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Objective: Hydranencephaly is a congenital central nervous system disorder characterized by the complete or near-complete absence of the cerebral cortex and basal ganglia. Because of its rarity, data on the clinical features and survivorship remain sparse. We aim to determine the clinical features and survivorship of a cohort of patients with hydranencephaly., Methods: We performed a retrospective cohort study of all patients diagnosed with hydranencephaly at our institution from 2008 to 2018. Data on demographics, clinical features, presence of comorbidities, surgical operations performed, and status on last follow-up were collected. Survival curves were generated using Kaplan-Meier analysis., Results: Fifty patients were included in the cohort, who had a median age at diagnosis of 4 months and a female predilection. The most common clinical manifestations were macrocephaly (92%) and seizures or myoclonic movements. Infection was present in 36% of cases, endocrinopathies in 22%, dysmorphisms in 20%, and cardiac disease in 8%. Twenty patients underwent shunt insertion, with half developing a postoperative complication at a mean follow-up of 14.9 months. The median survival of the cohort was not reached at 7.5 years. Among the patients with follow-up, characteristics were similar between the surgical and nonsurgical groups, except for the greater incidence of infections in the nonsurgical group. The survival curves among the groups were significantly different, with a hazard ratio of 3.731 in the nonsurgical group., Conclusions: In this large single-center retrospective cohort of patients with hydranencephaly, novel findings are presented regarding the clinical manifestations and survivorship of this condition., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Hydranencephaly is a rare and debilitating congenital condition in which most anesthesiologists are unfamiliar. Primary surgical treatment involves CSF diversion, though other palliative procedures requiring anesthesia are often required. With medical advancements and a resulting prolonged life expectancy, caring for these patients is becoming more routine., Case Presentation: We follow an infant with hydranencephaly over three different procedures requiring anesthesia from 5 months of age to 2 years, highlighting the various anesthetic considerations., Conclusions: Anticipation of difficult positioning, deliberate airway management, and attention to anesthetic recovery were all necessary to safely care for this patient. An understanding of the challenges this particular condition poses will help anesthesiologists provide the most safe and effective care when encountering these patients.

Purpose: Hydranencephaly is a rare condition that occurs during embryogenesis after neurogenesis and is characterized by the near complete absence of the cerebral hemispheres. In general, patients with hydranencephaly have been considered to have a markedly reduced life expectancy. We present 4 patients with hydranencephaly who have survived for over 5 years. The management and problems encountered in these cases are discussed., Methods: A retrospective review was conducted at our institution. Medical charts and radiographic studies were reviewed. Data including age at follow-up, sex, clinical complications, and surgical procedures were recorded., Results: Six patients were radiologically diagnosed with hydranencephaly during the period from January 2000 to December 2012. Two patients were excluded from our study: one because of death from pneumonia at 1 year of age and another because of transfer to another hospital. Four patients (3 males and 1 female) were included in the analysis. All 4 patients underwent ventriculoperitoneal shunt (VPS) placement and shunt revision. VPS infection occurred in 3 of 4 cases, and bloody cerebrospinal fluid (CSF) was observed in 2 of 4 cases. One patient underwent successful choroid plexus cauterization (CPC) and shunt removal after shunt infection., Conclusions: Prolonged survival with hydranencephaly is not unusual in the modern treatment era. CSF shunt problems, such as recurring shunt malfunction and shunt infection, represent one of the major problems, and avoiding CSF shunt with CPC is particularly desirable in patients with hydranencephaly.

Purpose: Hydranencephaly is a congenital condition characterized by the complete or near-complete absence of the cerebral cortex and basal ganglia, while central diabetes insipidus (CDI) is a condition characterized by the inability to concentrate urine due to a deficiency in antidiuretic hormone (ADH). CDI is known to occur in midline congenital malformations such as holoprosencephaly and septo-optic dysplasia, but its association with hydranencephaly is less well-established., Methods: We reported two cases of hydranencephaly complicated by CDI. We also performed a systematic review of the SCOPUS and PubMed databases for case reports and case series of patients with hydranencephaly and CDI, and compiled data on the clinical features and treatment options., Results: Seven cases of hydranencephaly complicated by CDI were identified from the systematic review in addition to the two cases reported here, resulting in a total of nine cases. The patients' age ranged from 4 days to 4 years, and there was a female sex predilection (3.5:1). Patients most commonly presented with macrocephaly, developmental delay, and seizures, with dysmorphic features noted in 33%. In addition to CDI, other endocrinologic derangements included hypothyroidism (22%), hypocortisolemia (22%), and panhypopituitarism (22%). CDI was treated using sublingual or oral desmopressin while hypopituitarism was treated with the appropriate hormone replacement therapy. Insertion of a ventriculoperitoneal (VP) shunt was reported in 44% of cases., Conclusion: The case reports and systematic review suggest a previously unknown association between hydranencephaly and CDI. Clinicians managing cases of hydranencephaly are advised to have a high index of suspicion for CDI in patients presenting with the characteristic signs and symptoms.

Bobble-head doll syndrome is a rare movement disorder that is usually associated with lesions involving the third ventricle. It is characterised by stereotypical rhythmic up-and-down or side-to-side head movements. The pathophysiology and anatomical basis for this unusual manifestation is still a subject of intense scrutiny. The syndrome has never been described in a patient with both hydranencephaly and Chiari type 3 malformation. We describe a 2-year-old female patient who presented with congenital hydrocephalus, an occipital encephalocele and rhythmic bobbling of the head. Imaging investigation revealed a Chiari type 3 malformation and hydranencephaly. The patient was taken to theatre for a ventriculoperitoneal shunt insertion, and at day 3 post operatively, the patient had a markedly decreased head circumference and a decrease in the frequency of the bobbling of the head. A further review at 2 weeks showed that the bobbling of the head had ceased. Although the pathophysiology of bobble-head doll syndrome is yet to be fully understood, there has been postulation of either a third ventricular enlargement or a cerebellar dysfunction to explain bobble-head doll syndrome. Our case illustrates that the pathophysiology is most likely multifactorial as illustrated by the fact that by just addressing the high intracranial pressure with a shunt was sufficient to treat the condition.

Introduction: The standard treatment for hydranencephaly and maximal hydrocephalus consists of inserting shunts, although complications frequently occur. Choroid plexus cauterization (CPC) is an alternative, but its long-term efficacy and the factors associated with the success and failure of controlling head circumference (HC) are not well defined., Objective: This study aims to evaluate the long-term efficacy and factors related to the success rate of CPC in the treatment of hydranencephaly and maximal hydrocephalus., Method: Forty-two children with maximal hydrocephalus and hydranencephaly underwent CPC from 2006 to 2014 and were retrospectively evaluated. Children with less than 3 months of follow-up were excluded. The long-term efficacy and success rate of possible variables (i.e., sex, type of malformation, type of surgery performed, treatment hospital, age, and HC at the time of surgery and birth) were evaluated., Results: Thirty-four children were considered for the effectiveness analysis. Treatment was successful in 24 children (70.6%), and failure occurred in 10 children (29.4%). Failure was detected soon after the endoscopic procedure (average 116 days). There was no difference in effectiveness when comparing the age at the moment of surgery (p = 0.473), type of malformation (p = 1), HC at birth (0.699), and HC at the time of surgery (p = 0.648). The surgical death rate was 7.14%., Conclusion: Endoscopic CPC was a valid procedure used to treat hydranencephaly and maximal hydrocephaly, and it was effective in 70.6% of cases, with an average follow-up period of 32 months. When failures occurred, they occurred early. None of the analyzed variables interfered with the success of the treatment.

We present the first case report describing a craniotomy for a glioblastoma in a patient with hemihydranencephaly (HHE). Ten years ago our patient had average cognitive and language functions, indicating very good adaption of his single right hemisphere. Due to the tumour he developed a deceleration, deficits in language functions and mild impairments of basic cognitive functions. Further neuroplastic reorganisations of his right hemisphere in response to the tumour growth are discussed. The favourable postoperative outcome supported the decision for careful tumour resection in this patient with HHE.

Arachnoid collapse is a previously unreported complication of endoscopic choroid plexus coagulation (ECPC) treatment of hydranencephaly and is demonstrated in this case report. The variable anatomy found in hydranencephaly supports the use of ECPC as the preferred treatment option. However, the same anatomical anomalies predispose the procedure to this unique complication. A brief literature review of hydranencephaly has also been performed and is reported through the discussion. Neurosurgeons must be aware of this very dangerous complication and a discussion of preventative measures are made. The use of ECPC and the clinical expectations of treating patients with hydranencephaly is also discussed.

Unlabelled: 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., Methods: 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., Results: 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., Conclusions: 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.

Background: Staphylococcus lugdunensis is a coagulase-negative staphylococcus with aggressive and rapidly progressive infectious behavior. This organism has emerged as an important pathogen implicated in both community-acquired and nosocomial infections, including meningitis, brain abscess, catheter-related bacteremia, and ventriculoperitoneal shunt infection., Case Description: We report the first known case of Staphylococcus lugdunensis intracranial abscess in a pediatric hydranencephalic patient, caused by a ventriculoperitoneal shunt-related infection. Further magnetic resonance imaging (MRI) confirmed a large abscess within the cranium that demonstrated multiple loculations. The patient received externalization of the right occipital ventricular catheter with evacuation of the brain abscess. Medical management included one week of intrathecal antibiotic treatment, and she was discharged on long-term intravenous rifampin and vancomycin, leading to cure of the infection., Conclusion: This case suggests that if Staphylococcus lugdunensis is identified, a virulent and prolonged clinical course with the production of destructive lesions, similar to those with S. aureus, should be expected. A course of antibiotic therapy and aggressive management that may include surgical treatment will be needed., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Hemi-hydranencephaly is a very rare condition characterized by complete or almost near-complete unilateral absence of the cortical cortex, which is filled by a sac of cerebrospinal fluid. Prenatal vascular disruption with occlusion of the carotid artery territories ipsilateral to the damaged brain is the presumed pathogenesis.We have selected nine cases that fit the clinical and pathologic characteristics of hemi-hydranencephaly, demonstrating that destruction of one hemisphere may be not always associated with severe neurologic impairment and may allow an almost normal life. This disorder is an example of a possible prenatal re-organization in which the right and left cerebral hemispheres present functional potentiality to make up the damaged brain.The cases reported in the literature are discussed, including a patient previously reported and followed-up for 10 years. A review of the cases is performed with an evaluation of the most important aspect of this rare and mysterious disorder.

Background/aims: This study evaluates endoscopic choroid plexus coagulation, in conjunction with third ventriculostomy when technically feasible, as a strategy to treat patients with hydranencephaly or hydrocephalus with a minimal cortical mantle., Methods: We retrospectively reviewed patients with hydranencephaly (n = 4) or hydrocephalus with the cortical mantle <1 cm in maximal thickness (n = 4) who underwent endoscopic choroid plexus coagulation from 2007 to 2010. Endoscopic third ventriculostomy was performed simultaneously when technically feasible (in 3 of 8 patients). Endoscopic management was considered successful if a shunt was not subsequently required., Results: Endoscopic management was successful in 4 of 8 patients (50%) who did not require a shunt over a median follow-up period of 15 months (range 2.5-24). Four patients (50%) failed endoscopic management and required a shunt 1 month to 11 weeks after endoscopic surgery. One patient died 4 days after shunt placement due to pneumonia and renal failure. Three of 4 patients who had a successful endoscopic procedure ultimately died of causes that were likely unrelated to hydrocephalus (pneumonia in 2 patients and sudden infant death syndrome in 1 patient). Besides failure to control hydrocephalus adequately in 4 patients, there were no additional complications noted after endoscopic surgeries., Conclusion: Endoscopic choroid plexus coagulation can enable some infants with hydranencephaly or massive hydrocephalus to avoid a ventriculoperitoneal shunt., (Copyright © 2012 S. Karger AG, Basel.)

Hydranencephaly is a rare condition characterised by complete or near complete absence of the cerebral hemispheres within relatively normal sized meninges and skull, the resulting cavity being filled with cerebrospinal fluid. The following is a case report of a five month old hydranencephalic child with right upper motor facial nerve palsy who presented with signs of hydrocephalus who developed intractable hypothermia rapidly post ventriculo-peritoneal shunt insertion and demised. Her preoperative condition was associated with hypothermia.

Objective: To prospectively evaluate the results of endoscopic choroid plexus cauterization (ECPC) and ventriculoperitoneal shunts (VPSs) in infants with hydranencephaly or near hydranencephaly., Methods: We prospectively collected clinical data from all untreated hydranencephalic and near hydranencephalic children from October 2006 to March 2008. All patients treated were randomly divided into 2 groups, ECPC or VPS, and submitted to either endoscopic choroid plexus cauterization or ventriculoperitoneal shunt placement., Results: Seventeen patients were entered into the study. ECPC was completed in 9 patients; the procedure successfully controlled excessive head circumference and signs of increased intracranial pressure in 8 of these patients (88.8%). One endoscopic procedure in a hydranencephalic child failed after 7 months, resulting in VPS placement. Thus, of the 10 patients randomized to ECPC, 8 were treated successfully by ECPC (80%), and 2 went on to have a VPS. There were no complications related to this method of treatment. Seven children were randomized to the VPS group; and of these, 2 patients (28.5%) required shunt revisions during follow-up. There were no complications related to shunt placement. There was no difference in the success rate between patients randomized to ECPC and VPS, but the ECPC was more economical., Conclusion: ECPC is an acceptable alternative to VPS for treatment of hydranencephaly and near hydranencephaly. It is a single, definitive, safe, effective, and economical treatment that may avoid the complications of shunting.

Introduction: The removal of the choroid plexus from the lateral ventricles was attempted by Dandy in the early 20th century but later discarded as complications arose and other methods of cerebrospinal fluid (CSF) diversion were introduced. We compare our experience with a variation of this operation to CSF diversion in patients with hydranencephaly or near hydranencephaly., Methods: The hospital and office charts of patients with a diagnosis of hydranencephaly were reviewed from the two institutions spanning the career of the senior author. Thirteen patients were identified, of whom 9 underwent CSF diversionary procedures (group A) and 4 underwent choroid plexectomy (group B)., Results: The mean number of reoperations (2 in group A, 0 in group B), neurosurgical readmissions (1.5 in group A, 0 in group B) and days of hospitalization related to neurosurgical readmissions (43.5 in group A, 0 in group B) were all less in patients who underwent choroid plexectomy. The total incidence of complications related to surgery was also lower in this group (7 in group A, 0 in group B)., Conclusion: In our experience, choroid plexectomy in patients with hydranencephaly reduces the incidence of reoperation and readmission, the number of days of hospitalization related to the surgical procedure and the total number of complications in comparison to patients undergoing CSF diversion. Further neurosurgical intervention is minimized as is the financial burden from multiple emergency department visits and radiological procedures for shunt evaluation. Choroid plexectomy is a viable alternative to CSF diversion in patients with hydranencephaly and a rapidly enlarging head. It avoids the chronic issues and complications surrounding CSF diversion in this difficult group of patients., (Copyright 2002 S. Karger AG, Basel)

To define better the syndromes of hydranencephaly and maximal hydrocephalus, we followed 10 neonates demonstrating (by computerized tomography) virtual absence of the cerebral substance with serial computerized tomographic (CT) scans, electroencephalograms (EEGs), and developmental evaluations. The follow-up period ranged from 4 to 23 months. Two well-defined syndromes emerged. Five patients showed absence of cortical activity on EEG and a CT picture of minimal occipital brain parenchyma connected by a thin bridge of tissue to intact basal ganglia. These patients were shunted and showed no improvement with time, either neurologically or radiologically. Five patients showed minimal frontal cerebral mantle on CT and the presence of electrical activity on EEG. These patients required bilateral shunts to control head size and showed remarkable progress with time. Serial CT scans demonstrated the appearance of considerable brain substance, and neurological development was either normal or slightly delayed. We believe that the former pattern of only minimal occipital brain and absent EEG activity defines true hydranencephaly, whereas the presence of small amounts of frontal brain tissue associated with EEG activity seems to represent maximal hydrocephalus. The differences in CT pattern, EEG, and clinical progress all suggest a different pathophysiology for these two conditions. (Neurosurgery, 6: 35--38, 1980).