Ultrasonographic images of spina bifida before obstetric anesthesia: a case series.

BACKGROUND: Spina bifida is a relatively common congenital malformation. As the functional prognosis of patients with spina bifida has improved over time, the number of cases resulting in pregnancy and delivery has increased. Lumbar ultrasonography has become a standard and helpful technique before neuraxial anesthesia. We believe that it might be valuable if we use lumbar ultrasonography to evaluate pregnant women with spina bifida before obstetric anesthesia. CASE PRESENTATION: We performed lumbar ultrasonography to evaluate four pregnant women with spina bifida. Patient 1 had no history of surgery. Lumbar radiography before pregnancy showed a bone defect from L5 to the sacrum as a result of incomplete fusion. Magnetic resonance imaging showed a spinal lipoma and a bone defect of the sacrum. Lumbar ultrasonography showed similar findings. We performed general anesthesia for emergency cesarean delivery. Patient 2 underwent surgical repair immediately after birth. Lumbar ultrasonography showed the same bone defect as well as a lipoma beyond the bone defect. We performed general anesthesia for cesarean delivery. Patient 3 had vesicorectal disorders but no prior surgery. Lumbar radiography before pregnancy showed congenital abnormalities such as incomplete fusion, scoliosis, rotation, and a notably small sacrum. Lumbar ultrasonography showed the same bone defect. We performed general anesthesia for cesarean section with no complications. Patient 4 complained of lumbago a few years after her first delivery and received a diagnosis of spina bifida occulta by lumbar radiography, with the incomplete fusion of only the 5th vertebra. Lumbar ultrasonography indicated the same abnormalities. We placed an epidural catheter to avoid the bone abnormality and achieved epidural labor analgesia with no complications. CONCLUSIONS: Lumbar ultrasonography shows anatomic structures easily, safely, and consistently, without X-ray exposure or the need for more expensive modalities. It is a helpful technique to explore anatomic structures potentially complicated by spina bifida before anesthetic procedures.

[Diagnosis and surgical treatment of tethered cord syndrome accompanied by congenital dermal sinus tract in adults].

OBJECTIVE: To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of tethered cord syndrome (TCS) accompanied by dermal sinus tract (DST) in adulthoods. METHODS: The authors retrospectively analyzed a series of 25 adult patients with TCS due to DST that were surgically treated under microscope from September 2010 to October 2019. There were 10 males and 15 females with an average age of 29.7 years (rang, 18-48 years). Characterized cutaneous malformation and dermal sinus were found in the lumbosacral region in all the 25 patients. Clinically, all the patients presented with chronic back and lower-extremity pain, numbness and weakness of lower limbs, and bowel and bladder dysfunction. Two cases were admitted to the emergency room with acute infectious cerebral spinal fluid (CSF) leakage complicated with meningitis. According to magnetic resonance imaging (MRI) images, the subdural course of DST whose traction of the spinal cord, the location of the conus medullaris, the presence of subdural lesions, and the diameter of the internal filum terminale were evaluated. The surgical procedure included separating and excising of the DST, section of the internal filum terminale, detethering of the TCS, and reconstruction of the dural sac under microscopy. The patients remained in prone position in 7 days postoperation. RESULTS: MRI showed that the position of the conus medullaris was lower than the level of lumbar 2 vertebrae, and the distal part of the DST entered the subdural stretched part of the spinal cord, to constitute one of the factors of TCS in all the 25 patients. Twenty patients had fatty infiltration of internal filum terminale and another patient had thickened (approximately 5 mm in diameter) internal filum terminale resulting in tightening the conus medullaris. A total of 25 operations were performed including completely dissection and resection of the DST through the skin down to the subdural space, section of the internal filum terminale, detethering of the TCS, and the subdural dermoid cysts were removed in two patients. There were no postoperative complications. The postoperative pathology was consistent with the structure of the DST and internal filum terminale. The local pain was relieved, and the lower-extremity weakness and bowel and bladder dysfunction were gradually relieved postoperatively. The period of follow-up ranged from 3 months to 9 years (mean, 3.9 years). The neurological function of all the patients was intact, and MRI showed that the physiological curvature of the thoracolumbar spine remained normal. There was no recurrence of TCS observed during the follow-up. CONCLUSION: The adult TCS accompanied with DST is characterized by typical cutaneous malformation in the lumbosacral region and tethering of the spinal cord. The patients are usually combined with internal filum terminale enlargement tightening of conus medullaris as well. The surgical treatment including totally resection of the DST and section of the internal filum terminale to detethering the TCS at the same time under microscopy. The outcome of surgical treatment is satisfactory.

Spondylolysis and spina bifida occulta in pediatric patients: prevalence study using computed tomography as a screening method.

PURPOSE: The prevalence of spondylolysis reported from radiograph-based studies has been questioned in recent computed tomography (CT)-based studies in adults; however, no new data are available in pediatric patients. Spina bifida occulta (SBO), which has been associated to spondylolysis, may be increasing its prevalence, according to recent studies in adults in the last decades, but without new data in pediatric patients. We aimed to determine the prevalence of spondylolysis and SBO in pediatric patients using abdomen and pelvis CT as a screening tool. METHODS: We studied 228 patients 4-15 years old (107 males), who were evaluated with abdomen and pelvis CT scans for reasons not related to the spine. The entire lumbo-sacral spine was evaluated to detect the presence of spondylolysis and SBO. We compared the prevalence of spondylolysis in patients with and without SBO. A logistic regression analysis was performed to determine the effect of age and sex as independent predictors of spondylolysis and SBO. RESULTS: The prevalence of spondylolysis was 3.5 % (1.1-5.9 %); 2/8 patients presented with olisthesis, both with grade I slip. The prevalence of SBO was 41.2 % (34.8-59.2 %) (94 patients). Spondylolysis was not more frequent in patients with SBO than in patients without SBO. Male sex and decreasing age independently predicted the presence of SBO, but not of spondylolysis. CONCLUSION: We observed a 3.5 % prevalence of spondylolysis and a 41.2 % prevalence of SBO. SBO was significantly more frequent in males and younger patients.

MICRODUPLICATION OF 17p[DUP(17)(12p11.2)]: REPORT OF A NEONATE WITH A SPINA BIFIDA AND CARDIAC ANOMALIES AND A LITERATURE REVIEW.

Duplication 17pll.2 syndrome is a recent recognized syndrome with multiple congenital anomalies and mental retardation. Most patients with duplication 17p11.2 syndrome harbor a common 3.7 Mb duplication (17p.11.2 duplication syndrome) resulting in congenital anomalies, neurodevelopmental and behavioral phenotypes. We report a case with spina bifida, tetralogy of Fallot and a small duplication (932 Kb) of 17pl1.2 containing approximately 20 genes, detected by array-CGH. We describe clinical features not reported previously for microduplication of 17p11.2.

[Pediatric Patient with anaerobic Bacterial Meningitis Who was Infected through a Spinal Congenital Dermal Sinus Route].

We report the case of a pediatric patient in whom a spinal congenital dermal sinus was detected after the onset of anaerobic bacterial meningitis. The patient was a 4-month-old boy. He had a recurrent fever for 2 weeks before admission. On admission, he presented with a convulsive status and a bulging anterior fontanel. The previously consulted physician had made a diagnosis of bacterial meningitis. Spinal fluid cultures tested positive for Peptoniphilus asaccharolyticus. Magnetic resonance imaging (MRI) showed a spinal subdural abscess and cranial subdural hydrops; therefore, the patient was transported to our hospital for surgical treatment. A sacral dimple was noted on his lower back, and an MRI showed a spinal congenital dermal sinus. Antimicrobial therapy, cranial subdural aspiration, dermal sinus excision, and drainage were performed. He was discharged on the 60th hospital day. When pathogens such as Staphylococcus aureus or Escherichia coli, Proteus sp. or anaerobic bacteria invade through a dermal sinus, it can result in meningitis. Involvement of a dermal sinus should be suspected when meningitis is caused by these pathogens or when recurrent meningitis occurs.

Intramedullary spinal cord abscess: The result of a missed congenital dermal sinus.

Congenital dermal sinus (CDS) and occult spinal dysraphism are suspected when a cutaneous marker overlies the spine of a newborn. CDS can have the appearance of a simple dimple and occur within the gluteal cleft without any skin markers. CDS are the commonest cause of intramedullary spinal cord abscess.

Dermal Sinus Tract of the Thoracic Spine Presenting with Intramedullary Abscess and Cranial Nerve Deficits.

Congenital dermal sinus tract of the spine is an unusual developmental defect which represents a failure of the surface ectoderm and dermal elements to separate from the neuroectoderm. A 15-month-old female presented with high fever, severe right hemiparesis, difficulty breathing and cranial nerve deficits. Magnetic resonance imaging (MRI) of the spine revealed a congenital dermal sinus tract at the Th6 level, an intramedullary collection extending up to the brainstem and a probable intramedullary cystic lesion. The child was operated acutely with ligation of the sinus tract, drainage of the abscess and partial removal of the intramedullary lesion. Due to abscess recurrence, she was reoperated with complete excision of the dermal sinus tract, abscess redrainage and subtotal excision of the dermoid cyst (retaining a part of its capsule). Pus culture isolated Corynebacterium species and Peptococcus species and histology of the lesion showed a dermoid cyst. Postoperatively, after an initial neurologic deterioration, she progressively improved. An MRI scan at 15 months neither showed recurrence of the collection nor regrowth of the lesion. Spinal dermal sinus tracts that remain unnoticed or untreated can result in serious complications and should be operated as soon as possible to prevent undesirable sequelae.

A rare case of split pons with double encephalocoele, dermal sinus tract, and lipomeningomyelocele: a case report and review of literature.

INTRODUCTION: Multiple neural tube defects and pontine anomalies are relatively rare. Cases of split pons and double encephalocoeles in combination with other spinal anomalies are even rarer. CASE REPORT: Here, we present a very rare case of split pons, twin encephalocoeles (one of which was atretic), lumbar dermal sinus tract, lumbosacral lipomeningomyelocele, thickened filum, and syringomyelia that was managed successfully at our institute. CONCLUSION: Cases of split pons and double encephalocoeles in combination with other spinal anomalies are very rare, and to the best of author's knowledge, this is the first case report of these multiple anomalies in world literature.

Congenital dermal sinus with an infected dermoid cyst in the cervico-thoracic spinal cord.

Congenital dermal sinuses (CDS) are epithelium-lined tracts that result from incomplete separation of cutaneous ectoderm from the underlying neuroectoderm. CDS may be associated with dermoid cysts and can cause complications by mass effect and by functioning as a pathway for infection. Cervical and thoracic tracts are rare, making up 1 and 10% of all CDS, respectively. We present an unusual case of a cervico-thoracic CDS with concomitant infected dermoid leading to neurological dysfunction. A 1-year-old male with a normal developmental history presented with a several-week history of progressive weakness. Previous visits to two outside emergency departments diagnosed the patient with acute otitis media. After another episode of fever and worsening of neurological symptoms, the patient was correctly diagnosed as having CDS with an infected dermoid cyst. Antibiotics were initiated, the lesion was resected, and the patient improved neurologically. Although cervical and thoracic CDS with infected dermoids are rare, one should have a high index of suspicion when cutaneous stigmata of spinal dysraphism are identified. Due to the risk of neurological deterioration, the recommended treatment of CDS with or without a concomitant intraspinal dermoid is prompt administration of antibiotics and definitive surgical intervention.

Access to the crista galli and the foramen caecum in nasal dermal sinus cysts - lessons learned in a single tertiary care centre.

OBJECTIVE: Several surgical techniques have been suggested for the treatment of nasal dermal sinus cysts (NDSC). We have used several different techniques and have developed a minimally invasive approach. The aim of this study is to describe the evolution to this approach and compare the results with those achieved with our experience of more traditional techniques. METHODOLOGY/PRINCIPAL: A retrospective data collection of patients with NDSC presenting to our clinic between 1998 and 2012 was performed. We initially performed external approaches as outlined elsewhere. With an increasing number of young children requiring surgery, the technique was modified to a less invasive form. This new approach starts with mobilisation of the pit via a tiny skin incision. An open rhinoplasty approach is used to follow the fistula on the nasal bone. Once the fistula passes underneath the nasal bone, an endoscopic endonasal approach is used. Following the fistula cranially, the area of the foramen caecum can be identified. RESULTS: Twelve out of 15 patients (80%) were treated surgically. The transfacial, coronal subcranial and minimally invasive approach was used in 3 (25%), 4 (33%) and 5 patients (42%), respectively. Radical resection was achieved in all patients. Cosmetic problems were present in all patients undergoing a transfacial and in half of the patients after the coronal subcranial approach. Patients treated by the minimally invasive technique remained without sequelae. CONCLUSION: The minimally invasive approach enables a perfect exposure of the fistula up to the crista galli and provides less morbidity and better cosmetic results than the transfacial and subcranial approach.

The longest faun tail forming dreadlocks with underlying spina bifida occulta.

Spina bifida is a developmental anomaly characterized by defective closure of the bony encasement of the spinal cord through which the spinal cord and meninges may or may not protrude. We report a rare case of a very long faun tail, which was in the form of a 20 inch long tail originating from the lumbosacral area in a rhomboidal pattern, measuring 10 x 8 inches. The case is being reported for its rare presentation of a 20 inch long faun tail with underlying spina bifida occulta.

Posterior cervical congenital dermal sinus tract: case report and review of literature.

BACKGROUND AND IMPORTANCE: Congenital dermal sinus tract (DST) is a rare spinal dysraphism characterized by a persistent tract lined by epithelial cells, beginning at the epidermis and terminating in deeper tissue layers. With 1% of all congenital DST cases found in the cervical region, only 4% of all cases are diagnosed after the age of 20. CLINICAL PRESENTATION: In this case, a 65-year-old woman with a congenital DST at the cervical level presented with symptoms of neck and some arm pain, suboccipital headaches, and unique external characteristics. Neck Disability Index and visual analog scale were used to assess the patient's preoperative and postoperative pain, and quality of life. Patient underwent an operative intervention, where the DST was surgically removed followed by interlaminar decompression at C1-C2, excision of the epidural component, and biopsy followed by plastic surgical repair. Pathology analysis indicated a squamous epithelial-lined sinus tract interacting with the dura. Most notably, a meningothelial proliferation with associated psammomatous calcifications was identified, similar to a meningioma. CONCLUSION: A review of literature was conducted to further discuss clinical and radiological presentation as well as to document the novel appearance of this congenital DST. As one of the oldest cases of DST, it demonstrated unusual pathological characteristics with a meningothelial proliferation, compatible with meningioma, reported at the epidural level.

Cranial dermal sinus: presentation, complications and management.

OBJECTIVE: Cranial dermal sinus is a very rare anomaly mostly found in the midline occipital area, presenting with diverse clinical features from asymptomatic dermal sinus to symptomatic situations including meningitis, dermoid tumor or abscess. METHODS: Medical records of 18 patients with 19 cranial dermal sinuses operated at the Children's Hospital Medical Center in Tehran from 1999 to 2011 were retrospectively studied. Their presentations, accompanying anomalies, radiological and surgical findings were evaluated. RESULTS: Patients were aged 1 month to 6 years and were followed for 2-12 years. Most sinuses were located in the midline occipital region. Infection was the most common reason for referral (50%) followed by skin dimple (33.3%). Creamy white discharge was found in 50% of patients. Posterior fossa abscess, hydrocephalus and meningitis were observed in 38.9, 38.9 and 22.2% of children, respectively. All sinus tracts with associated dermoid tumors or abscesses were removed en bloc to decrease the risk of recurrence. CONCLUSION: The sinus tracts are a portal of entry for pathogens which result in serious complications of meningitis or abscess. In this series, 67% of children presented with serious symptoms; therefore, we recommend prophylactic surgery in all cranial dermal sinuses. The exact time of surgery is not obvious as the natural course of the disease is unknown. If any symptom is observed surgery should be done as soon as possible. For asymptomatic sinuses identified by the pediatrician during a regular visit prophylactic surgery is recommended as soon as the child can tolerate operation.

Cranial dermal sinuses in pediatrics: Presentation, diagnosis, complications, and management. Experience at a tertiary care children's hospital. / Senos dérmicos craneales en pediatría: presentación, diagnóstico, complicaciones y tratamiento. Experiencia de un hospital pediátrico de nivel III.

INTRODUCTION: A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complications. There have been isolated reports of this condition. OBJECTIVE: A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complications. There have been isolated reports of this condition. POPULATION AND METHODS: Observational, descriptive study of a series of pediatric patients with cranial dermal sinus treated at a tertiary care children's hospital between 2014 and 2019. RESULTS: Observational, descriptive study of a series of pediatric patients with cranial dermal sinus treated at a tertiary care children's hospital between 2014 and 2019. CONCLUSIONS: In this series, dermal sinuses presented as specific lesions on the scalp. The most common site was the occipital midline, and more than 50% of these extended through the bone. The treatment of choice was complete resection of dermal sinus and associated lesions. An emergency surgery was performed when the dermal sinus was symptomatic or associated with intracranial lesions.

Introducción. El seno dérmico cefálico es un tracto tubular formado por una separación incompleta entre el ectodermo neural y el ectodermo epitelial; puede tener complicaciones infecciosas. Existen reportes aislados de esta patología. OBJETIVO: describir una serie de pacientes con seno dérmico craneal, las formas de presentación, el diagnóstico, el tratamiento y las complicaciones. Población y métodos. Estudio observacional descriptivo de una serie de pacientes pediátricos con seno dérmico cefálico atendidos en un hospital pediátrico de nivel III entre 2014 y 2019. RESULTADOS: se incluyeron 18 pacientes. La clínica de presentación fue, en 12 casos, una lesión puntual en el cuero cabelludo, hipertensión endocraneana en 4 casos, ataxia en 1 caso y lesión puntual con fístula en 1 caso. La mitad presentó síntomas de infección. La localización fue en la línea media sobre el hueso occipital en 13 casos, en la línea media sobre el hueso frontal en 3 casos y en la línea media interparietal en 2 casos. En 5 casos se encontró asociada una imagen extracraneana y, en 11 casos, una complicación intracraneana. Ningún paciente presentó recidiva de la lesión y en todos se realizó un solo procedimiento quirúrgico. CONCLUSIONES: Los senos dérmicos en esta serie se presentaron como lesiones puntuales en cuero cabelludo. La localización más frecuente fue a nivel occipital sobre línea media y, en más de la mitad, atravesaba el hueso. El tratamiento de elección fue la exéresis completa del seno dérmico y las lesiones asociadas. Ante la presencia de senos dérmicos sintomáticos o asociados a lesiones intracraneanas, la cirugía se realizó de urgencia.

Confirmation of microevolutionary increase in spina bifida occulta among Swiss birth cohorts.

Previous studies on the prevalence of spina bifida occulta have indicated a microevolutionary increase in its frequency and possible population differences in the prevalence of the condition. We studied the frequencies of closed and open sacral canals at each sacral level among two birth cohorts in Switzerland. Transverse CT scans and multiplanar reconstruction images of sacra of 95 males and 96 females born in 1940-1950 and 99 males and 94 females born in 1970-1980 in Switzerland were reviewed. We found that individuals born later have significantly more open sacral arches at all sacral levels compared to those born 30-40 years earlier. When results were related to previously published data on Australian cohorts, the trend was the same, but Swiss in both cohorts were less likely to have an open section than Australians at all locations apart from S2. This study confirmed a microevolutionary trend in the opening of sacral canal among two different generations in Switzerland and demonstrated a population difference in the prevalence of spina bifida occulta.

Infected spinal dermal sinus tract with meningitis: a case report.

PURPOSE: Congenital dermal sinus tract (DST), an uncommon entity of cranial or spinal dysraphism, occurs along the midline neuraxis that may arise from nasion and occiput down to the lumbar and sacral region. It is often diagnosed in infants and children for skin signs, neurological deficits, local infection, meningitis, or abscess. For spinal DST, there is a paucity of case or series report in Taiwan. CASE REPORT: In this paper, we report a case in a 6-year-old girl. The girl presented with midline lumbar skin dimple, hypertrichosis, and history of bacterial meningitis. She was successful treated by surgical excision of the DST with local infection that ended within the subarachnoid space between L2-3 vertebrae. CONCLUSION: This case highlights the importance of a thorough examination of the midline craniospinal axis in children with meningitis or history of meningitis.

Unusual CNS infection from a subtorcular dermal sinus.

UNLABELLED: A 14-month-old toddler with a previously unrecognized dermal sinus tract presented with hydrocephalus and raised intracranial pressure. CT and MRI revealed a large posterior fossa dermoid mass. Cultures obtained from CSF and posterior fossa subdural empyema were positive for both Staphylococcus aureus and Escherichia coli. To the authors's knowledge, this is the first such report. CONCLUSION: This case suggests that children who present with CNS infections with unusual pathogens should be carefully evaluated for the presence of anatomic abnormalities including dermal sinus tracts.