Sacrococcygeal teratoma is a rare tumor, it occurs once in every 40,000 live births. Most of the reported cases appear in female infant (M:F=1:4). They deserve clinical attention because they are potentially malignant and are curable if diagnosed and treated early. The patients may have associated congenital anomalies. This patient has spina bifida cystica in lower sacrum. Brief review of related literature is included in the report.
Female ; Humans ; Infant ; Infant, Newborn* ; Live Birth ; Sacrum ; Spina Bifida Cystica* ; Spinal Dysraphism* ; Teratoma*

OBJECTIVE: There are two types of spina bifida aperta: cystic type and flat type. Unruptured cystic sac of spina bifida may interfere free leakage of cerebrospinal fluid into amnionic fluid space. Therefore its presence could influence the appearance of the intracranial signs on prenatal ultrasound. The purpose of this study was to define the ultrasound findings causing the appearance of indirect intracranial signs. METHODS: Twenty-one fetuses with spina bifida were evaluated from Jan/1998 to March/2004. Incidence of indirect cranial signs (changes of ventricular width, morphologic change of cerebellum, obliteration of cisterna magna, decreased biparietal diameter and lemon sign) were evaluated according to gestational age at diagnosis, size, location and morphology of the spinal lesion. RESULTS: Meningeal sac was visible in 10 cases (48%), flat form in 11 cases (52%) on prenatal ultrasound. All of the intracranial signs seemed to appear more frequently on flat spina bifida, but there were no statistical significance. CONCLUSION: It seems like that the presence of the sac in spina bifida aperta did not interfere the appearance of intracranial signs on prenatal ultrasound.
Amnion ; Cerebellum ; Cerebrospinal Fluid ; Cisterna Magna ; Diagnosis ; Fetus* ; Gestational Age ; Incidence ; Neural Tube Defects* ; Neural Tube* ; Spina Bifida Cystica ; Spinal Dysraphism ; Ultrasonography

This 22-year-old female patient has had a lozenge shaped hair tuft with terminal hair on the lumbosacral area since birth. A limping gait due to discrepancy of the length of the lower extremities has also been noted since 5 year age. Subsequently multiple, recurrent trophic ulceration developed on the left foot because of sensory loss. Simple cervico-lumbar spine x-ray showed spina bifida occulta of L-2 to sacrum. Lumbar myelography revealed widened vertebral canal without other spinal anomaliea.
Female ; Foot ; Gait ; Hair ; Humans ; Lower Extremity ; Myelography ; Nevus* ; Parturition ; Sacrum ; Spina Bifida Occulta* ; Spinal Dysraphism* ; Spine ; Ulcer ; Young Adult

STUDY DESIGN: We established induced pluripotent stem cells (iPSCs) and neural stem/progenitor cells (NSPCs) from three newborns with spina bifida aperta (SBa) using clinically practical methods. PURPOSE: We aimed to develop stem cell lines derived from newborns with SBa for future therapeutic use. OVERVIEW OF LITERATURE: SBa is a common congenital spinal cord abnormality that causes defects in neurological and urological functions. Stem cell transplantation therapies are predicted to provide beneficial effects for patients with SBa. However, the availability of appropriate cell sources is inadequate for clinical use because of their limited accessibility and expandability, as well as ethical issues. METHODS: Fibroblast cultures were established from small fragments of skin obtained from newborns with SBa during SBa repair surgery. The cultured cells were transfected with episomal plasmid vectors encoding reprogramming factors necessary for generating iPSCs. These cells were then differentiated into NSPCs by chemical compound treatment, and NSPCs were expanded using neurosphere technology. RESULTS: We successfully generated iPSC lines from the neonatal dermal fibroblasts of three newborns with SBa. We confirmed that these lines exhibited the characteristics of human pluripotent stem cells. We successfully generated NSPCs from all SBa newborn-derived iPSCs with a combination of neural induction and neurosphere technology. CONCLUSIONS: We successfully generated iPSCs and iPSC-NSPCs from surgical samples obtained from newborns with SBa with the goal of future clinical use in patients with SBa.
Cells, Cultured ; Ethics ; Fibroblasts ; Humans ; Induced Pluripotent Stem Cells* ; Infant, Newborn* ; Meningomyelocele ; Plasmids ; Pluripotent Stem Cells ; Regenerative Medicine ; Skin ; Spina Bifida Cystica* ; Spinal Cord ; Spinal Dysraphism* ; Stem Cell Transplantation ; Stem Cells

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

Spinal dysraphism, first described by Lichtenstein in 1940, is a congenital anom- aly due to incomplete fusion or malformation of the midline dorsal embryonic structures including the ectoderm, mesoderm, and neuroectoderm. Spina bifida occulta is a spinal dystraphism and a manifestation of a midline defect of osseous spine and related structures without cyst formation. Spinal dysraphism may be associated with hypertrichosis, lipoma, dimpling, pigmentation, hemangioma, congenital scar, sinus, cyst, or skin defect in the midline dorsal area. We observed a 23-year-old female patient with spina bifida occulta who had a blue scar surrounded by circumscribed hair growth of the lumbosacral area since 1-montb-old age. Histopathologic finding of the blue scar revealed common blue nevus showing intradermal melanocytes and melanophages. Spine X-ray showed hemivertebra of L5 and spina bifida occulta of Sl. There were no abnormal neurologic signs.
Cicatrix ; Ectoderm ; Embryonic Structures ; Female ; Hair ; Hemangioma ; Humans ; Hypertrichosis* ; Lipoma ; Melanocytes ; Mesoderm ; Neural Plate ; Neurologic Manifestations ; Nevus, Blue* ; Pigmentation ; Skin ; Spina Bifida Occulta* ; Spinal Dysraphism* ; Spine ; Young Adult

Acute bacterial meningitis is an uncommon, potentially life-threatening infection and recurrent episodes of bacterial meningitis are rarely seen. But when they occur, an exhaustive search for the mechanisms underlying the recurrent episodes must be pursued, especially some possible routes of migration of bacteria to the cerebrospinal fluid. Dermal sinus tracts are common skin manifestations seen with spinal dysraphism and may be associated with recurrent meningitis. Here, we present a case of a 2-month-old girl who had a small dimple on the lower lumbosacral area. She was attacked twice by purulent bacterial meningitis due to a dermal sinus tract and a tethered cord. She was treated with the excision of the tract, the removal of the dermoid and the detethering of the cord. Staphylococcus and Klebsiella were cultured separately and assumed to be causative agents. The lesion was suspected on the physical examination and demonstrated by lumbosacral magnetic resonance imaging.
Bacteria ; Cerebrospinal Fluid ; Dermoid Cyst ; Female ; Humans ; Infant ; Klebsiella ; Magnetic Resonance Imaging ; Meningitis ; Meningitis, Bacterial* ; Physical Examination ; Skin Manifestations ; Spina Bifida Occulta* ; Spinal Dysraphism ; Staphylococcus

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

A myelomeningocele with spina bifida in the thoracic region is a relatively rare, congenital anomaly. This case, which we have experienced recently, is reported with a review of the literatures.
Meningomyelocele* ; Spinal Dysraphism

With advancements in corrective surgery for spina bifida since the 1960s, affected women are now reaching adulthood and achieving pregnancies. However, the implications on reproductive health?especially in pregnancy?are rarely studied. We are presenting a case of a woman born with spina bifida who has undergone surgical repair and closure of the defect at 4 years old, as well as surgical management for tethered cord at 13 years old. She lived productively into adulthood, became pregnant and delivered vaginally to a healthy baby despite various obstetrical challenges. In the care for this patient, we faced recurrent urinary tract infections, the risk of preterm delivery and the task of coordinated multidisciplinary care to solve dilemmas in decision making. Through this case report, we were able to share our experience, explore the most recent evidence to support our clinical decisions and hopefully serve as a basis for future clinical practice recommendations.
Pregnancy ; Female ; Spinal Dysraphism