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.
Male ; Female ; Humans ; Adult ; Spina Bifida Occulta/surgery* ; Retrospective Studies ; Neural Tube Defects/surgery* ; Cauda Equina/surgery* ; Spinal Cord/abnormalities* ; Magnetic Resonance Imaging/methods* ; Pain

Infantile hemangioma (IH) usually presents solely as a cutaneous manifestation, and rarely accompanies diverse anomalies such as spinal dysraphism. A 2-month-old girl presented with IH on her lumbar skin as a coin-sized red plaque with adjacent depressed skin and a child-palm-sized red plaque on her left ankle since birth. Considering the coexistence of IH and depressed skin on the midline in her lumbosacral area, magnetic resonance imaging of her spine was performed, which showed intraspinal/dermal vascular tumors with spina bifida occulta at the 12th thoracic vertebrae level. Furthermore, no neurologic deficits were observed. She has been taking oral propranolol with topical timolol to prevent neural complications and the lesions clinically improved. However, additional surgery for the intraspinal lesions was considered due to urination/defecation abnormalities since she was 13 months of age. In cases of midline IH, particularly with additional skin lesions, appropriate imaging studies to identify accompanying anomalies should be performed, and referrals to neurosurgical specialists should be considered.
Ankle ; Female ; Hemangioma ; Humans ; Infant ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Parturition ; Propranolol ; Referral and Consultation ; Skin ; Specialization ; Spina Bifida Occulta ; Spinal Dysraphism ; Spine ; Thoracic Vertebrae ; Timolol

PURPOSE: To investigate the prevalence of spina bifida occulta (SBO) and its relationship with the presence of overactive bladder (OAB) in middle-aged and elderly people in China. METHODS: A cross-sectional community-based survey was carried out at 7 communities in Zhengzhou City, China from December 15, 2013 to June 10, 2014, where residents aged over 40 years were randomly selected to participate. All of the participants underwent lumbosacral radiographic analysis and relevant laboratory tests. A questionnaire including basic information, past medical history and present illness, and the OAB symptom score was filled out by all participants. Chi-square tests and logistic regression were used for data analysis with a P-value of <0.05 denoting statistical significance. RESULTS: A total of 1,061 subjects were qualified for the final statistical analysis (58.8±11.7 years; male, 471 [44.4%]; female, 590 [55.6%]). The overall prevalence of SBO was 15.1% (160 of 1,061): 18.3% (86 of 471) in men and 12.5% (74 of 590) in women. Among these subjects, 13.7% (145 of 1,061) had OAB: 13.2% (62 of 471) in men and 14.1% (83 of 590) in women. The results of logistic regression showed that age, SBO, history of cerebral infarction (HCI), and constipation were risk factors for OAB (P<0.05), while sex, history of childhood enuresis (HCE), body mass index (BMI), and diabetes mellitus (DM) were not (P>0.05). In men, age, SBO, and constipation were risk factors for OAB (P<0.05), while HCE, BMI, DM, HCI, and benign prostate hyperplasia were not (P>0.05). In women, age, SBO, and HCI were risk factors for OAB (P<0.05), while HCE, BMI, DM, vaginal delivery, and constipation were not (P>0.05). CONCLUSIONS: The prevalence of SBO is high and it is related to OAB in middle-aged and elderly people in China.
Aged* ; Asian Continental Ancestry Group* ; Body Mass Index ; Cerebral Infarction ; China ; Constipation ; Diabetes Mellitus ; Enuresis ; Female ; Humans ; Hyperplasia ; Logistic Models ; Male ; Prevalence* ; Prostate ; Risk Factors ; Spina Bifida Occulta* ; Spinal Dysraphism* ; Statistics as Topic ; Urinary Bladder, Overactive*

Spinal dermal sinus tract (DST) is a rare congenital dysraphism that occurs in approximately one in every 2,500 live births. Many studies have reported that cutaneous stigmata overlying the posterior midline neuroaxis may be associated with dermal sinuses, including abnormal pigmentation, hemangioma, hypertrichosis, and benign sacrococcygeal dimples. Spinal DSTs that are connected with sinuses may have diverse clinical presentations and may involve clinical complications, occasionally caused by a neurologic defect, or infectious complications, including life-threatening conditions such as meningitis. A 3-month-old girl had experienced recurrent meningitis without typical abnormal findings on physical and laboratory examinations. Magnetic resonance imaging (MRI) was performed to evaluate infectious conditions, and DST was found incidentally. Although MRI findings revealed DST, we could not find any small opening on the skin on careful observation. Thus, first-line physicians should keep several important factors in mind when initially evaluating patients with recurrent meningitis, such as cutaneous findings over the midline neuroaxis and early neuroimaging studies for congenital dysraphism.
Christianity ; Female ; Hemangioma ; Humans ; Hypertrichosis ; Infant* ; Infant, Newborn ; Live Birth ; Magnetic Resonance Imaging ; Meningitis* ; Neuroimaging ; Pigmentation ; Skin ; Spina Bifida Occulta*

Congenital dermal sinus (CDS) is a type of occult spinal dysraphism characterized by a midline skin dimple. A 12-month-old girl presented with fever and ascending quadriparesis. She had a midline skin dimple in the upper sacral area that had been discovered in her neonatal period. Imaging studies revealed a holocord intramedullary abscess and CDS. Overlooking CDS or misdiagnosing it as benign sacrococcygeal dimple may lead to catastrophic infection and cause serious neurological deficits. Therefore, further imaging work-up or consultation with a pediatric neurosurgeon is recommended following discovery of any atypical-looking dimples in the midline.
Abscess* ; Diagnosis ; Female ; Fever ; Humans ; Infant ; Neural Tube Defects ; Quadriplegia ; Skin ; Spina Bifida Occulta*

PURPOSE: Sacral cutaneous lesions in newborns are associated with numerous spinal abnormalities. Early detection is important, because spinal abnormalities may cause neurological symptoms. Radiologic screening tests have been performed on newborns with sacral cutaneous lesions. This study aimed to substantiate the associations between sacral cutaneous lesions and spinal abnormalities. METHODS: From January 2007 until November 2013, we retrospectively reviewed the charts of 743 newborns with sacral cutaneous lesions that included sacral dimples, which were deeper than 5 mm and situated further than 2.5 cm from the anus, deviated gluteal furrow, hairy patch, hemangioma, dyspigmentaion, and the presence of mass, and skin tag. RESULTS: 743 newborns with sacral cutaneous lesions were examined, including 24 newborns with abnormal ultrasonographic images. Tethered cord which affected 18 (2.4%) of the newborns, was the most commonly found spinal abnormaility. Of these 9 newborns had other spinal abnormalities in addition to tethered cord including lipoma, cyst, spina bifida occulta, lipomyelomeningocele, and dermal sinus tract, and 9 newborns had isolated tethered cord only. Other spinal abnormalities found included isolated lipoma (3 newborns, 0.4%), and subarachnoid cyst (2 newborns, 0.3%), and of the 2 newborns (0.3%) who had dermal sinus tract, 1 also had a lipoma and the other also had a tethered cord. Normal variants included coccygeal pit (43 newborns, 5.8%), and ventriculus terminalis (10 newborns, 1.4%). Of the 646 newborns with isolated sacral cutaneous lesion, 11 (1.7%) had abnormal ultrasonographic images, and of the 97 newborns with combined sacral cutaneous lesions, 13 (13.4%) had abnormal ultrasonograpic images. CONCLUSION: Sacral cutaneous lesions in newborns can be associated with spinal abnormalities, and the strongest marker of spinal abnormality is a combined lesion. Therefore, ultrasonography should be performed on newborns who present with sacral cutaneous lesions to detect and investigate any underlying spinal abnormalities.
Anal Canal ; Hemangioma ; Humans ; Infant, Newborn ; Lipoma ; Mass Screening ; Retrospective Studies ; Skin ; Spina Bifida Occulta ; Ultrasonography

PURPOSE: Congenital spinal dermal sinus tract is a rare lesion connecting skin to deeper structures including neural tissue. It results from the failure of the neuroectoderm to separate from the cutaneous ectoderm in the third to fifth week of gestation. The common locations are the lumbosacral and occipital regions. Sometimes it extends to spinal canal. In this paper we report a case of congenital spinal dermal sinus tract in the coccyx. METHODS: A 21-month-old male child born after an uncomplicated full-term pregnancy was admitted to our institute with a midline dermal sinus and a cartilaginous protrusion in the coccygeal region. There were no signs of infection. Neurologic examination showed no functional deficit in both lower limbs. He was treated with complete excision of the tract and an underlying accessory cartilage. RESULTS: The spinal dermal sinus tract was extended from the skin to the coccyx. The stalk was loosely attached to the accessory cartilage of coccyx. At that point, it was dissected from the accessory cartilage and resected. The accessory cartilage was also resected at the bone and cartilage junction. During the follow-up period of 6 months, the wound healed well without any complication nor recurrence. CONCLUSION: Congenital spinal dermal sinus tract is known as a form of spinal dysraphism. In order to prevent complications, timely surgical intervention including complete resection of sinus tract with correction of associated abnormalities is of utmost importance.
Cartilage ; Child ; Coccyx ; Ectoderm ; Follow-Up Studies ; Humans ; Infant ; Lower Extremity ; Male ; Neural Plate ; Neurologic Examination ; Pregnancy ; Recurrence ; Sacrococcygeal Region ; Skin ; Spina Bifida Occulta ; Spinal Canal ; Spinal Dysraphism

Intracranial dermoid cyst is a rare congenital disease originating in the embryo. Intracranial dermoid cysts are usually detected before five years of age; however, asymptomatic cysts have been discovered after the third decade of life through various complications. Sometimes, there are neurologic symptoms caused by a cystic mass effect and if there is a dermoid sinus, purulent infections can occur. Early diagnosis is important and as such, CT or MR imaging for recognition of the location, size, and characteristics of the cysts are recommended before surgical resection. Extradural dermoid cysts with an intact occipital dermoid sinus are a rare event. We report a case of intracranial dermoid cyst associated with scalp abscess. In this case, a three-year-old boy was presented with scalp abscess and we performed MR imaging, which revealed a cystic region. The prognosis after resection operation was good without any complication.
Abscess ; Dermoid Cyst ; Early Diagnosis ; Embryonic Structures ; Neurologic Manifestations ; Prognosis ; Scalp ; Spina Bifida Occulta

Intramedullary spinal abscesses are rare and potentially devastating lesions. The first case of spinal abscess was described in 1830 and fewer than 100 cases have been reported subsequently. Only sixteen previous reports have described an intramedullary abscess of the central nervous system secondary to a dermal sinus. Involvement of the entire cord is thus exceedingly rare. We present a case of a 7-month-old girl who had rapidly progressing quadriplegia with urinary and bowel retention and was found to have an intramedullary abscess as a result of a dermal sinus. Spine MRI shows thick irregular ring enhancement appeared within the broadest area of the spinal cord on the thoracic and lumbar area on mid thoracic level to L3 and high cervical area which extended to medulla. Immediate decompressive surgery and antibiotic treatment were performed. Excellent clinical outcome was obtained with a combination of medical and surgical management. Complete neurological assessment and diagnostic study of all patients with a congenital dermal sinus are very important. Prophylatic surgery is indicated in many cases to prevent dangerous and recurrent infections of the central nervous system.
Abscess ; Central Nervous System ; Humans ; Infant ; Quadriplegia ; Retention (Psychology) ; Spina Bifida Occulta ; Spinal Cord ; Spine

Child, Preschool ; Diagnostic Errors ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Spina Bifida Occulta ; diagnosis