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.

Measured gap length and outcomes in oesophageal atresia.

AIMS: Oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF) is the most common congenital anomaly of the oesophagus. There is limited literature suggesting a linear relationship between increasing gap length and the incidence of all major complications. We sought to assess whether measured gap length at the time of surgery was related to outcomes in our patients. METHODS: All patients with a diagnosis of OA +/- TOF who underwent repair under a single surgeon between 1983 and 2012 were included. The length between the oesophageal pouches was measured at the time of surgery. Patients were then divided into three groups; short ≤1cm, intermediate >1-≤2cm and long >2-≤5cm. Outcome measures were anastomotic leak, strictures requiring dilatation, gastrooesophageal reflux disease (GORD) and need for fundoplication. RESULTS: 122 patients were included in the study. The outcomes for patients with short (n=53), intermediate (n=51) and long gaps (n=18) were as follows: anastomotic leak - 1.9%, 2%, 5.5% (P=0.66), strictures requiring dilatation - 32%, 33%, 50% (P=0.67), GORD - 51%, 59%, 72% (P=0.58) and need for fundoplication - 11%, 20%, 44% (*P=0.02). There were no deaths related to the repair. CONCLUSIONS: Measured gap length at the time of surgery did not have a linear relationship with leak or stricture rate. Our experience suggests that when primary repair is possible absolute gap length is irrelevant to the development of post-operative complications. There is however a significant increase in the need for fundoplication in those with a long gap.

Ibuprofen-induced papillary necrosis causing bilateral ureteric obstruction.

Ibuprofen, a non-steroidal anti-inflammatory drug, is the mainstay in the management of pain and fever in children. In most children, ibuprofen is well tolerated and does not produce significant adverse effects. We report a case of acute papillary necrosis causing bilateral ureteric obstruction induced by ibuprofen.

Transpubic radical prostatectomy: a novel approach for rhabdomyosarcoma of the prostate in children.

Rhabdomyosarcoma is the most common soft tissue sarcoma, representing 10-15% of all malignant solid tumours in children and can occur in any site. The prognosis for rhabdomyosarcoma of the prostate has improved significantly in the last few decades. The management involves a combination of chemotherapy, radiotherapy and surgery. Several approaches to resect the prostate in adults have been described. These include radical retropubic, transperineal, posterior sagittal as well as the transpubic approach. In the child, the transpubic approach allows direct access to the prostate and proximal urethra whilst obviating problems noted in adults. We describe this approach to the prostate in a 3-year-old boy.