Severe Hydrocephalus Research Articles

Camk2a-Cre-mediated conditional deletion of chromatin remodeler Brg1 causes perinatal hydrocephalus

Mammalian SWI/SNF-like BAF chromatin remodeling complexes are essential for many aspects of neural development. Mutations in the genes encoding the core subunit Brg1/SmarcA4 or other complex components cause neurodevelopmental diseases and are associated with autism. Congenital hydrocephalus is a serious brain disorder often experienced by these patients. We report a role of Brg1 in the pathogenesis of hydrocephalus disorder. We discovered an unexpected early activity of mouse Camk2a-Cre transgene, which mediates Brg1 deletion in a subset of forebrain neurons beginning in the late embryonic stage. Brg1 deletion in these neurons led to severe congenital hydrocephalus with enlargement of the lateral ventricles and attenuation of the cerebral cortex. The Brg1-deficient mice had significantly smaller subcommissural organs and narrower Sylvian aqueducts than mice that express normal levels of Brg1. Effects were non-cell autonomous and may be responsible for the development of the congenital hydrocephalus phenotype. Our study provides evidence indicating that abnormalities in Brg1 function result in defects associated with neurodevelopmental disorders and autism.

Cyclin O (Ccno) functions during deuterosome-mediated centriole amplification of multiciliated cells.

Mucociliary clearance and fluid transport along epithelial surfaces are carried out by multiciliated cells (MCCs). Recently, human mutations in Cyclin O (CCNO) were linked to severe airway disease. Here, we show that Ccno expression is restricted to MCCs and the genetic deletion of Ccno in mouse leads to reduced numbers of multiple motile cilia and characteristic phenotypes of MCC dysfunction including severe hydrocephalus and mucociliary clearance deficits. Reduced cilia numbers are caused by compromised generation of centrioles at deuterosomes, which serve as major amplification platform for centrioles in MCCs. Ccno-deficient MCCs fail to sufficiently generate deuterosomes, and only reduced numbers of fully functional centrioles that undergo maturation to ciliary basal bodies are formed. Collectively, this study implicates CCNO as first known regulator of deuterosome formation and function for the amplification of centrioles in MCCs.

Infratentorial complications following preresection CSF diversion in children with posterior fossa tumors.

This report presents the incidence, causes, and morbidity and mortality of infratentorial complications following CSF diversion before resection in children with posterior fossa tumors. The medical records of 437 children admitted to Abo El-Reesh Pediatric University Hospital with a diagnosis of posterior fossa tumor between 2005 and 2012 were retrospectively reviewed. Seven children developed neurological deterioration following CSF diversion due to infratentorial complications. Computed tomography scans revealed intratumoral hemorrhage (ITH) in 5 cases, while upward transtentorial herniation (UTH), as evidenced by obliteration of the quadrigeminal and ambient cisterns, was diagnosed in 2 cases. Hydrocephalus was noted in 381 patients, and 301 patients underwent CSF diversion before resection. A ventriculoperitoneal (VP) shunt was used in 214 patients, and 6 children (2.8% of shunted cases) deteriorated neurologically (4 due to ITH and 2 due to UTH). Endoscopic third ventriculostomy (ETV) was performed in 87 patients, 1 of whom developed ITH (1.1% of the patients undergoing ETV). Six patients deteriorated within 8 hours (85.7%), whereas 1 patient, the only survivor, deteriorated after 24 hours. The incidence of infratentorial complications between VP shunts and ETVs was not found to be significantly different (p = 0.659). There was a higher risk of such complications in large posterior fossa tumors (diameter ≥ 4 cm) extending close to the tentorial incisura, especially in patients with severe hydrocephalus and significant peritumoral edema. Infratentorial complications (ITH and UTH) in children with posterior fossa tumors are not uncommon (2.3%) after preresection CSF diversion (VP shunt or ETV) and are associated with a very poor prognosis in most cases, even with surgical intervention.

History of the Family Practice Section National Medical Association

This work was presented at the 57th Quarterly Grand Rounds at Fairview Developmental Center, Costa Mesa, California on October 17, 2012.Authors are grateful to Stephan Reynolds CRT, and Indira Makwana for records.None of the authors has any conflict of interest with the information provided in this article.We report the case of a 45-year-old patient with large congenital hydrocephalus. Patient was born prematurely at 26-weeks’ gestation with a weight of 1.14 Kg. His head-circumferences have been: Birth, 27.9 cms; age 3-months, 40.6 cms; 18-months, 74 cms; 36-months, 80 cms; current, 88.4 cms (normal adult: 52–58 cms). During infancy, his prognosis was considered “terminal” due to rapid progression of severe hydrocephalus; therefore no neurosurgical intervention was undertaken. At 18-months’ age, he was admitted to our developmental center where he is presently comfortable and clinically stable. Due to habitual unilateral decubitus, his head is now markedly flattened on the right (deformational plagiocephaly), and disproportionately long and narrow (scaphocephaly: occipitofrontal length 36.8 cms, biparietal width 12.5; ratio 2.9). We credit patient’s extended survival to diligent professional care.PubMed

Immunohistochemical Study of Aquaporins in an African Grey Parrot (Psittacus erithacus) With Hydrocephalus.

A 5-month-old African grey parrot (Psittacus erithacus) was examined after 3 weeks of weakness, ataxia, mental depression, and seizures. Results of a complete blood cell count and plasma biochemical analysis were unremarkable. Magnetic resonance imaging revealed a severe bilateral hydrocephalus. The bird failed to improve with supportive care, and the owner requested euthanasia. Necropsy findings were severe bilateral hydrocephalus with no evidence of cerebrospinal fluid obstruction. Histologic examination of the brain revealed microspongiosis, edema, gliosis, and neuronal chromatolysis of surrounding periventricular tissue. Aquaporins (AQP) and astrocytes were examined to elucidate the participation of these water channel proteins and glial cells in the pathophysiology and resolution of hydrocephalus. Results showed AQP4 and glial fibrillary acidic protein were overexpressed, especially near the ventricles, but expression of AQP1 was decreased. This is the first report, to our knowledge, of AQP immunolabeling in hydrocephalus in avain species.

ED-01 * MEDULLOBLASTOMA PRESENTING AS AN INTRACRANIAL HEMORRHAGE

CASE REPORT: Children with medulloblastoma usually present with ataxia, nystagmus, dysmetria, intention tremor, cranial nerve deficits or signs of elevated intra-cranial pressure. On MRI a midline non-homogenously enhancing lesion is seen often with associated brainstem compression, ventricular dilatation and transependymal CSF migration. We describe a 12 year old child who presented with dizziness, emesis, neck pain and occipital headache resulting from minor head trauma on the preceding day. Past history included recent onset intermittent diplopia corrected with lenses. Neurological examination was normal except for mild left hearing loss and decreased visual acuity. CT scan at admission revealed an acute subarachnoid hemorrhage in the left cerebellopontine angle (CPA) extending through the left foramina of Luschka and Magendie and compressing the adjacent cerebellum and brainstem. MRI /MRA/Cerebral angiogram did not uncover any underlying pathology. After observation for 3 days he was discharged with residual mild left hearing loss and left horizontal nystagmus. On follow up four months later he was found to have lost all hearing (left) and had difficulty with balance. MRI showed a 2.8 x 2.6 x 2.4 cm left CPA mass hypo-intense on T1 with hyperintense foci on T2. The mass extended into the internal auditory canal and compressed the brainstem without causing hydrocephalus. A peri-lesional hemorrhagic stain was observed. On repeat review of an MRI performed 2 months earlier the mass was noted to be present measuring 1.5 x 1.1 x 1.2 cm. Spinal cord MRI was negative. Gross total resection of the mass was complicated by left facial nerve palsy and severe obstructive hydrocephalus requiring temporary EVD placement. The patient is on treatment for medulloblastoma (classic histology/WNT subgroup).Hearing loss persists but his 7th CN palsy is improving. We seek to highlight that on MRI an acute intracranial hemorrhage can camouflage a neoplastic process.

Hydranencephaly: cerebral spinal fluid instead of cerebral mantles

The authors report a wide and updated revision of hydranencephaly, including a literature review, and present the case of a patient affected by this condition, still alive at 36 months.Hydranencephaly is an isolated and with a severe prognosis abnormality, affecting the cerebral mantle. In this condition, the cerebral hemispheres are completely or almost completely absent and are replaced by a membranous sac filled with cerebrospinal fluid. Midbrain is usually not involved. Hydranencephaly is a relatively rare cerebral disorder. Differential diagnosis is mainly relevant when considering severe hydrocephalus, poroencephalic cyst and alobar holoprosencephaly. Ethical questions related to the correct criteria for the surgical treatment are also discussed.

Pilot study of radiation dose reduction for pediatric head CT in evaluation of ventricular size.

CT is a ubiquitous, efficient, and cost-effective method to evaluate pediatric ventricular size, particularly in patients with CSF shunt diversion who often need emergent imaging. We therefore sought to determine the minimum dose output or CT dose index required to produce clinically acceptable examinations. Using a validated noise insertion method and CT projection data from 22 patients, standard pediatric head CT images were reconstructed with weighted filtered back-projection and sinogram-affirmed iterative reconstruction corresponding to routine, 25%, and 10% dose. Reconstructed images were then evaluated by 3 neuroradiologists (blinded to dose and reconstruction method) for ventricular size, diagnostic confidence, image quality, evidence of hemorrhage, and shunt tip location, and compared with the reference standard. There was no significant difference in the ventricular size ranking, and the sensitivity for moderate to severe hydrocephalus was 100%. There was no significant difference between the full-dose level and the ventricular size rankings at the 25% or the 10% dose level for either reconstruction kernel (P > .979). Diagnostic confidence was maintained across doses and kernel. Hemorrhage was more difficult to identify as image quality degraded as dose decreased but was still seen in a majority of cases. Shunts were identified by all readers across all doses and reconstruction methods. CT images having dose reductions of 90% relative to routine head CT examinations provide acceptable image quality to address the specific clinical task of evaluating ventricular size.

Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2

Hydrocephalus is caused by the accumulation of cerebrospinal fluid (CSF) in the cerebral ventricular system which results in an enlargement of the cranium due to increased intraventricular pressure. The increase in pressure within the brain typically results in sloughing of ciliated ependymal cells, loss of cortical gray matter, and increased gliosis. Congenital hydrocephalus is associated with several syndromes including primary ciliary dyskinesia (PCD), a rare, genetically heterogeneous, pediatric syndrome that results from defects in motile cilia and flagella. We have examined the morphological and physiological defects in the brains of two mouse models of PCD, nm1054 and bgh, which have mutations in Pcdp1 (also known as Cfap221) and Spef2, respectively. Histopathological and immunohistochemical analyses of mice with these mutations on the C57BL/6J and 129S6/SvEvTac genetic backgrounds demonstrate strain-dependent morphological brain damage. Alterations in astrocytosis, microglial activation, myelination, and the neuronal population were identified and are generally more severe on the C57BL/6J background. Analysis of ependymal ciliary clearance ex vivo and CSF flow in vivo demonstrate a physiological defect in nm1054 and bgh mice on both genetic backgrounds, indicating that abnormal cilia-driven flow is not the sole determinant of the severity of hydrocephalus in these models. These results suggest that genetic modifiers play an important role in susceptibility to severe PCD-associated hydrocephalus.

Choroid plexus coagulation in infants with extreme hydrocephalus or hydranencephaly.

OBJECT.: Severe hydrocephalus and hydranencephaly are common congenital conditions in Kenya. In patients with these conditions, ventriculoperitoneal (VP) shunts are associated with appreciable complications and endoscopic third ventriculostomies (ETVs) have limited success. Endoscopic choroid plexus coagulation (CPC) to diminish CSF production is a potential treatment option. The purpose of this study was to evaluate the effect of CPC without ETV in infants with severe hydrocephalus or hydranencephaly. Medical records of infants with severe congenital hydrocephalus or hydranencephaly who underwent CPC in Kijabe Hospital from November 2010 to April 2013 were reviewed retrospectively. Thirty-three patients with complete medical records and preoperative radiographic images were identified. After CPC, the infants were followed in the Kijabe Hospital outpatient department, in mobile clinics, or by telephone. Success of the CPC was defined as resolution of preoperative symptoms, stabilization of head size, and avoidance of VP shunt placement. Patients were followed from 30 to 608 days (median of 120 days). Three patients were lost to follow-up. Of the 30 evaluable patients, CPC was considered to be successful in 13 (43.3%), including 8 of 20 patients with severe hydrocephalus and 5 of 10 with hydranencephaly. Failure of CPC was evident from increased head circumference in 14 (82%) of 17 patients and from CSF leakage in 3. Of the 17 failures, 13 occurred within 3 months of surgery. Six patients died: 3 whose CPC procedures were failures, 2 whose CPC was successful, and 1 postoperatively. Of the 17 in whom CPC failed, 10 subsequently underwent VP shunt insertion. CPC stabilizes macrocephaly in approximately 40% of infants with severe congenital hydrocephalus and hydranencephaly and can be considered as an alternative to VP shunt placement.

Motor developmental delay in a healthy 11-month-old infant: answer

MRI showed a large, well-defined non-enhancing thin-walled cyst with a T2 hypointense circumferential rim, arising above the sella turcica. Occlusion of the bilateral foramina of Monro by the thin-walled cyst resulted in severe obstructive hydrocephalus. Radiographic characteristics of the cyst content were consistent on all image sequences with cerebrospinal fluid (CSF), suggesting the diagnosis of a suprasellar arachnoid cyst (SAC). The child was taken to the operating room for cyst fenestration, restoration of CSF ventricular drainage, and relief of hydrocephalus. A right transfrontal endoscopic approach was undertaken (Fig. 1A, as viewed along the arrow trajectory seen in Fig. 2A of Images in Neuroscience: Question), confirming the presence of an occlusive cyst. Fenestration of this wall revealed the cyst interior (Fig. 1B). Chronic thinning of the third ventricle floor allowed striking visualization of the posterior circle of Willis (Fig. 1B, arrowheads), pituitary stalk and gland (black arrow), and bilateral third cranial nerves (white arrows). Arachnoid cysts are CSF-filled structures formed by arachnoid membranes, commonly found arising in the subarachnoid cisterns or major fissures of the brain. These cysts account for 1% of all intracranial space-occupying lesions, and are typically single, left-sided, and have a male to female predominance of 2:3 [1]. SAC represent only 9% of all arachnoid cysts in adults and 15% of all arachnoid cysts in the pediatric population [2]. While often an incidental finding on cranial imaging, surgical intervention can become necessary when compression of adjacent structures results in hydrocephalus, neurological symptoms, endocrine abnormalities, or developmental delay. As seen with this classic presentation of a SAC, approximately 90% of symptomatic SAC present with hydrocephalus due to obstructed third or lateral ventricular CSF outflow [3]. The exact etiology of SAC is unknown, though it may be linked with weakening of an arachnoid wall that later forms an inversion of the membrane, favoring cyst development [4]. Early identification of symptomatic SAC can facilitate prompt surgical interventions to relieve symptomatic compressions [1–3,5]. The morphology and periventricular location of SAC often permit endoscopic fenestration. These procedures are often effective, minimally disruptive to collateral structures, and associated with low morbidity and mortality rates, particularly in children [5]. Here, fenestration of the cyst floor allowed decompression into the adjacent subarachnoid cisterns. Despite initial successful cyst decompression, our patient later developed hydrocephalus requiring ventriculoperitoneal shunting with improvement in psychomotor development. SAC should be considered in patients demonstrating evidence of progressive hydrocephalus and a suprasellar lesion, as surgical intervention may dramatically improve cognitive trajectories and relieve potentially life-threatening hydrocephalus. Further elucidation of the natural history and evolution of SAC, including associated CSF hydrodynamic abnormalities, may help direct the optimum treatment strategy.

Efficacy of cerebrospinal fluid lavage replacement in severe tuberculous hydrocephalus

Efficacy of cerebrospinal fluid lavage replacement in severe tuberculous hydrocephalus

Diagnóstico ecográfico de la ventriculomegalia fetal. Seguimiento posnatal

ObjetivesTo determine the prevalence of fetal ventriculomegaly and identify the neurological outcomes of newborns with a prenatal diagnosis. MethodObservational study of ventriculomegaly prenatally diagnosed by ultrasound in our department between 2004 and 2012. ResultsWe diagnosed 61 cases of ventriculomegaly. The prevalence rate was 2.25 per 1,000. Diagnosis was made during the second trimester in 54 patients and during the third trimester in 7. Bilateral ventriculomegaly was found in 91% of the fetuses. Ventriculomegaly was mild (10-12 mm) in 79.4% and moderate (12-15 mm) in 5.6%. Severe ventriculomegaly (>15 mm) or hydrocephalus was diagnosed in 15% of the fetuses. The clinical course during pregnancy was unfavorable in 13%. Associated anomalies were diagnosed in 23 cases. ConclusionsMild, isolated and non-progressive ventriculomegaly has the best neurological prognosis.

The Lhx9 homeobox gene controls pineal gland development and prevents postnatal hydrocephalus

Lhx9 is a member of the LIM homeobox gene family. It is expressed during mammalian embryogenesis in the brain including the pineal gland. Deletion of Lhx9 results in sterility due to failure of gonadal development. The current study was initiated to investigate Lhx9 biology in the pineal gland. Lhx9 is highly expressed in the developing pineal gland of the rat with transcript abundance peaking early in development; transcript levels decrease postnatally to nearly undetectable levels in the adult, a temporal pattern that is generally similar to that reported for Lhx9 expression in other brain regions. Studies with C57BL/6J Lhx9(-/-) mutant mice revealed marked alterations in brain and pineal development. Specifically, the superficial pineal gland is hypoplastic, being reduced to a small cluster of pinealocytes surrounded by meningeal and vascular tissue. The deep pineal gland and the pineal stalk are also reduced in size. Although the brains of neonatal Lhx9(-/-) mutant mice appear normal, severe hydrocephalus develops in about 70% of the Lhx9(-/-) mice at 5-8 weeks of age; these observations are the first to document that deletion of Lhx9 results in hydrocephalus and as such indicate that Lhx9 contributes to the maintenance of normal brain structure. Whereas hydrocephalus is absent in neonatal Lhx9(-/-)mutant mice, the neonatal pineal gland in these animals is hypoplastic. Accordingly, it appears that Lhx9 is essential for early development of the mammalian pineal gland and that this effect is not secondary to hydrocephalus.

Long-term outcome in children with low grade tectal tumours and obstructive hydrocephalus

BackgroundNeuropsychological deficits after treatment of paediatric brain tumour are well known, but not the role of hydrocephalus in these deficits. AimsTo study long-term neurological, cognitive, and behavioural deficits in children with a low grade tectal tumour and acquired obstructive hydrocephalus. MethodsIn a consecutive series of 12 children with low-grade tectal tumour diagnosed in our hospital between 1994 and 2008, neurologic, neuropsychological, and radiologic data were prospectively collected. Intelligence, memory, attention, language, visual-spatial, and executive functions were assessed. Median follow-up was 2 years and 9 months. ResultsAt follow-up, most frequent neurologic disability was fatigue in children with a low-grade tectal tumour. They scored lower on sustained attention, long-term memory and had more behavioural problems. Factor influencing cognition was persisting severe hydrocephalus at time of assessment. The cognitive problems resulted in 60% of children needing assistances of special services at school. ConclusionsAt long-term, children with a low-grade tectal tumour display invalidating neuropsychological impairments resulting in educational problems. Adequate treatment of hydrocephalus may result in better cognitive functioning. Our findings suggest that part of the symptoms of the cerebellar cognitive affective syndrome may not have resulted from a cerebellar lesion itself but rather from a cerebral dysfunction or compression of supratentorial structures in the cerebello-cortical circuitry due to the obstructive hydrocephalus.

Ultrastructural alterations of human cortical capillary basement membrane in human brain oedema.

The capillary basement membranes are examined in severe traumatic brain injuries, vascular malformation, congenital hydrocephalus and brain tumours. They exhibit homogeneous and nodular thickening, vacuolization, rarefaction, reduplication, and deposition of collagen fibers. Their average thickness varied according to the aetiology and severity of brain oedema. In moderate brain oedema the thickness ranged from 71.97 to 191.90 nm in width, and in patients with severe brain oedema it varied from 206.66 to 404.22 nm. The basement membrane complex appears apparently intact in moderate oedema, and shows glio-basal dissociation in severe oedema. In areas of highly increased cerebro-vascular permeability, the basement membrane shows matrix disorganization, reduplication, and bifurcations protruding toward the endothelial cells, and acting as abluminal transcapillary channels. In regions of total brain necrosis, its structural stability is lost showing loosening, dissolution and rupture. Basement membrane swelling is due to overhydration of its protein-complex glycoprotein matrix. The thickening, rarefaction and vacuolization are induced by the increased vacuolar and vesicular transendothelial transport. The degenerated basement membrane areas exhibit a finely granular precipitate interpreted as protein, proteoglycan, glycoprotein, and agrin degraded matrix.

Transperitoneal 3D laparoendoscopic single-site (LESS) nephrectomy: the first clinical case in China

Objective To summarize our experience on the first clinical case of transperitoneal 3Dlaparoendoscopic single-site(LESS)nephrectomy in mainland China,and to assess its safety and feasibility after obtaining the informed consents. Methods On August 5,2013,we performed a 3DLESS nephrectomy using Karl Storz 3Dendoscope and a multi-channel Quad-Port(OlympusTM)through a 3cm skin incision at our institute.The patient was a 69years old man,with a body mass index(BMI)of 27.7kg/m2 and a preoperative serum creatinine level of 81μmol/L.99mTc DTPA was used to determine the glomerular filtration rate(GFR).The preoperative unilateral renal function was 65mL/min for the left side and 9.5mL/min for the right side.And therefore he was diagnosed as"right kidney severe hydrocephalus"and received right nephrectomy.A 3cm external retus incision was made at the umbilicus level at 1cm site.After dissection of the renal pedicle blood vessels the renal blood vessels,the ureterogonadal packet was left en bloc and transected at the level when crossing the common iliac vessels. The kidney was extracted through the original incision after the skin incision was extended to 4cm.Results The procedure was smoothly completed without any extra skin incision.The operating time was 154min,with an estimated blood loss of 150mL. The gastrointestinal function recovered on the first day after operation.Postoperative serum creatinine level on the first day was 76μmol/L.Donor Visual Analog Pain Scores at postoperative day 1,2and 3were 2/10,1/10and 0/10,respectively.The recovery of the patient was uneventful and he was discharged on the 3rd postoperative day.Conclusion Transperitoneal 3D laparoendoscopic single-site nephrectomy is safe,feasible and effective.It has the clinical benefits of less pain,rapid recovery and small incision.The procedure allows for a strong stereo perception,accurate manipulation,and a decreased demand for hand-eye coordination of hand,with a promising clinical future.But more clinical experience needs to be accumulated through more cases.

Endoscopic Coagulation of Choroid Plexus in Three Children with Severely Advanced Forms of Hydrocephalus

The development of modern neuroendoscopic technology brought back the use of choroid plexus surgery for the treatment of some patients with hydrocephalus. This study explored the use of endoscopic coagulation of the choroid plexus (ECCP) for the treatment of severely advanced forms of hydrocephalus in three children. Three children with markedly dilated ventricles underwent ECCP. The first child had fetal hydranencephaly, the second had holoprosencephaly, and the third had severe hydrocephalus associated with other congenital malformations. All three children had had previous ventriculoperitoneal (VP) shunts, and ECCP was done because of shunt malfunction. All three children underwent computed tomography and magnetic resonance imaging to confirm the presence and to measure the size of the choroid plexus before surgery. ECCP was performed through a right frontal burr hole. A rigid neuroendoscope and monopolar coagulator were used to achieve choroid plexus coagulation. The first child died 21 days after surgery because of generalized infection originating from the lungs. The second child needed a ventriculoatrial shunt 45 days after ECCP, and the third child needed VP shunt 3 months after ECCP. ECCP, although not an alternative to ventricular shunt, is a safe procedure and should be tried in some children with severe advanced, low progressive, and hopeless forms of hydrocephalus. Long-term watchful follow-up is mandatory after surgery. When the risk of complications after VP shunting is relatively high in children with severe forms of hydrocephalus, ECCP seems to be worth trying. Delayed timing of VP shunting after ECCP would be meaningful for children with a high risk of shunt complications.

A Newborn With Hydrops, Hydrocephalus, and Ophthalmologic Abnormalities.

A male infant was born at an estimated gestational age of 34-3/7 weeks to a 24-year-old G3P2A0 mother, with unknown group B Streptococcus carriage status, immunity to rubella, and negative serologies for hepatitis B, syphilis, and human immunodeficiency virus (HIV). Pregnancy was complicated by fetal hydrops, initially detected by ultrasound 3 weeks prior to delivery. Prenatal magnetic resonance imaging (MRI) detected severe polyhydramnios, fetal hydrocephalus, and hydrops with ascites. Evaluation at that time also included a normal karyotype and negative Coombs test. The mother presented to our center in active labor, and Caesarian section was performed. Artificial rupture of membranes at the time of delivery revealed a large amount of clear fluid. The infant initially had a weak cry and was intubated in the delivery room, and he was transferred to the Neonatal Intensive Care Unit. Apgar scores were 6 at 1 minute and 7 at 5 minutes. The mother lives in St. Louis with her 2 children. She denied pets, animal exposure, travel outside of Missouri, and exposure to sick contacts or to raw or exotic foods. She also denied fevers, diarrhea, headaches, or other illnesses during her pregnancy. Birth weight was 3060 grams (>97th percentile for gestational age), length was 46 cm (50th percentile), and head circumference was 30 cm (10th percentile). Temperature was 36.7°C, heart rate 182/min, respiratory rate 49/min, and blood pressure 96/74 mmHg; oxygen saturation was 95% in room air. On examination, the infant was orally intubated and had spontaneous movements and normal tone. The anterior fontanelle was soft with sutures widely spaced. Red reflexes were absent bilaterally, and a detailed ophthalmologic examination subsequently revealed bilateral cataracts and bilateral retinal colobomas involving the macula. The abdomen was soft but distended; no organomegaly was appreciable. He had significant inguinal and scrotal edema. There were no rashes or skin lesions. The remainder of the physical examination was unremarkable. Complete blood count was notable for thrombocytopenia (81000/µL); chemistry panel was normal (glucose was 42 mg/dL), and liver enzymes were normal (alanine

Trigeminal Neuralgia Due to Dandy-Walker Syndrome

Trigeminal neuralgia (TN) is a common pain in the orofacial region. Dandy-Walker syndrome (DWS) is a congenital malformation of the cerebellar and the fourth ventricle foramina atresia. Dandy-Walker syndrome is rarely found in patients with TN. This article presents a 36-year-old man with the symptoms of typical TN. His physical examination was entirely normal. An enhanced magnetic resonance imaging was taken. Magnetic resonance imaging revealed the bilateral lateral ventricle, the fourth and third ventricle significantly enlarged with severe obstructive hydrocephalus, a huge posterior fossa cyst connected with the fourth ventricle, and hypoplastic vermis. The pain was controlled by Tegretol. The reported case suggests that DWS is an unusual cause of TN.