Local conceptions of the role of folate in neural tube defects in Zambia.

OBJECTIVE: In sub-Saharan Africa, neural tube defects (NTDs) are the second most common birth defect, occurring eight times more frequently than in the US. The objective of this study was to assess baseline Zambian caregiver understanding of folate and NTDs and the effectiveness of an NTD prevention educational program. METHODS: This prospective survey-based study included Zambian caregivers of children born with NTDs who completed pre- and post-educational program surveys between January 2020 and January 2021. The verbal survey was administered in English or local Zambian dialects. The 1-hour educational program administered by local Zambian research nurses sought to facilitate understanding of the direct relationship between prenatal folate supplementation and NTDs. RESULTS: Sixty-one eligible caregivers with a median age of 20 (IQR 24-29) years completed the survey. Participants were predominantly from regions outside of Lusaka Province (68%, 41/60) rather than the capital city, Lusaka (32%, 19/60). Most had received prenatal care (91%, 57/61), and 80% (47/59) reported folate use in pregnancy. Of the mothers who took folate during pregnancy, 24% (11/45) reported use within the first 4 weeks after conception, while 76% (34/45) started thereafter. Myelomeningocele was the most common NTD (74%, 32/43), followed by meningocele (14%, 6/43). Prior to the educational program, 52% (29/56) of caregivers reported that NTDs were caused by a vitamin deficiency, which improved to 98% (55/56) after the program (p < 0.001). Furthermore, only 54% (33/61) of caregivers believed that folate should be taken before conception on the baseline survey evaluation, which improved to 95% (58/61, p < 0.001) after the program. All survey participants (58/58) found the educational session helpful. CONCLUSIONS: This study found that a high proportion of Zambian caregivers had received prenatal care and even had taken folate during pregnancy, but none had taken it prior to pregnancy. An educational program effectively improved understanding about the role and timing of perinatal folate administration in NTD prevention. This result also emphasizes the need for folate fortification and folate education for not only mothers but also primary care providers.

Sensory neuronopathy in a patient with neurofibromatosis type 1: A case report.

RATIONALE: Neurofibromatosis type 1 (NF-1) can manifest with various neurological symptoms. However, sensory ataxia has not been reported. PATIENT CONCERNS: A 44-year-old man with NF-1 presented with several weeks of unsteady gait. He was diagnosed with gastric neuroendocrine tumor with multiple hepatic metastases 6 years ago and received palliative chemotherapy. Neurological examination revealed ataxia veering to the right side with no motor weakness. DIAGNOSES: Clinical manifestations and electrodiagnostic studies suggested the dysfunction of the thoracic dorsal column (DC). Initial magnetic resonance imaging showed a lateral thoracic meningocele (LTM) located in the right paravertebral area at the T3-T4 vertebral level, but the spinal cord was unremarkable. Gait disturbance worsened after 9 months, and follow-up magnetic resonance imaging showed high signal intensity involving the right DC at the level adjacent to the LTM and spinal cord atrophy distal to the DC lesion. Tests for well-characterized paraneoplastic antibodies were negative. Ultimately, the patient was assumed to have sensory neuronopathy due to compressive damage to the dorsal root ganglia within the intervertebral foramina by LTM. INTERVENTIONS: Empirical treatment with vitamin B12 supplementation and corticosteroids failed to improve his condition. The patient underwent decompressive laminectomy and excision of the meningocele with dura repair. OUTCOMES: The patient temporarily improved to walk with assistance postoperatively. However, he developed dyspnea and hypotension 5 weeks later. Carcinoid heart disease confined the patient to the bed. The patient died of pneumonia 3 months after the operation. LESSONS: This case with NF-1 shows asymmetric sensory ataxia of subacute progression. LTM may contribute to the development of sensory neuronopathy by damaging sensory neurons of the dorsal root ganglia. The comorbidities of the patient, including gastric neuroendocrine tumor and LTM, made it challenging to investigate the pathomechanism.

Awareness of spina bifida among family of affected child. A cross sectional questionnaire.

OBJECTIVES: To evaluate the awareness of spina bifida (SB), risk factors and possible preventive measures among mothers who had been following in our clinic with a spina bifida affected child. Methods: A cross-sectional questionnaire-based study with 38 mothers of SB patients who are following at SB and hydrocephalus clinic at King Fahad University Hospital, Khobar, Eastern Province, Saudi Arabia. RESULTS: Thirty-eight mother were included in this questionnaire. Most of the participants were Saudi (94.7%). Ten out of 38 women (26.3%) had used medications during pregnancy (pain medications and herbal drugs), 4 out of 38 (10.5%) had been exposed to imaging radiation, while 9 (23.7%) had experienced moderate to high grade fever (39-41°C) during pregnancy. Moreover, the majority (86.8%) of these women did not receive folic acid (FA) before pregnancy, and 42.1% of them did not have FA during their first trimester. Only one (2.6%) gave positive family history of SB while, 6 (15.8%) reported having other SB children.  Conclusion: There is a considerably low level of awareness in mothers of SB patients despite prevalence of this anomaly in the Eastern province. This necessitates an effort from health care providers to educate the community about this birth defect entity. Furthermore, genetic counseling should be encouraged especially in those who have a positive familial history for better understanding. Also, larger sample size with randomized controlled trials and larger epidemiological studies should be implemented.

OPTIC NERVE MENINGOCELE SIMULATING EXTRAOCULAR EXTENSION OF CHOROIDAL MELANOMA.

PURPOSE: To report a case of optic nerve meningocele simulating massive, recurrent extraocular extension of choroidal melanoma. METHOD: Case report. RESULTS: A 53-year-old white man with choroidal melanoma in his left eye of 7.3-mm thickness was treated with plaque radiotherapy and transpupillary thermotherapy. On 1-year follow-up examination, visual acuity was 20/20 in the right eye and 20/30 in the left eye. The regressed choroidal melanoma scar in the left eye measured 1.5 mm in thickness with stable margins. The optic disk was normal. Ultrasonography demonstrated regressed echogenic choroidal scar, with an echolucent multilobulated retrobulbar mass, suspicious for extraocular extension. On magnetic resonance imaging, the retrobulbar mass corresponded to a distended and kinked optic nerve sheath, filled with extensive subarachnoid fluid and normal-size optic nerve with apposition against the posterior globe. There was no extraocular extension of tumor. Similar but less distended right optic nerve sheath was documented, consistent with optic nerve sheath meningocele in both eyes. Observation was advised and the findings remained stable. CONCLUSION: Optic nerve sheath meningocele is a benign dilatation of the optic nerve sheath that can simulate orbital tumor or extraocular extension of intraocular tumor. Magnetic resonance imaging can reliably differentiate these conditions.

Sacral nerve stimulation lead implantation in partial sacral agenesis using intra-operative computerized tomography.

AIM: Sacral nerve stimulation (SNS) lead implantation is a straightforward procedure for individuals with intact spinal vertebrae. When sacral anomalies are present, however, the anatomical and radiological reference points used for the accurate placement of the electrode may be absent or difficult to identify. METHOD: We describe an innovative surgical procedure of percutaneous nerve evaluation for SNS in a patient with faecal incontinence secondary to a congenital imperforate anus and partial sacral agenesis using a surgical imaging platform (O-arm system) under neurophysiological control. RESULTS: Using intra-operative CT and neuronavigation, the insertion point at the skin was identified. The lead was introduced into the right-sided S3 foramen and placed at the correct depth. An appropriate motor response was obtained after stimulation and neurophysiological control confirmed that the right S3 root was being stimulated. CONCLUSION: Our experience showed that O-arm guided navigation can be used to overcome the difficulty of SNS lead placement in patients with partial sacral agenesis who have faecal incontinence.

Novel Use of Pudendal Neuromodulation in a Pediatric Patient With Caudal Regression and Partial Sacral Agenesis for Refractory Bowel Bladder Dysfunction.

Sacral neuromodulation (SNM) has been used off-label in the United States for over a decade in the pediatric population. Many published studies have demonstrated efficacy with SNM in this population; however, a significant number of children with refractory bowel bladder dysfunction (BBD) also have underlying comorbidities. Children with certain spinal abnormalities pose a problem for the urologist treating BBD. Patients with caudal regression can have various sacral anomalies, making SNM challenging or impossible. We present the first case in the United States of pudendal neuromodulation in a pediatric BBD patient with caudal regression.

PATTERNS OF NEURAL TUBE DEFECTS AT TWO TEACHING HOSPITALS IN ADDIS ABABA, ETHIOPIA A THREE YEARS RETROSPECTIVE STUDY.

BACKGROUND: Neural tube defects (NTDs), one of the most common congenital malformations, are potentially preventable cause of perinatal morbidity and mortality. OBJECTIVES: To give baseline description of NTDs and their outcome at two teaching hospitals in Addis Ababa, Ethiopia. MATERIALS AND METHODS: A retrospective cross sectional descriptive study conducted from September 2009 to August 2012. RESULTS: During the study period out of 28,961 deliveries 177 cases of NTDs were identified, giving an overall NTD prevalence of 6.1/1000. Only 12% (21/177) were diagnosed before 28 weeks of gestation. The mean gestational age at diagnosis of NTDs was 33.8 weeks (±5.5). Majority, 93.2% (165/177), had antenatal care (ANC) follow-up. Most, 72% (127/177), were diagnosed by ultrasound before delivery while 28% (50/177) were identified at the time of delivery or expulsion. Majority, 85.3% (151/177), never received folic acid supplementation. Only less than 1% (2/177) of the mothers started taking folic acid supplementation pre-conceptionally. Only a third, 33.3% (59/177), of the fetuses were born alive while only 13.6% (24/177) were discharged alive. Myelomeningocele, identified in 51.4% (91/177), was the commonest NTD in this study. CONCLUSION AND RECOMMENDATIONS: The proportion of NTDs in this study is among the highest globally reported rates. The practice of periconceptional folic acid supplementation is negligible. And although most had ANC follow-up the vast majority of NTDs were diagnosed late in the third trimester. It is, therefore, highly recommended to consider implementing national preventive strategies to reduce the prevalence of NTDs in Ethiopia.

The cephaloceles: A clinical, epidemiological and therapeutic study of 50 cases.

INTRODUCTION: Cephaloceles are congenital malformations characterized by externalization of the meninges and/or brain tissue through a congenital skull bone defect. In developed countries, this malformation is rare but in our developing countries, this pathology remains one of our concerns. OBJECTIVE: To describe the epidemiological, clinical and therapeutic aspects of the cephaloceles. MATERIALS AND METHOD: A retrospective study was conducted from 2007 to 2013 on all cases of cephalocele supported in the department of neurosurgery of the Yalgado Ouédraogo University Teaching Hospital of Ouagadougou. The malformations were supplemented by CT scan. All patients were operated. The surgical procedure in cephaloceles of the convexity consisted of a direct approach. Sincipital cephaloceles were operated by transcranial approach or combined approach associating transcranial and transnasal approaches. RESULTS: Fifty patients were gathered during the 7-year period. There were 18 boys and 32 girls. The ages ranged from 1 day to 11 years. The cephaloceles were located on the vault of the skull in 78%; the lesion was sincipital in 22%. The malformation was covered with normal skin in 92%; it was pediculated in 78%. CT scan allowed the classification of cases as meningo-encephaloceles in 31 cases (62%) and meningoceles in 18 cases (36%) and one pure encephalocele. The immediate postoperative course was uneventful in 42 cases (84%); 8 postoperative deaths were recorded. At medium and long term, 4 patients (8%) developed complications of psychomotor deficiency in 3 cases and hydrocephalus in 1 case. The main reasons for the poor prognosis were superinfection, hydrocephalus and/or other brain malformations. CONCLUSION: Cephaloceles remain relatively frequent in our practice. After surgery, mental failure and hydrocephalus can occur impairing the prognosis. The most suitable solution is a policy of prevention with folic acid treatment before and during pregnancy and following up adequate pregnancies.

Currarino's syndrome in twins presenting as neonatal intestinal obstruction--identical presentation in non-identical twins.

We report a case of non-identical twins who presented with identical neonatal intestinal obstruction with features of anorectal stenosis, presacral mass and sacral anomaly consistent with Currarino's syndrome or triad. Plain sacral radiograph, contrast enema and MRI were diagnostic. Initial management involved a defunctioning colostomy followed by a posterior sagittal anorectoplasty with excision of the teratoma ± anterior sacral meningocele and finally closure of colostomy in a staged multidisciplinary approach. The twins' father is also affected with features of Currarino's syndrome but was diagnosed during family screening. Currarino's syndrome presenting with identical neonatal low intestinal obstruction in a non-identical set of twins is rare and interesting. Antenatal diagnosis of Currarino's syndrome is difficult and may prove to be a challenge even in the postnatal period. Sacral spine radiograph, contrast enema and MRI are diagnostic. Management requires high index of suspicion, low threshold for MRI and multidisciplinary staged approach.

[Cervical meningocele with filiform connection. Case report]. / Meningocele cervical con conexión filiforme. Reporte de un caso.

The meningocele is the least common form of spina bifida cystica and represents less than 10% of cases of this disorder. A case of a female patient aged 26 with a history of two pregnancies and two previous cesarean sections, present pregnancy unplanned, uncontrolled during the first trimester prenatal and without supplementation with folic acid. Family history of consanguinity with her husband (second cousins) and psychomotor reassessed in four maternal cousins. The first follow-up visit the patient was at 34 weeks of pregnancy. The ultrasonographic findings were: cervical meningocele posterior filiform connection between the first and second cervical vertebrae, lateral ventriculomegaly and third and fourth ventricles and hydrocephalus secondary. Cesarean section was performed at 37 weeks gestation and was a newborn male 3.000 g, 52 cm, head circumference of 36 cm, Apgar 8/9, Capurro 37 weeks of gestation. In the posterior cervical region tumor was located a soft 5 x 5 cm with intact skin, adhered to deep planes. Movement of all four extremities without neurological involvement. He referred to the department of neurosurgery for shunt placement and subsequently performed surgical excision of the meningocele.

Cystoperitoneal shunt for a giant intrathoracic meningocele under local anesthesia.

Giant intrathoracic meningoceles are extremely rare, and the standard treatment for giant intrathoracic meningoceles remains controversial. We present the case of a patient with giant intrathoracic meningoceles associated with neurofibromatosis type I. Our patient had poor respiratory function because of the giant intrathoracic meningocele, so we performed a cystoperitoneal shunt under local anesthesia. We describe our cystoperitoneal shunt technique using an adjustable-pressure valve. This simple, minimally invasive treatment is a valuable alternative treatment option in patients at high operative risk, especially those with low respiratory function.

[Electrophysiologial evaluation of ozone in treating of lumbar disc herniation with curving sheath-needle multi-direction rotating injection].

OBJECTIVE: To compare electrophysiological changes in treating lumbar disc herniation (LDH) with ozone by curving sheath-needle multi-direction rotating injection (CSNMRI) and conventional injection method. METHODS: From May 2005 to June 2009,100 patients with LDH were studied, included 68 males and 32 females, ranging in age from 25 to 58 years with an average of 44 years, in course of disease from 3 months to 8 years with an average of 8.8 months. All patients were numbered according to sequence of visit, and were completely randomly divided into group A and group B with DPS software, 50 cases in each group. All patients were injected ozone into lesion of intervertebral disc, in group A with CSNMRI and in group B with conventional method. The electrophysiologic study of all patients was performed respectively before treatment and at the 3 month after treatment. The electromyogram (EMG) of the main muscle groups of involved lower limb and the corresponding segments of sacrospinal muscle was tested; the duration and multiphase-wave rate of MUP were calculated. H-reflex of tibial nerve in both lower limbs was observed and the number of abnormal H-reflex and the H-wave latency were recorded. RESULTS: After treatment, the number of muscles with abnormal EMG was reduced to different degrees in each group, but there was more significant reduction in group A (P < 0.05 or 0.01); the duration and multiphase-wave rate of MUP in the two groups were both reduced and close to the normal level (P < 0.01), yet the changes in group A was more than that of group B (P < 0.05 or 0.01). There was no significant difference in the number of abnormal H-reflex before treatment between two groups, whereas was markedly lower in group A than that of group B after treatment (P < 0.05). After treatment, H-wave latency in two groups was shortened and become close to normal, but group B was more statistically significant than group B (P < 0.05). CONCLUSION: The neural electrophysiological abnormalities can reflect the degree of nerve root compression and damage, and is one of the objective indicators to estimate neuromuscular function. It can better meliorate abnormal electrophysiology to inject ozone to treat LDH with CSNMRI than conventional method.

Equoterapia na reabilitação da meningoencefalocele: estudo de caso / Hyppotherapy in meningoencephalocele rehabilitation: a case study

A equoterapia é uma das técnicas de reabilitação utilizada para pacientes com disfunções neurológicas. Os movimentos provocados pelo cavalo no corpo do paciente podem interferir positivamente em seu controle postural, melhorando a mobilidade geral e o desempenho motor funcional. O objetivo deste estudo foi verificar o efeito da equoterapia no equilíbrio, coordenação motora e funcionalidade de uma criança com meningoencefalocele. Uma menina de 3 anos e 6 meses foi submetida a 18 sessões de equoterapia (em três fases - alimentação, escovação e montaria) realizadas uma vez por semana. Para a avaliação foram utilizadas as escalas de equilíbrio de Berg e Tinneti e o Inventário de avaliação pediátrica de incapacidade (PEDI) para habilidades motoras gerais e funcionais, aplicados antes e após o período de terapia; e uma reavaliação foi feita oito semanas (sem intervenção) depois. A equoterapia melhorou de maneira significativa o equilíbrio e a coordenação motora da criança, o que se refletiu no controle de movimentos funcionais básicos para a realização de atividades de vida diária. O ganho funcional também foi percebido pela mãe da criança. Esse estudo oferece uma nova perspectiva sobre o uso da equoterapia como modalidade fisioterapêutica na reabilitação motora e funcional de criança com meningoencefalocele.

Hippotherapy is one among techniques used in rehabilitation of patients with neurological dysfunctions. The patient body's reactions to horse movements are known to have positive effects on patients' postural control, thus improving general mobility and motor functional performance. The purpose of this paper was to assess the effects of hippotherapy on balance, motor coordination, and functionality of a child with meningoencephalocele. A 3-and-a-half year-old girl with meningoencephalocele was submitted to 18 hippotherapy sessions (in a three- phase procedure - feeding, brushing, and riding) once a week. Outcome measures were obtained by means of the Berg and Tinetti balance scales and the Brazilian version of the Pediatric Evaluation of Disability Inventory (for general functional abilities), all applied before and after therapy, and at an 8-week follow-up assessment. Results showed the child's significantly improved balance and motor coordination, which reflected on functional movement control, basic for performing daily living activities. Functional gain was also perceived by the child's mother. This study provides a new insight into hippotherapy as a useful physical therapy modality for improving motor and functional ability of children with encephalocele.

Congenital fusion of the thalami (atresia of the third ventricle) associated with parietooccipital meningocele.

In this article we present a case of a male newborn with a CNS malformation that is characterized mainly by complete fusion of the thalami resulting in atresia of the 3rd ventricle accompanied by fusion of the anterior peduncles of the fornix, the presence of a single occult interventricular foramen lying at the midline, absence of the septum pellucidum, hypoplasia of the corpus callosum, disorganization of the head of the left caudate nucleus, and greatly dilated lateral ventricles (hydrocephalus). The patient underwent surgical correction of the meningocele on his 4th postnatal day. On his 13th postnatal day he had projectile vomiting due to a left parietooccipital hygroma that was drained via a shunt. On his 31st postnatal day he developed seizures and marked dilatation of the lateral ventricles, for which he underwent a ventriculoperitoneal shunt (Brown). On the 14th postoperative day the patient developed aspiration pneumonia and died.

Gastric bypass: a risk factor for neural tube defects? Case report.

Gastric bypass surgery has become a safe and acceptable surgical weight loss treatment for individuals who suffer from morbid obesity. Patients who undergo this procedure are subject to vitamin deficiencies due to an iatrogenic malabsorptive state. Folate, a vitamin known for its role in the prevention of neural tube defects (NTDs), can be part of the deficiency spectrum resulting from this procedure. The authors describe the case of a woman who was nonadherent to multivitamin treatment after undergoing gastric bypass surgery. Her lack of understanding and appreciation of the relationship between gastric bypass surgery, folate deficiency, and NTDs may have contributed to her noncompliance with daily multivitamin consumption. As a result, her potential problems with folate absorption could have contributed to her subsequently giving birth to a child with a myelomeningocele. Thus, patient awareness and counseling along with aggressive vitamin supplementation among this particular population may help prevent the occurrence of NTDs after gastric bypass surgery.

Perioperative management for surgical correction of frontoethmoidal encephalomeningocele in children: a review of 102 cases.

BACKGROUND: Frontoethmoidal encephalomeningocele (FEEM) is a congenital neural tube defect characterized by herniation of brain and meninges through an anterior skull defect. The extruding mass results in a cutaneous expanding lesion and facial deformity. The objective of this study was to review perioperative management for surgical correction of this condition. METHODS: We reviewed the charts of FEEM children who were treated by surgical correction in King Chulalongkorn Memorial Hospital during 1995-2005. The collected data were categorized into preoperative, intraoperative and postoperative data. In addition, average blood loss was calculated. RESULTS: A total of 102 charts were reviewed. Preoperative data: The mean age of the patients was 4.62 years. Forty-six patients (45%) were smaller than average weight or height. Preoperative-associated abnormal conditions were found in 69 cases (68%). Intraoperative data: The mean duration of the anesthesia and surgery were 6.35 h and 5.51 h, respectively. The estimated mean red cell mass loss was 13.04 ml.kg(-1). There is one case of difficult intubation. No major anesthetic complication was found. Postoperative data: The average intensive care unit stay and hospital stay were 1.55 days and 11.51 days, respectively. The main postoperative complications were tonic-clonic seizure and serious infection including septic shock from wound infection and meningitis. CONCLUSIONS: We reported perioperative management in 102 FEEM children. Comprehensive care during preoperative, intraoperative and postoperative period is essential for successful outcome.

Endocrine abnormalities in patients with frontoethmoidal encephalomeningocele. A preliminary study.

BACKGROUND: Frontoethmoidal encephalomeningocele (FEEM) is a congenital neural tube defect which is more common in Southeast Asia. Its etiology is unknown. No one has ever reported endocrine abnormalities in this condition. METHOD: We retrospectively reviewed medical records of 84 patients with FEEM, primarily focused on the clinical data suggestive of endocrine disorders including growth parameter, thyroid function test, serum electrolyte and serum osmolality. RESULTS: Nasoethmoidal was the most common subtype found in this study. Sixty-four percent of our patients had their heights below the mean height of normal Thai children. The incidence of hypothyroidism (1:28), central DI (1:42) and GHD (1:42) were higher than that in the general population. CONCLUSION: A significant number of patients with FEEM had clinical evidences suggestive of hypothalamopituitary insufficiency. Appropriate replacement therapy is mandatory in order to prevent untoward complications.

Association between lower hair zinc levels and neural tube defects.

Though folic acid supplementation has reduced the incidence of Neural Tube Defects (NTD), NTD still constitutes one of the important congenital malformations having wide medical, social and ethical implications. Zinc deficiency has been reported to produce NTD in animals. This study was designed to evaluate zinc status of the newborn babies with NTD and their mothers. Eighty newborn babies with NTD and their mothers served as cases. Eighty apparently normal newborn babies and their mothers served as controls. Serum and scalp hair zinc levels were analyzed by atomic absorption spectrophotometry. The mean (+/- SD) serum and hair levels in normal mothers were 74.1 +/- 4.1 micrograms/dl and 142.3 +/- 8.0 micrograms/g respectively. The mean (+/- SD) serum and hair levels of the mothers who delivered NTD babies were 75.7 +/- 5.6 micrograms/dl and 129.9 +/- 5.3 micrograms/g respectively. The mean (+/- SD) serum and hair levels in normal newborn babies were 77.8 +/- 5.3 micrograms/dl and 188.8 +/- 6.2 micrograms/g respectively. The mean (+/- SD) serum and hair levels in NTD babies were 80.1 +/- 12.9 micrograms/dl and 174.2 +/- 10.7 micrograms/g respectively. The hair zinc levels of the affected babies and their mothers were significantly lower (P < 0.001) than the controls. This study has found association between NTD and decreased hair zinc levels and large population based studies are recommended to confirm the association between zinc and NTD and to investigate whether zinc supplementation would reduce the overall incidence of NTD.

The spectrum of imaging in Currarino triad.

The aim of this study was to evaluate the spectrum of findings and the efficacy of different imaging modalities in order to formulate recommendations for diagnostic imaging of Currarino triad (ASP syndrome), including screening of relatives. The imaging films of five female patients (age range 6 weeks to 12 months) were analysed retrospectively. The studied material consisted of US and MRI of the lower spine (5 patients each), lumbosacral plain radiography (4 patients), contrast enema (4 patients), urinary US (2 patients), genitography (1 patient) and myelo-CT (1 patient). Depiction of pathological findings with different imaging modalities was reviewed and validated with special respect to their demonstrability by US. Ultrasonography detected the sacral bony defect as well as the presacral pathology (meningocele and/or tumour) and thereby gave the basic diagnosis in all of the cases. It also depicted tethered cord and urinary tract abnormalities correctly. Magnetic resonance imaging gave a more distinct visualization of pre- and intraspinal pathology with additional demonstration of intraspinal lipoma in two cases. Regarding anorectal and genital malformations, radiographic contrast agent studies had been used in all patients. Two blind-ending retrorectal fistulas, depicted by enema, were missed by MRI. Patients with congenital or early infancy obstipation, anorectal malformations and complex urinary tract malformations should have spinal and pelvic sonography first. A plain film of the sacrum is recommended in equivocal cases. The need for MRI and contrast agent studies depends on the individual pathology, whereas presently MRI has made further radiographic imaging increasingly dispensable. A screening program with lumbosacral US or plain radiography for families with Currarino triad should be obligatory.