vascular abnormalities in children with SCD. In SCD children with no history of TIA or stroke, MRA following a normal TCDI examination is unlikely to show vascular abnormality. However, almost a third of these patients show silent infarcts on MRI, unassociated with MRA changes.
Canada has come a long way since Dr. C. Henry Kempe first described battered-child syndrome in 19... more Canada has come a long way since Dr. C. Henry Kempe first described battered-child syndrome in 1962. The year 1999 was crucial in Canada’s battle against shaken baby syndrome/abusive head trauma (SBS/AHT), when the first national conference on the topic was held in Saskatoon. This was followed by the issuance of a national statement and multidisciplinary guidelines, recently updated in 2020. Incidence of AHT in Canada is similar to that found in population-based studies from Switzerland and New Zealand. The mainstay of prevention of AHT in Canada is education of parents and caregivers with respect to their response to infant crying. Population-based data for global incidence of AHT are lacking, largely because of social and cultural differences contributing to poor understanding of AHT as a medico-legal entity. India faces a distinct challenge in the battle against female feticide and infanticide.
BackgroundThere are few data in the literature on the characteristics and natural history of intr... more BackgroundThere are few data in the literature on the characteristics and natural history of intracranial arterial infundibular dilatations in children.MethodsAn institutional review board-approved retrospective review was performed of infundibula reported on MR angiography in patients <18 years of age at our tertiary pediatric institute from 1998 to 2016. Clinical data (age, sex, diagnosis, other vascular variants/pathologies) were recorded and images assessed for vessel of origin, infundibulum size and exact location. Ratios of infundibulum:parent artery were assessed at diagnosis and last follow-up. Temporal evolution to aneurysm was evaluated.ResultsWe found 60 intracranial infundibula in 60 children (male:female=27:33; mean age 9.7±5.2 years, range 2–18 years,). Family history of aneurysms was present in 2/60 (3.3%). Syndromic association was found in 14/60 (23.3%), most frequently sickle cell disease (4/14=28.6%). Mean infundibulum size was 2.2±0.5 mm, with mean ratio to pa...
Ultrasound assistance improves success rates and reduces adverse outcomes of lumbar punctures (LP... more Ultrasound assistance improves success rates and reduces adverse outcomes of lumbar punctures (LPs) among adult patients in the emergency room and the operating room, but has not been evaluated in pediatric patients with cancer. Our objectives were (1) to determine whether pediatric oncologists could perform ultrasound‐assisted LPs following a structured teaching curriculum, and (2) to determine the feasibility of recruiting pediatric cancer patients to a clinical trial of this procedure.
Magnetic resonance (MR) imaging is emerging as one of the most important tools in identifying the... more Magnetic resonance (MR) imaging is emerging as one of the most important tools in identifying the etiology of neonatal encephalopathy as well as in predicting long-term outcomes. This makes it imperative to have a broader understanding of normal myelination of the neonatal brain on MR imaging and to be familiar with the spectrum of imaging features in ischemic and non-ischemic neonatal encephalopathy. Hypoxic ischemic injury (HIE) is one of the most common causes of neonatal encephalopathy and imaging appearances are influenced by factors such as the stage of maturation of the neonatal brain and severity as well as duration of ischemic insult. Other common causes of neonatal encephalopathy include infectious diseases, congenital disorders and inborn errors of metabolism.
Cancers arising from germline DNA mismatch-repair or polymerase-proofreading deficiencies (MMRD a... more Cancers arising from germline DNA mismatch-repair or polymerase-proofreading deficiencies (MMRD and PPD) in children harbour the highest mutational and microsatellite insertion/deletion (MS-indel) burden in humans and are lethal due to inherent resistance to chemo-irradiation. Although immune checkpoint inhibitors (ICI) have failed to benefit children in previous studies, we hypothesized that hypermutation caused by MMRD and PPD will improve outcomes following ICI in these patients. ICI treatment of 45 progressive/recurrent tumours from 38 patients revealed durable objective responses in the majority, culminating in 3-year survival of 41.4%. High mutation burden predicted response for ultra-hypermutant cancers (>100 mutations/Mb) enriched for combined MMRD+PPD, while MS-indels predicted response in MMRD tumours with lower mutation burden (10-100 mutations/Mb). Further, both mechanisms were associated with increased immune infiltration even in “immunologically-cold” tumours such a...
Purpose To evaluate the safety and efficacy of long vascular sheaths for transfemoral neuroendova... more Purpose To evaluate the safety and efficacy of long vascular sheaths for transfemoral neuroendovascular procedures in children. Materials and Methods A retrospective evaluation of transfemoral neuroendovascular procedures in children <18 years, using long sheaths was undertaken analyzing procedure type, fluoroscopic times, technical success, access site and systemic complications. Twenty-seven consecutive procedures were included over a 2-year period. Mean age was 8.4 years (standard deviation [SD] 6.3) (range 17.0 months–16.3 years). Results Patients were 44% female and mean weight was 35.0 kg (SD 22.8) (range 9.8–72.2 kg). A third of the procedures were performed in ≤15 kg children. The most common procedure was for embolization (n=13, 48.1%) and the most common indication was dual microcatheter technique (52%). The most common device used was the 5 Fr Cook Shuttle sheath. Mean fluoroscopy time was 61.9 minutes (SD 43.1). Of these procedures, 93% were technically successful. Fe...
Pediatric patients present with a wide variety of head and neck pathologies. Although some lesion... more Pediatric patients present with a wide variety of head and neck pathologies. Although some lesions can be diagnosed on clinical grounds, there are many others that require imaging for diagnosis. Imaging plays an important role in distinguishing among the potential diagnoses, as often, history and clinical assessment is limited in these young patients. Beyond diagnosis, imaging has a crucial role in evaluating the location and extent of a lesion for possible intervention. Understanding the imaging appearance of the myriad pathologies which are commonly encountered and their potential complications facilitates optimal management. In this chapter, we review the various imaging modalities available, benefits and limitations of each modality, and discuss the ten subsets of pediatric head and neck pathologies.
BACKGROUND Given the expanding evidence of clinico-radiological differences between moyamoya dise... more BACKGROUND Given the expanding evidence of clinico-radiological differences between moyamoya disease (MMD) and moyamoya syndrome (MMS), we compared the clinical and radiographic features of childhood MMD and MMS to identify predictors of ischemic event recurrence. METHODS We reviewed a pediatric moyamoya cohort followed between 2003 and 2019. Clinical and radiographic characteristics at diagnosis and follow-up were abstracted. Comparisons between MMD and MMS as well as between MMD and two MMS subgroups (neurofibromatosis [MMS-NF1] and sickle cell disease [MMS-SCD]) were performed. RESULTS A total of 111 patients were identified. Patients with MMD presented commonly with transient ischemic attacks (TIAs) (35 % MMD versus 13% MMS-NF1 versus 9.5% MMS-SCD; P = 0.047). Symptomatic stroke presentation (MMD 37% versus MMS-NF1 4% versus 33%; P = 0.0147) and bilateral disease at diagnosis (MMD 73% versus MMS-NF1 22 % versus MMS-SCD 67%; P = 0.0002) were uncommon in MMS-NF1. TIA recurrence was common in MMD (hazard ratio 2.86; P = 0.001). The ivy sign was absent on neuroimaging in a majority of patients with MMS-SCD (MMD 67% versus MMS-NF1 52% versus MMS-SCD 9.5%; P = 0.0002). Predictors of poor motor outcome included early age at diagnosis (odds ratio [OR] 8.45; P = 0.0014), symptomatic stroke presentation (OR 6.6; P = 0.019), and advanced Suzuki stage (OR 3.59; P = 0.019). CONCLUSIONS Moyamoya exhibits different phenotypes based on underlying etiologies. Frequent TIAs is a common phenotype of MMD and symptomatic stroke presentation a common feature of MMD and MMS-SCD, whereas unilateral disease and low infarct burden are common in MMS-NF1. In addition, absence of ivy sign is a common phenotype in MMS-SCD.
Antithrombotic therapy is currently recommended for stroke prevention in pediatric cardioembolic ... more Antithrombotic therapy is currently recommended for stroke prevention in pediatric cardioembolic stroke where the recurrence risk is high; however, safety concerns remain. The primary objective of this study was to evaluate clinical and radiographic predictors of hemorrhagic transformation and stroke recurrence in children with cardiac disease to ascertain the safety and failure rates for secondary stroke prevention.
BackgroundFailure to appreciate deep venous drainage pathways is a major cause of severe complica... more BackgroundFailure to appreciate deep venous drainage pathways is a major cause of severe complications in the endovascular treatment of vein of Galen aneurysmal malformations (VOGMs).ObjectiveTo report deep venous drainage patterns in patients with VOGM, emphasizing the internal cerebral veins, and to describe the challenges in evaluating these.MethodsPatients with VOGM presenting to our institute between 2000 and 2018 were retrospectively analyzed. Patients with complete and good quality imaging datasets were included in the study. Three neuroradiologists with expertise in the subject independently analyzed the deep venous drainage patterns on multi-sequence MRI and digital subtraction angiography. Follow-up imaging studies were analyzed for alterations in deep venous drainage patterns that occurred following endovascular treatment. Descriptive statistics were used to report findings.ResultsTwenty-three patients had optimal quality MRI imaging and 25 had optimal quality DSA imaging...
Journal of Stroke and Cerebrovascular Diseases, 2020
PURPOSE The purpose of this study was to determine the additional information provided by Magneti... more PURPOSE The purpose of this study was to determine the additional information provided by Magnetic Resonance Angiography (MRA) in pediatric sickle cell disease (SCD) patients with normal Transcranial Doppler imaging (TCDI) examinations. METHODS This cohort study included all pediatric SCD patients over an 18-year period who had no history of stroke and had normal TCDI examinations and subsequently underwent MRA. Routine TCDI inclusive of time-averaged mean of maximum velocities (TAMMV) were assesses and compared with tortuosity on MRA and silent infarct on MRI. RESULTS 86 children (52.3% female; mean age 8.7 ± 3.5years) were included. There were 77 patients (89.5%) with Hb-SS disease and 9(10.4%) with HB-S beta-thalassemia. All patients had normal TAMMV (<170 cm/s) on TCDI. 76/86 (88.3%) patients also had one or more velocity readings <70 cm/s, albeit none in the middle cerebral arteries. Posterior cerebral arteries had the lowest velocities, <70 cm/s in 51.7% (right) and 60.9% (left). Silent MRI infarcts were seen in 27/86 (31.4%) patients. No new lesions were identified on follow-up MRI. Although mild vascular tortuosity was appreciated in 31/86 (36.0%) of the patients, there were no steno-occlusive lesions in the circle of Willis. CONCLUSIONS TCDI and MRA are routinely performed for non-invasively evaluating intracranial vascular abnormalities in children with SCD. In SCD children with no history of TIA or stroke, MRA following a normal TCDI examination is unlikely to show vascular abnormality. However, almost a third of these patients show silent infarcts on MRI, unassociated with MRA changes.
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Papers by Manohar Shroff