International Consensus Statement on the Radiological Evaluation of Dysraphic Malformations of the Spine and Spinal Cord.

Dysraphic malformations of the spine and spinal cord (DMSSC) represent a spectrum of common congenital anomalies typically (though not exclusively) affecting the lower spinal segments. These may be responsible for varying degrees of neurologic, orthopedic, and urologic morbidity. With advances in neuroimaging, it is now possible to better diagnose and evaluate these disorders both prenatally and postnatally. Neuroimaging, performed at the right time and with technique optimization, is integral in guiding clinical management. However, the terminology used to describe these lesions has become increasingly confusing, and there is a lack of consensus regarding the essential radiologic features and their clinical weighting. This variability in radiologic practice risks unstructured decision making and increases the likelihood of suboptimal, less informed clinical management. In this manuscript, the first of a series of consensus statements, we outline a standardized international consensus statement for the radiologic evaluation of children with suspected DMSSC derived from a critical review of the literature, and the collective clinical experience of a multinational group of experts. We provide recommendations for plain radiography, sonography, CT, and MR imaging in the evaluation of DMSSC with an emphasis on technique of imaging and imaging protocols.

Patterns of spinal cord malformation in cloacal exstrophy.

OBJECTIVE: The objective of this study was to assess the prevalence and spectrum of spinal dysraphism in a cohort of children with cloacal exstrophy (CEX) using MRI. METHODS: Children with CEX presenting between 1999 and 2019 with baseline spinal MRI were included. The images were reviewed in consensus to assess the type of dysraphism. The dysraphisms were initially reviewed and described based on their descriptive anatomy, and then classified according to anomalies of gastrulation, primary neurulation, or secondary neurulation. RESULTS: Thirty-four children were included. Thirty-three of these children had closed spinal dysraphism, and 1 had a normal spine. Of the 33 cases of closed spinal dysraphism, the conus and/or filum terminale were involved in all cases. The most common malformations were spinal lipoma (n = 20) and terminal myelocystocele (n = 11). The lipomas were heterogeneous: 4 dorsal, 9 transitional, 4 chaotic, and 3 terminal. A large subgroup (10/20, 50%) within the lipomas had an unusual morphology of noncontiguous double lipomas, the proximal fat related to the conus and the distal fat within the filum. These were difficult to characterize using existing classifications. In 2 cases, only a thickened filum was noted. The majority of these malformations were compatible with a disorder of secondary neurulation. CONCLUSIONS: Complex spinal dysraphisms are consistently associated with CEX. The unusual dysraphism patterns found in this group of patients highlight the limitations of current embryological classifications. Given the propensity for neurological deterioration in this group of patients, spinal MRI should be routinely performed. The type and distribution of malformations seen have implications for the wider understanding of the pathogenesis and classification of lumbosacral lipomas.

Isolated Unilateral Cerebellar Hemispheric Dysplasia: A Rare Entity.

A 9-year-old female presented to neurology outpatient department of our hospital with complaints of recurrent generalized tonic-clonic seizures since birth and was being treated with anticonvulsants for the same. Patient also had complaints of giddiness and episodes of momentary loss of consciousness. There was history of twitching of left hemiface and eyelid during infancy, often associated with deviation of eyes to the left and groaning. The birth history was unremarkable. Family history revealed no known consanguinity. General examination revealed no dysmorphic features. Neurological examination revealed no cognitive deficits/signs to suggest cerebellar pathology. An electroencephalogram was done in view of her recurrent seizures, which was normal. Initial laboratory work-up was normal. The patient then underwent magnetic resonance imaging (MRI) brain, acquired with a 1.5-T unit (Siemens, Erlangen, Germany). MRI brain revealed hemihypertrophy of left cerebellar hemisphere with disorganized architecture, fissural malorientation with individual folia running vertically rather than horizontally with disorganized foliation, abnormal arborization of white matter predominantly involving mid and dorsal surface of left cerebellar hemisphere and a few suspicious areas of abnormal T2-hyperintense signal in subcortical white matter. Right cerebellar hemisphere and cerebellar vermis were normal. Corpus callosum was normal. Cerebral parenchyma was normal in signal intensity pattern with normal gray-white matter differentiation. Ventricular system was normal (Figures 1 and 2). Cerebellar malformations are uncommon and are usually associated with Dandy-Walker continuum, Joubert syndrome, rhombencephalosynapsis, lissencephaly, Fukuyama congenital muscular dystrophy, Walker-Warburg syndrome, muscle-eye-brain disease, congenital cytomegalovirus infection to name a few.1,2 Isolated unilateral cerebellar hemispheric dysplasia is exceedingly rare with only a few cases previously described in English literature. Cerebellar malformations are less adequately understood entity partly because of the complex cerebellar embryology and limited histologic studies of these disorders. Genes expressed in migration and maintenance of the Purkinje cells and/or in the generation and migration of granular cells when mutated will disrupt cerebellar migration and foliation and thus cause cerebellar malformation.3-5 Cerebellum is known to be a centre for motor learning, coordination, and higher cognitive functions. Clinical presentation of cerebellar malformations is highly variable and depends on the degree of cerebellar involvement, presence of associated cerebral involvement and the underlying disorders such as muscular dystrophy if any. Patel and Barkovich suggested an imaging-based classification of cerebellar malformations and classified the malformations broadly into two types, malformations with cerebellar hypoplasia and the ones with cerebellar dysplasia. Each of these was further classified into focal and diffuse.1 Demaerel gave a classification of abnormalities of cerebellar foliation and fissuration.2 Our index case with disorganized architecture, fissural malorientation and disorganized foliation of left cerebellar hemisphere associated with normal cerebellar vermis, corpus callosum, and absence of cerebral malformation falls into Type 2 category as per the classification by Demaerel.2 Treatment depends upon the severity of symptoms and the underlying disorder in case of syndromic malformations. Generally, treatment is symptomatic and supportive. Understanding of the basics of cerebellar embryology, knowledge of the imaging features, and clinical presentation aids in the precise diagnosis of this disorder and its optimal management.

Endovascular glue embolization for control of massive hemoptysis caused by peripheral pulmonary artery pseudoaneurysms: Report of 7 cases.

PURPOSE: Pulmonary artery pseudoaneurysms are a rare cause of massive hemoptysis and need to be considered as a differential with prompt recognition preventing mortality from life-threatening hemorrhage. We report the clinical details and imaging findings for a series of patients presenting with massive haemoptysis due to peripheral pulmonary artery pseudoaneurysm, managed by endovascular glue embolization. METHODS: Hospital based retrospective study wherein records and image database of patients presenting with massive hemoptysis between January 2014 and March 2016 were studied. The immediate technical success, reintervention rate, and recurrence of massive hemoptysis were recorded. RESULTS: Among patients with massive hemoptysis who underwent endovascular management in our department, seven patients fulfilled the inclusion criteria. The mean age was 54.3 years; mean lesion diameter was 10.8 mm (range 6-14 mm); underlying pathology being infective (tuberculosis) in all cases (n = 7). All lesions were treated with endovascular glue embolization. The technical success was 100%. Mean follow-up was 11.7 months. There was no case of recurrence of massive hemoptysis. There were no major complications with a single case of minor complication (in the form of chest pain and discomfort) which resolved with medical management. CONCLUSION: The endovascular approach using glue (N-butyl cyanoacrylate) is a minimally invasive and technically feasible, effective technique for emergent management of pulmonary artery pseudoaneurysms presenting as massive hemoptysis.

Triptan-induced Reversible Cerebral Vasoconstriction Syndrome Presenting With Thunderclap Headache and Paraparesis.

Reversible cerebral vasoconstriction syndrome is a rare clinical syndrome characterized by sudden thunderclap headache often an under diagnosed neurological emergency. It is often provoked by postpartum state or exposure to provocative drugs. We report a rare case of Rizatriptan-induced reversible cerebral vasoconstriction syndrome presenting with thunderclap headache and paraparesis with complete recovery of neurological and imaging findings.

Spontaneous Isolated Inferior Mesenteric Artery Dissection With Chronic Mesenteric Ischemia: Case Report and Endovascular Management.

We report the clinical details, imaging findings, and management for a 39-year-old female presenting with recurrent episodes of pain in abdomen due to systemic lupus erythematous vasculitis associated with spontaneous isolated inferior mesenteric dissection. Spontaneous mesenteric artery dissection is an uncommon cause of mesenteric ischemia. Symptomatic spontaneous isolated inferior mesenteric artery (IMA) dissection is a rare condition, and its association with systemic lupus erythematosus is not previously described in the English literature. The optimal treatment options are debatable and include medical management, surgical reconstruction, and endovascular therapy. We wish to highlight spontaneous isolated IMA dissection as a rare etiology for chronic mesenteric ischemia and its management by endovascular methods.