Lumbosacral tuft of hair as a key stigma for early detection of occult spinal dysraphism: A case report of diastematomyelia with tethered cord in a newborn.

Early detection of occult spinal dysraphism, such as diastematomyelia with tethered cord, is vital to prevent neurological damage. Though rarely diagnosed in the neonatal period, cutaneous stigmata can aid early identification. Utilizing neonatal ultrasound enables timely management and multidisciplinary intervention.

Dorsal ectopic breast-a cutaneous marker of occult spinal dysraphism.

Ectopic breasts represent uncommon medical anomalies characterized by the presence of additional breasts situated outside the typical milk line. In this case report, we present a case encompassing dorsal ectopic breasts accompanied by three nipples. A 14-year-old teen girl presented with a significant dorsal mass, exhibiting no clinical manifestations except mild pain and a sense of heaviness. She underwent investigations, and a magnetic resonance imaging scan revealed a large, inadequately encapsulated congenital mass with a fatty composition, alongside intraspinal epidural engagement and a bony structure bridging the spinous processes from the C6 to the T3 vertebral bodies. She subsequently underwent excision of the entire mass. The histopathological analysis identified the presence of a mammary hamartoma. Follow-up at 2 weeks postoperatively showed that there were no further complications. Dorsal ectopic breast necessitates careful and comprehensive evaluation to either confirm or exclude the presence of occult spinal dysraphism prior to intervention to avoid complications.

A 31-year follow-up of those with occult spinal dysraphism, who underwent spinal cord untethering: Lower urinary tract function, skin stigmata, fertility, and work participation.

OBJECTIVES: This study evaluated patients with occult spinal dysraphism who underwent spinal cord untethering. METHODS: Twenty-four patients who visited us between 1983 and 2000 were followed-up for a mean duration of 31 years. We studied their lower urinary tract function, skin stigmata, fertility, and work participation. RESULTS: Questionnaires sent in 2022 revealed that 5 patients had normal voiding (Group A) and 19 patients had abnormal voiding (Group B). Groups A and B underwent spinal cord untethering at a mean age of 5.7 and 13.0 years, respectively, showing a significant statistical difference (p = 0.036). After spinal cord untethering, the number of patients with detrusor normoactivity increased from 0 to 5, i.e., 3 of 6 with detrusor overactivity (50%), 1 of 2 not examined, and 1 of 5 not known. Patients with detrusor underactivity also increased from 11 to 19. Severity of incontinence in the International Consultation on Incontinence Questionnaire-Short Form resulted in a mean value of 2.4 in Group A, which was significantly superior to the mean value of 9.1 in Group B (p = 0.004). Fourteen patients (58.3%) were married and had 21 healthy children. A majority of patients have had full-time jobs. A variety of skin stigmata were present in the lumbosacral region, and changes in vesico-urethral configurations were observed during a video-urodynamic study. CONCLUSIONS: Our study identified that the early timing of spinal cord untethering performed in neonates or infants and detrusor overactivity prior to untethering surgery are important factors in achieving normal bladder function.

Complex lumbosacral spinal cord lipomas: A longitudinal study on outcomes from a Singapore children's hospital.

BACKGROUND: Total/near-total resection (TR/NTR) of complex lumbosacral lipomas (CSL) is reported to be associated with better long-term functional outcomes and lower symptomatic re-tethering rates. We report our institutional experience for CSL resection in affected children. METHODS: This is a single-institution, retrospective study. Inclusion criteria consist of patients with CSL with dorsal, transitional and chaotic lipomas based on Pang et al's classification. The study population is divided into 2 groups: asymptomatic patients with a normal preoperative workup referred to as 'prophylactic intent' and 'therapeutic intent' for those with pre-existing neuro-urological symptoms. Primary aims are to review factors that affect post-operative clean intermittent catheterization (CIC), functional outcomes based on Necker functional score (NFS), and re-tethering rates. RESULTS: 122 patients were included from 2000 to 2021. There were 32 dorsal lipomas (26.2 %), 74 transitional lipomas (60.7 %), and 16 chaotic lipomas (13.1 %). 82 % patients achieved TR/NTR. Favourable NFS at 1-year was 48.2 %. The re-tethering rate was 6.6 %. After multivariable analysis, post-operative CIC was associated with median age at surgery (p = 0.026), lipoma type (p = 0.029), conus height (p = 0.048) and prophylactic intent (p < 0.001). Next, extent of lipoma resection (p = 0.012) and the post-operative CSF leak (p = 0.004) were associated with re-tethering. Favourable NFS was associated with lipoma type (p = 0.047) and prophylactic intent surgery (p < 0.001). CONCLUSIONS: Our experience shows that TR/NTR for CSL is a feasible option to prevent functional deterioration and re-tethering. Efforts are needed to work on factors associated with post-operative CIC.

Hypospadias management in children with anorectal malformation: a multidisciplinary single center experience.

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.

Split Spinal Cord Malformation Fed by Bilateral, Enlarged Radiculopial Arteries.

Anatomical variations of the spinal cord are seen in many manifestations; one rare variant that does not stem from a neural tube defect is known as a split cord malformation (SCM). In this variation, a deviation from normal development causes the spinal cord to divide into two hemicords, typically in the lumbar region. In the case described here, a SCM was observed with large, bilateral, radiculopial arteries. To our knowledge, such large vessels in conjunction with a SCM has not previously been documented in the literature. Such variants could be problematic during surgical approaches to the lumbar spine. Herein, we report the case and discuss the development of the findings with relevant clinical applications.

Fibrofatty filum terminale: long-term outcomes from a Singapore children's hospital.

OBJECTIVE: The role of prophylactic detethering a fibrofatty filum terminale (FFT) remains equivocal. Furthermore, long-term studies focusing on urological outcomes are sparse. The aims of this study were to present an institutional experience on the perioperative and long-term outcomes of FFT surgery and to assess for factors that contribute to postoperative clean intermittent catheterization (CIC). METHODS: This was a single-institution, retrospective study conducted over a 20-year period. Patients younger than 19 years of age who underwent surgery for FFT were included. Variables of interest included patient demographics, clinical presentation, radiological findings, postoperative complications, and long-term need for CIC. Outcomes were measured using the Necker functional score and modified Hoffer Functional Ambulation scale score at 3, 6, and 12 months postdischarge. RESULTS: A total of 164 surgeries were performed for FFT from 2000 to 2020. The median age at surgery was 1.1 years, and the mean follow-up duration was 8.3 years. There were 115 patients (70.1%) who underwent prophylactic-intent surgery and 49 patients (29.9%) who underwent therapeutic-intent surgery. The proportion of therapeutic-intent surgeries increased significantly with age percentiles (0-20th, 21.9%; 20th-40th, 9.1%; 40th-60th, 18.2%; 60th-80th, 36.4%; and 80th-100th, 63.6% [p < 0.001]). Thirty patients (18.3%) had an associated syndrome, the most common (n = 19, 11.6%) being VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities). Forty-eight patients (29.3%) had an associated malformation (anorectal anomaly = 37, urogenital anomaly = 16, and sacral anomaly = 3). Cutaneous manifestation was the most common presentation (n = 96, 58.5%), followed by lower-limb neurological deficits (n = 21, 12.8%). A low-lying conus was present in 36.0% of patients (n = 59), and 16.5% had an associated syrinx (n = 27). There were 26 patients (18.8%) with an abnormal preoperative urodynamic study. Three patients (1.8%) had postoperative complications that required repeat surgery. There were no cases of CSF leakage. One patient (0.6%) developed retethering requiring another surgery. Postoperative CIC was required in 11 patients (6.7%). Multivariable analyses showed that an abnormal preoperative urodynamic study (adjusted OR 5.5 [95% CI 1.27-23.9], p = 0.023) and having an intraspinal syrinx (adjusted OR 5.29 [95% CI 1.06-26.4], p = 0.042) were associated with the need for CIC. CONCLUSIONS: The authors' results demonstrate that detethering surgery for FFT is a relatively safe procedure and can be performed prophylactically. Nonetheless, the risks of postoperative CIC should be emphasized during the preoperative counseling process.

Cerebrospinal fluid hypotension following fall in a child: Case report / Hipotensión del líquido cefalorraquídeo tras caída en un niño: caso clínico

CSF hypotension arises in the context of a leak of CSF which causes negative intracranial pressure. Sacral fractures result from high-energy trauma which are frequently underdiagnosed. A ten-year-old boy presented with hip pain, after a fall. He mobilized both lower limbs, reported no leg pain, irradiation nor lack of sphincter control. The neurological examination was normal. When asked to stand, he began biparietal headache, nausea and vomiting, which improved laying down. CT scan showed an occult intrasacral meningocele; the MRI revealed collections of CSF along the spine, a S3 fracture with potential laceration of the meningocele and opening of a CSF fistula. Our diagnosis was the CSF hypotension, secondary to the fistula opening. The diagnosis was challenging. The child first presented with symptoms of CSF hypotension without evident cause. The discovery of the meningocele led us to hypothesize the opening of a fistula, a rare diagnosis, later confirmed by MRI (AU)

La hipotensión del LCR surge en el contexto de una fuga de dicho líquido que causa presión intracraneal negativa. Las fracturas del sacro son originadas por traumatismos de alta energía que a menudo no se diagnostican. Un niño de diez años acudió con dolor de cadera tras una caída. Podía mover las piernas, no reportando ningún dolor en las mismas, ni irradiación o falta de control del esfínter. El examen neurológico resultó normal. Cuando se le pidió que se pusiera de pie comenzó a sufrir cefalea biparietal, náuseas y vómitos, que mejoraban al tumbarse. La TC mostró un meningocele intrasacro oculto. La RM reveló rastros de LCR a lo largo de la columna, fractura en S3 con laceración potencial del meningocele y apertura de una fístula de LCR. Nuestro diagnóstico fue hipotensión de LCR, secundaria a la apertura de la fístula. El diagnóstico resultó difícil. El niño presentó síntomas de hipotensión del LCR sin causa evidente. El descubrimiento del meningocele nos llevó a conjeturar la apertura de una fístula, un diagnóstico infrecuente que fue confirmado posteriormente mediante RM (AU)

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association.

PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Cerebrospinal fluid hypotension following fall in a child: Case report.

CSF hypotension arises in the context of a leak of CSF which causes negative intracranial pressure. Sacral fractures result from high-energy trauma which are frequently underdiagnosed. A ten-year-old boy presented with hip pain, after a fall. He mobilized both lower limbs, reported no leg pain, irradiation nor lack of sphincter control. The neurological examination was normal. When asked to stand, he began biparietal headache, nausea and vomiting, which improved laying down. CT scan showed an occult intrasacral meningocele; the MRI revealed collections of CSF along the spine, a S3 fracture with potential laceration of the meningocele and opening of a CSF fistula. Our diagnosis was the CSF hypotension, secondary to the fistula opening. The diagnosis was challenging. The child first presented with symptoms of CSF hypotension without evident cause. The discovery of the meningocele led us to hypothesize the opening of a fistula, a rare diagnosis, later confirmed by MRI.

Preoperative Videourodynamic Study Is Helpful in Predicting Long-term Postoperative Voiding Function in Asymptomatic Patients With Closed Spinal Dysraphism.

PURPOSE: Controversy exists regarding the role preoperative urodynamic study for asymptomatic closed spinal dysraphism as it has failed to reveal the benefit in surgical decision and expectation of urological outcomes. We explore the relationship between preoperative videourodynamic study and postoperative urological outcomes after toilet training completed, focusing on their capability of spontaneous voiding. METHODS: We retrospectively reviewed the data of 181 patients who underwent preventive spinal cord untethering and followed at least till the completion of toilet training. Before untethering, patients underwent preoperative videourodynamic study. Postoperative voiding function was evaluated in 3 phases: (1) till postoperative 6 months, (2) till the completion of toilet training, and (3) 2 years after toilet training. Changing distribution of emptying pattern at each period was described. Also, relevance to preoperative urodynamic parameters on spontaneous voiding and urinary continence after toilet training was assessed. RESULTS: Spinal lipoma and low lying conus were found in 145 (80%) and 128 patients (70.7%), respectively. Spontaneous voiding was found in 125 (69.1%), 164 (90.6%), and 162 patients (89.5%) at postoperative 6 months, till the toilet training, and 2 years after toilet training, respectively. Videourodynamics helped to clarify the presence of vesicourethral synergy. This was correlated with spontaneous voiding at postoperative 6 months and better urinary continence after 2 years of toilet training. CONCLUSION: Eventual spontaneous voiding was achieved till toilet training in 90% patients following preventive untethering. Those showing preoperative vesicourethral synergy was associated with faster achievement of spontaneous voiding and better urinary continence when they enter elementary school.

International survey on the management of lumbosacral cutaneous stigmata in infants with suspected occult spinal dysraphism.

OBJECTIVE: Occult spinal dysraphism (OSD) is a common pediatric neurosurgical diagnosis rife with controversy surrounding both the screening of asymptomatic infants and the threshold to offer a prophylactic detethering operation. The authors sought to clarify international practice patterns with a survey of pediatric neurosurgeons. METHODS: A survey asked pediatric neurosurgeons whether they would perform imaging in patients with a variety of cutaneous stigmata associated with OSD and whether they would offer prophylactic detethering surgery for asymptomatic patients with a variety of imaging findings on the OSD spectrum. RESULTS: Completed surveys were received from 141 pediatric neurosurgeons. Broad consensus was demonstrated on the need for obtaining images in sample patients with more severe stigmata ranging from large lipoma with a skin appendage to focal dysplastic skin in the lumbar midline. Ninety percent of respondents would perform MRI for these patients. In contrast, for patients with a low-sacral dimple, flat hemangioma, and symmetric (Y-shaped) splaying of the intergluteal cleft, opinion on the need for imaging varied considerably (between 57% and 89% recommended imaging). Respondents differed on the type of imaging that they would perform, with 31% to 38% recommending ultrasound screening. The responses reflected less consensus on when to offer surgery to patients with simple spinal tethering (low-lying conus medullaris and fatty filum terminale). Both a lower level of the conus and increased thickness of the filum terminale affected decision-making. CONCLUSIONS: The results of this survey showed significant consensus on the recommendation for screening imaging in patients with more dramatic cutaneous stigmata, although these stigmata are the rarest. A significant variance in opinions was reflected in the recommendation for imaging of the most common cutaneous stigmata. Consensus was also lacking on which lesions deserve prophylactic detethering surgery. Significant equipoise exists for future study of screening imaging and of surgical decision-making in patients with asymptomatic OSD and associated cutaneous stigmata.

Risk of occult spinal dysraphism based on lumbosacral cutaneous manifestations.

OBJECTIVES: Occult spinal dysraphism is a congenital failure of fusion of the posterior vertebral arches with intact skin overlying the defect. Lumbosacral cutaneous manifestations are associated with a variable risk of occult spinal dysraphism. Tethered cord syndrome is a type of occult spinal dysraphism that puts abnormal traction on the spinal cord. This study analyzed neonates and infants who were referred to our pediatric urology practice and had evidence of lumbosacral cutaneous manifestation on physical examination. METHODS: We reviewed the presence of lumbosacral cutaneous manifestations in neonates and infants evaluated in our pediatric urology clinic at our Institution over a 6-year period (1 March, 2015-28 February, 2021) with no prior diagnosis of lumbosacral cutaneous manifestation. All patients underwent a spinal ultrasound. RESULTS: The most common lumbosacral cutaneous manifestations were bifurcated/duplicated gluteal folds (33%), gluteal asymmetry (19%), and sacral dimples (14%). A total of 34 (24%) patients had an abnormal spinal ultrasound; 15 (44%) of these infants underwent a lumbar magnetic resonance imaging. A coccygeal pit was statistically marginally higher in abnormal versus normal spinal ultrasound (p = 0.07). Patients with only one lumbosacral cutaneous manifestation (N = 121) were significantly more likely to have a normal spinal ultrasound compared to those with two or more lumbosacral cutaneous manifestation (N = 17) (79% vs 53%, p = 0.03). CONCLUSION: Due to the varying risk of certain lumbosacral cutaneous manifestations with occult spinal dysraphism, all patients with a lumbosacral cutaneous manifestation should undergo spinal ultrasound. This study also highlights the importance of urodynamic studies when there are abnormal cutaneous findings. Routine physical examinations of the lumbar region for cutaneous manifestations of occult spinal dysraphism are vital to ensure prompt management of tethered cord syndrome and avoid potentially devastating consequences.

Cutaneous Stigmata of the Spine: A Review of Indications for Imaging and Referral.

Cutaneous stigmata of the midline spine are a common question in pediatrics. They are known to be related to a higher likelihood of underlying dysraphic spinal abnormalities. Clear understanding of different types of cutaneous stigmata and correlating dysraphic findings can aid in appropriate imaging workup and timely management of patient pathology. In this article, the authors review midline spinal cutaneous findings in the pediatric population with occult spinal dysraphism.

Cerebrospinal fluid hypotension following fall in a child: Case report.

CSF hypotension arises in the context of a leak of CSF which causes negative intracranial pressure. Sacral fractures result from high-energy trauma which are frequently underdiagnosed. A ten-year-old boy presented with hip pain, after a fall. He mobilized both lower limbs, reported no leg pain, irradiation nor lack of sphincter control. The neurological examination was normal. When asked to stand, he began biparietal headache, nausea and vomiting, which improved laying down. CT scan showed an occult intrasacral meningocele; the MRI revealed collections of CSF along the spine, a S3 fracture with potential laceration of the meningocele and opening of a CSF fistula. Our diagnosis was the CSF hypotension, secondary to the fistula opening. The diagnosis was challenging. The child first presented with symptoms of CSF hypotension without evident cause. The discovery of the meningocele led us to hypothesize the opening of a fistula, a rare diagnosis, later confirmed by MRI.

Sacral Extradural Angiolipoma Associated with Tight Filum Terminale and Spina Bifida Coexisting with Spinal Arteriovenous Fistula.

BACKGROUND: Spinal arteriovenous fistula (AVF) may rarely associate with spinal dysraphism, that is, tethered spinal cord and spinal intradural lipoma. Spinal extradural angiolipoma coexisting with spinal AVF has not been reported in the literature. We reported an extremely rare case of sacral angiolipoma associated with tight filum terminale and sacral spina bifida coexisting with spinal AVF within this tumor. CASE DESCRIPTION: A 55-year-old women presented with progressive myelopathy for 10 months. She had a painless, slow-growing mass at her left buttock since birth. Magnetic resonance imaging of the lumbosacral spine showed an extradural mass at the level of S3-S4, extending from the spinal canal through the spina bifida to the subcutaneous fat of the left buttock. There was a low conus medullaris at S2. Magnetic resonance imaging of the thoracic spine disclosed venous congestion with tortuous intradural flow voids along both ventral and dorsal surfaces of the spinal cord. Magnetic resonance angiography and spinal angiography revealed a hypervascular mass at the sacral level and associated arteriovenous shunt with cranial drainage into an enlarged medullary vein. Due to an infected pressure sore on the mass, endovascular treatment was initially performed with minimal recovery. Six months after complete healing of her infected pressure ulcer, the patient underwent surgical removal of extradural mass containing the AVF, and subsequent release of the tight filum. Histologic findings were consistent with angiolipoma. CONCLUSIONS: Sacral extradural angiolipoma in the present case may be congenital in origin with development of an acquired spinal AVF within the tumor.

Caudal Regression Syndrome-A Review Focusing on Genetic Associations.

Caudal regression syndrome (CRS) represents a spectrum of clinical phenotypes with varying degrees of malformation of the lower body with involvement of structures deriving from all 3 layers of the trilaminar embryo. We review areas of active investigation in the diagnosis, etiology, epidemiology, and treatment of the disease with a focus on underlying genetics. CRS pathobiology is complex and multifactorial with a significant contribution from environmental factors as evidenced in twin studies. Contemporary genomic and genetic investigations in both human primary tissue and murine in vitro and in vivo models implicate various genes associated with caudal differentiation and neural cell migration in embryogenesis. A large number of identified targets center around the metabolic regulation of retinoic acid and its derivatives. Dysregulation of retinoic acid homeostasis has been associated with abnormal embryonic cell migration, differentiation, and organogenesis with resulting malformations and agenesis in both a laboratory and a clinical setting. There appears to be a significant overlap in potential genetic targets with CRS and other developmental syndromes with similar presentations, such as VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) association. CRS represents a spectrum of caudal developmental abnormalities with treatment options limited to mild and moderate expressions of disease. Continued research is necessary to further clarify mechanisms of disease pathobiology and complex polygenetic and environmental interaction. Despite this, progress has been made in identifying genetic targets and downstream effectors contributing to preclinical and clinical progression.

Spinal cord anomalies in children with anorectal malformations: Ultrasound is a good screening test.

PURPOSE: The purpose of this study is to correlate spinal ultrasound (US) and magnetic resonance imaging (MRI) findings in patients with anorectal malformations (ARMs). METHODS: A retrospective analysis of records was performed for children with ARM presenting to two major pediatric hospitals between 2009 and 2017. The primary outcome analyzed was detection of spinal cord anomalies. Spinal US was performed up to 4 months and MRI within the first year of life. The conus medullaris was considered normal if it had a tapering contour and terminated at or above the Lumbar 2-3 disk space. RESULTS: One hundred ninety-three patients with ARM presented during the study period with a slight male preponderance (108, 56%). Spinal imaging was performed in 157(82%) - 137(87%) had US, 64(41%) had MRI and 44 (28%) had both. Of the 44 who had both; US was abnormal in 25 children-confirmed by MRI in 20 (80%). US was normal in 17 children- MRI showed a filum cyst in 1 and a lipoma in 2 children and was inconclusive in 2 children (p<0.001). All who required surgery except one child, were reported on spinal US to have a low lying cord, borderline low cord or tethered cord (p<.05). No child who was reported to have a normal spinal US required de-tethering at a later stage. Spinal US had an overall sensitivity of 91% and specificity of 75% compared to MRI for detecting spinal cord anomalies in children with ARM CONCLUSIONS: Spinal US performed in a tertiary pediatric imaging department was a good screening test for spinal cord anomalies in children with ARM. The finding of a low, borderline low or tethered cord on US mandates an MRI to confirm the findings and correlates with the need for operative correction of spinal cord tethering. STUDY TYPE: Clinical research paper. LEVEL OF EVIDENCE: 2.

Pediatric tethered cord release: an epidemiological and postoperative complication analysis.

BACKGROUND: Tethered cord release (TCR) is a common procedure in pediatric neurosurgery. Despite a reputation for being relatively safe, the risk factors for postoperative complications are poorly understood. METHODS: In this study, the American College of Surgeons-National Surgical Quality Improvement Program Pediatric Database (ACS-NSQIP-P) was reviewed to identify the demographics, risk factors, and 30-day postoperative complications for tethered cord release using univariate and multivariate analysis. A detailed analysis of reasons for readmission and reoperation was also performed. RESULTS: Three thousand and six hundred eighty-two pediatric patients were studied. Males undergoing TCR were younger (5.6 vs. 6.1 years) and had a higher rate of pre-operative comorbidities but lower 30-day complication rate versus females. Patients who later developed complications were more likely to require a microscope intraoperatively, had longer operative times, and worse preoperative American Society of Anesthesiologists (ASA) class. CONCLUSIONS: Despite being a relatively safe procedure, TCR in the pediatric population carries a finite risk of complications. In this large, international database study, males were found to have a greater number of risk factors prior to TCR, while females exhibit a higher risk of developing postoperative complications. This paper provides a large sample size of multi institutional pediatric patients undergoing TCR and may serve as a contemporary "snapshot" for future studies.

Importance of Physical Examination and Imaging in the Detection of Tethered Cord Syndrome.

Tethered cord syndrome (TCS) is a type of occult spinal dysraphism that may lead to permanent neurologic and orthopedic deficits. Infants with TCS may have lumbosacral cutaneous malformations (LsCMs). We studied 67 infants referred to a single pediatric urology practice for a urological concern unrelated to occult spinal dysraphism with no prior diagnosis of LsCM between March 1, 2015 and September 30, 2018. Each infant underwent a spinal ultrasound. If an abnormality was detected, they were referred to a pediatric neurosurgeon. The most common cutaneous manifestations were duplicated or bifurcated (46%) gluteal folds and gluteal asymmetry (16%). Fourteen (21%) of the 67 patients had an abnormal spinal ultrasound; 5 of the 14 infants underwent a lumbar magnetic resonance imaging. One infant had urodynamics studies and a tethered cord release. Pediatricians should be familiar with TCS and perform lumbar physical examinations for LsCMs suggestive of TCS to ensure prompt diagnosis and management and avoid potentially devastating complications.