Spinal Dysraphisms: A New Anatomical–Clinicoradiological Classification

Background Spinal dysraphisms refer to the congenital abnormalities of the spine and spinal cord due to aberrations in the processes of gastrulation, primary neurulation, and secondary neurulation. Embryology of many complex spinal dysraphisms are yet poorly understood and there is no agreeable anatomical–clinicoradiological classification with inclusion of recently documented and complex spinal dysraphisms. Aims and Objectives The main objective of this study was to review the imaging features of spinal dysraphisms with a better understanding of embryological abnormalities and propose a new classification inclusive of all complex and unusual dysraphisms based on anatomical and clinicoradiological correlation. Materials and Methods This was a retrospective single institutional observational study of 391 cases of spinal dysraphism for 10 years in our institution. Of 391 cases included in the study, 204 were males and 187 were females. Also, 123 cases belonged to the 0–6 months age group, 38 cases belonged to the 7–12 months age group, 156 belonged to the 1–5 years age group, 39 cases belonged to the 6–10 years age group, and 35 cases belonged to 10–20 years age group. Results An anatomical–clinicoradiological analysis of cases yielded a high proportion of cases of spinal lipomas, including lipomyeloceles and lipomyelomeningoceles (31.3%) and posterior myelomeningocele (14.2%). Anterior myelocoele (0.2%), sacral chordoma(0.2%), and intrasacral meningocele (0.2%) formed the least proportion of cases. A new classification was proposed based on the analysis of acquired data. Conclusion A structured approach in imaging spinal dysraphism is necessary for imaging evaluation in recent years. The proposed new classification based on clinicoradiological correlation and anatomic location is inclusive of unusual and complex dysraphisms.

Management of a Rare Pathology in Children it is Lipomyelomenengocele about Casis Report

The lipomyelomeningocele (LMMC) is a type of congenital occult spinal dysraphism consistent with the presence of lipomatous tissue adhering to the epinetic marrow, which falls through a defect of the vertical colon with the former meninges and the marrow under posture the skin. LMMC is the cause of the most frequent congenital attache between the cadre of the syndrome of the attached cord and causes a neurological deterioration due to the compression of the medical bone and the root system which is superadded. It is therefore about a girl from 6 years ago with a swelling located in the region of Lombardy after the birth of the size of a tangerine which has increased the size gradually with age. He presented the interrogation of the signs of urinary incontinence and the fact that he confirmed that he left us to fellow therapists who sent him to us for support. The local examination of the lumbosacral region revealed a single mass without other distinctive skin signs, spherical in shape, 12 × 10 cm, soft and fixed in consistency. A medullary MRI with effect, which reveals a mass of lipomate consistency at the level of lumbosacral associated with a bone spina extending from L5 to S2. Surgical treatment was indicated as the patient had urinary problems. There were no postoperative complications and after a few months postoperative symptoms improved.

Refractory CSF leakage following untethering surgery performed 10 months after birth for enlarging terminal myelocystocele associated with OEIS complex

Background: Terminal myelocystocele (TMC) is an occult spinal dysraphism characterized by cystic dilatation of the terminal spinal cord in the shape of a trumpet (myelocystocele) filled with cerebrospinal fluid (CSF), which herniates into the extraspinal subcutaneous region. The extraspinal CSF-filled portion of the TMC, consisting of the myelocystocele and the surrounding subarachnoid space, may progressively enlarge, leading to neurological deterioration, and early untethering surgery is recommended. Case Description: We report a case of a patient with TMC associated with OEIS complex consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S). The untethering surgery for TMC had to be deferred until 10 months after birth because of the delayed healing of the giant omphalocele and the respiration instability due to hypoplastic thorax and increased intra-abdominal pressure. The TMC, predominantly the surrounding subarachnoid space, enlarged during the waiting period, resulting in the expansion of the caudal part of the dural sac. Although untethering surgery for the TMC was uneventfully performed with conventional duraplasty, postoperative CSF leakage occurred, and it took three surgical interventions to repair it. External CSF drainage, reduction of the size of the caudal part of the dural sac and use of gluteus muscle flaps and collagen matrix worked together for the CSF leakage. Conclusion: Preoperative enlargement of the TMC, together with the surrounding subarachnoid space, can cause the refractory CSF leakage after untethering surgery because the expanded dural sac possibly increases its own tensile strength and impedes healing of the duraplasty. Early untethering surgery is recommended after recovery from the life-threatening conditions associated with OEIS complex.

Spinal dysraphism with tripedus morphology: A case report

This is a rare case report of a 5-month-old child with a complex spinal dysraphic state, and an accessory limb (tripedus morphology), accessory genitalia, and anal dimple. The child was brought to the hospital with an accessory limb arising from the back. On clinical examination, an accessory limb arising from the lower back with a partially developed foot with the presence of toes and nails was noted. Spinal MRI was advised which revealed dysraphic features including spina bifida with the low lying and posteriorly tethered cord with diastematomyelia along with a supernumerary appendage attached to the vertebral column having rudimentary bones resembling those of extremities. The presence of an accessory limb with spinal dysraphism is quite a rare anomaly. The condition can be treated by surgical intervention and involves excision of the accessory limb with adequate dural and paraspinal muscle cover.

Estimating the prevalence of neurosurgical interventions in adults with spina bifida using the Health Facts data set: implications for transition planning and the development of adult clinics

OBJECTIVE As the care of patients with spina bifida continues to evolve, life expectancy is increasing, leading to a critical need for transition planning from pediatric-based to adult-based care. The burden of neurosurgical care for adults with spina bifida remains unknown. In this study, the authors sought to use a large national data set to estimate the prevalence of neurosurgical interventions in adults with spina bifida. METHODS This study utilized Health Facts, which is a de-identified proprietary data set abstracted from all Cerner electronic health records. It includes 69 million unique patients with > 500 million encounters in 580 centers. Validation, technical exclusions, and data filters were applied to obtain an appropriate cohort of patients. The ICD-9 and ICD-10 codes for all types of spinal dysraphism, as well as the Current Procedural Terminology (CPT) codes for hydrocephalus procedures, spinal cord untethering, and Chiari decompression, were queried and records were retrieved. Demographic variables along with differences in age groups and temporal trends were analyzed. RESULTS Overall, 24,764 unique patients with ≥ 1 encounter with a spinal dysraphism diagnosis between 2000 and 2017 were identified. The pediatric cohort included 11,123 patients with 60,027 separate encounters, and the adult cohort included 13,641 patients with 41,618 separate encounters. The proportion of females was higher in the adult (62.9%) than in the pediatric (51.4%) cohort. Annual encounters were stable from 2 to 18 years of age, but then decreased by approximately half with a precipitous drop after age 21 years. The sex distribution of adults and children who underwent procedures was similar (54.6% female adults vs 52.4% female children). Surgical interventions in adults were common. Between 2013 and 2017, there were 4913 procedures for hydrocephalus, with 2435 (49.6%) adult patients. Similarly, 273 (33.3%) of the 819 tethered cord procedures were performed in adults, as were 307 (32.9%) of 933 Chiari decompressions. CONCLUSIONS The Health Facts database offered another option for studying care delivery and utilization in patients aging with spina bifida. The median age of this population has now reached early adulthood, and a significant number of neurosurgical procedures were performed in adults. An abrupt drop in the rate of encounters occurred at 21 years of age, possibly reflecting transition issues such as access-to-care problems and lack of coordinated care.

Non-dysraphic Subpial Intramedullary Lipoma of Cervicothoracic Spine

Intramedullary spinal cord tumors are rare and comprise less than one percentage of all central nervous system (CNS) neoplasms. When encountered, most are found in the lumbosacral region with coinciding spinal dysraphism. Here, we discuss a case of isolated non-dysraphic intramedullary lipoma of cervicothoracic spine in an adolescent who was surgically managed with posterior decompression and subtotal resection via laminoplasty. A 21-year-old male adolescent, without a history of spinal dysraphism, presented with a progressive sensory ataxia. This manifestation was attributed to magnetic resonance documented intramedullary lipoma, extending from C7 to T2 spinal level. Patient underwent subtotal surgical resection of the lesion; and histopathology report was confirmed as spinal lipoma. Surgical management of this rare pathology has a wide variety of options depending on clinical presentation. In our case, a subtotal resection and laminoplasty was achieved with no further worsening of neurologic symptoms.

P.234 “Inappropriate” spinal ultrasounds for suspected occult spinal dysraphism in northern Alberta

Background: Occult spinal dysraphism (OSD) may be associated with visible cutaneous manifestations. A common non-pathological mimic of a dermal sinus tract is an incidental sacrococcygeal dimple. Choosing Wisely Canada Guidelines recommend these dimples not be imaged. Methods: This study assessed the appropriateness of spinal ultrasounds performed for the investigation of an OSD. We interrogated our local imaging system to analyze spinal ultrasounds being performed in babies less than 6 months of age, from 2017-2018. Results: 429 children had spinal ultrasounds ordered by pediatricians (55%), family doctors (21%), and neonatologists (20%). 183 children (43%) had imaging indications that was deemed “inappropriate”. Some of this cohort had further MRI imaging (5/183) or neurosurgical referral (8/183). None of these children underwent neurosurgery. 231 (54%) children had appropriate indications for imaging. Within this cohort MRI imaging (23/231), neurosurgical referral (24/231) and neurosurgical intervention occurred (4/231). All four “surgical children” harboured either a dorsal appendage or a subcutaneous lipoma. We estimated, declining inappropriate scans would save $22, 500 annually. Conclusions: A significant portion of local spinal ultrasound requests for OSD are inappropriate. Collaboration with other sites is ongoing to investigate if the Choosing Wisely guidelines for imaging this patient population are being practiced more efficiently.

Tatalaksana Anestesi Posisi Telungkup pada Pasien Pediatrik yang Menjalani Prosedur Reseksi dan Rekonstruksi Human Tail

Human tail or additional tail is an unusual malformation and is divided into true tails and pseudotails (lipoma, teratoma, fetal parasitic mylomeningocele). Human tail is associated with spinal dysraphism. Myelomeningocele is spinal dysraphism in which the spinal cord and its contents herniate through a bone defect. congenital to the posterior element. The surgical management of MMC poses challenges, not only to the implementation of anesthesia but also to provide perioperative care depending on the pediatric age group, comorbid conditions, and associated systemic disorders. Anesthesia for the MMC in the lumbosacral region was performed in the prone position. Changing the patient's position to the prone position is a critical maneuver. Complications that occur due to the wrong prone position can cause morbidity and some cases cause mortality so that anesthesia with the prone position must be well understood to avoid the risks and complications that can occur. A 5 year old girl with myelomeninocele will undergo a resection and reconstruction procedure in a prone position. The hemodynamic status during the procedure which lasted 3 hours was quite stable with a hemorrhage of about 10cc. After surgery, patients were treated for 1 day in PICU and ward for 3 days.