Early intervention and neurodevelopmental outcome of infants with posthemorrhagic hydrocephalus: A case series and literature review.

OBJECTIVE: Intraventricular hemorrhage (IVH) is the most common central nervous system pathology in preterm infants. No consensus has been reached over the best indication for intervention in patients with posthemorrhagic hydrocephalus (PHH). The authors present the neurological outcome of infants with IVH and an early treatment approach, defined as an intervention when ventricular dilation is less than 4-mm over the 97th-percentile of Levene's index. METHODS: We performed a retrospective case-series study of 12 infants who had IVH and an early intervention, their neurological development was evaluated after 18-months of corrected age using the Bayley-III Scales. Measures of central tendency and Pearson's correlation were used for data analysis. RESULTS: In a 15-month period, twelve patients were diagnosed with IVH and underwent an early intervention. At the time of diagnosis, 2 patients had grade II IVH, 7 grade III, and 3 grade IV. Subgaleal shunt was the first intervention. 9 (75 %) ultimately required a ventriculoperitoneal shunt. A total of 9 (75 %) patients had normal cognitive scores, 7 (58.3 %) for the language-composite, and 8 (66.7 %) for the motor-composite. 6 (50 %) patients had normal scores in all composites. The average scores reported normal results (CC:98.33 ± 22.59; LC:98.25 ± 23.93; MC:88.58 ± 21.47). There was a significant correlation between antenatal steroids and the LC-score (p = 0.044). CONCLUSIONS: Half of the patients with PHH and early neurosurgical interventions had an average or above average neurodevelopmental score in all three areas. Current literature and undergoing clinical trials have shown promising results on the implementation of this type of intervention.

Spontaneous intracranial hypotension: key features for a frequently misdiagnosed disorder.

Spontaneous intracranial hypotension (SIH) is a rare neurological condition caused by low cerebrospinal fluid (CSF) volume, most commonly due to a CSF leak. The most common presenting symptom is an orthostatic headache, but some patients may present with atypical neurological manifestations such as cranial nerve palsies, an altered mental status, and movement disorders, which complicate the clinical diagnosis. Therefore, the diagnosis is based on the combination of clinical signs and symptoms, neuroimaging, and/or a low cerebrospinal fluid pressure. In this review, we describe the wide variety of neurological manifestations and complications seen in patients with SIH as well as the most common features described on imaging studies, including both subjective and objective measurements, in order to lead the clinician to a correct diagnosis. The prompt and correct management of patients with SIH will help prevent the development of life-threatening complications, such as subdural hematomas, cerebral venous thrombosis, and coma, and avoid unnecessary invasive procedures.

Intraventricular hemorrhage and posthemorrhagic hydrocephalus in preterm infants: diagnosis, classification, and treatment options.

PURPOSE: Intraventricular hemorrhage is the most important adverse neurologic event for preterm and very low weight birth infants in the neonatal period. This pathology can lead to various delays in motor, language, and cognition development. The aim of this article is to give an overview of the knowledge in diagnosis, classification, and treatment options of this pathology. METHOD: A systematic review has been made. RESULTS: The cranial ultrasound can be used to identify the hemorrhage and grade it according to the modified Papile grading system. There is no standardized protocol of intervention as there are controversial results on which of the temporizing neurosurgical procedures is best and about the appropriate parameters to consider a conversion to ventriculoperitoneal shunt. However, it has been established that the most important prognosis factor is the involvement and damage of the white matter. CONCLUSION: More evidence is required to create a standardized protocol that can ensure the best possible outcome for these patients.

Impact of skull base development on endonasal endoscopic surgical corridors.

OBJECT: Scarce morphometric data exist on the developing skull base as a corridor for endonasal endoscopic approaches (EEAs). Furthermore, the impact of skull base lesions on its development has not been assessed. The authors describe a novel set of anatomical parameters characterizing the developmental process as well as the utility of these parameters in preoperative planning and a feasibility assessment of EEAs for neurosurgical treatment of skull base lesions in children. METHODS: Based on specific MRI sequences in 107 pediatric patients (2-16 years of age) without skull base lesions (referred to here as the normal population), 3 sets of anatomical parameters were analyzed according to age group and sex: drilling distance, restriction sites, and working distance parameters. A separate set of patients undergoing EEAs was analyzed in similar fashion to address the impact of skull base lesions on the developmental process. RESULTS: The volume of the sphenoid sinus significantly increases with age, reaching 6866.4 mm(3) in the 14-16 years age group, and directly correlates with the pneumatization type (r = 0.533, p = 0.0001). The pneumatization process progresses slowly in a temporal-posterior direction, as demonstrated by the growth trend of the sellar width (r = 0.428, p = 0.0001). Nasal restriction sites do not change significantly with age, with little impact on EEAs. The intercarotid distance is significantly different only in the extreme age groups (3.9 mm, p = 0.038), and has an important impact on the transsphenoidal angle and the intracranial dissection limits (r = 0.443, p < 0.0001). The 14.9° transsphenoidal angle at 2-4 years has a 37.6% significant increase in the 11-13 years age group (p = 0.001) and is highly dependent on pneumatization type. Age-dependent differences between working parameters are mostly noted for the extreme age groups, such as the 8.6-mm increase in nare-vomer distance (p = 0.025). The nare-sellar distance is the only parameter with significant differences based on sex. Skull base lesions induce a high degree of variance in skull base measurements, delaying development and decreasing parameter values. Skull base parameters are interdependent. Nare-sellar distance can be used to assess global skull base development because it highly correlates with the intercarotid distance in both the normal population and in patients harboring skull base lesions. CONCLUSIONS: Skull base development is a slow, gradual, age-dependent, sex-independent process significantly altering endonasal endoscopic corridors. Preoperative MRI measurements of the pediatric skull base are thus a useful adjunct in choosing the appropriate corridor and in assessing working angles and limits during dissection or reparative surgery. Skull base lesions can significantly impact normal skull base development and age-dependent growth patterns.

Gasket seal closure for extended endonasal endoscopic skull base surgery: efficacy in a large case series.

OBJECTIVE: To assess long-term efficacy of the gasket seal, a method for watertight closure of the cranial base using autologous fascia lata held in place by a rigid buttress, in a large case series. METHODS: A prospectively acquired database of all endonasal endoscopic surgeries performed over a 5-year period at Weill Cornell Medical College starting in September 2005 was reviewed. RESULTS: The gasket seal was used in 46 consecutive patients. Mean age was 53 years (range 7-83 years). All patients had extensive intracranial disease with a significant intraoperative cerebrospinal fluid (CSF) leak. Pathology included craniopharyngioma (39.1%), meningioma (23.9%), and pituitary adenoma (17.4%). After a mean follow-up of 28 months (range 3-63 months), two (4.3%) patients had a postoperative CSF leak. Excluding the patients with adenomas, the CSF leak rate was 5.2% (2 of 38 patients). One leak was controlled with reoperation, and the other was stopped with a lumbar drain (LD). The significance of pathology, type of approach, exposure of the ventricular system, use of fat graft, use of nasoseptal (NS) flap, and use of lumbar drain (LD) was examined, and none of these were significant predictors of postoperative CSF leak. CONCLUSIONS: Gasket seal closure is a reliable long-term effective method for achieving watertight closure of the cranial base. It can be used in association with an intracranial fat graft, NS flap, LD, and tissue sealants. In this series, none of these other factors were significant predictors of postoperative CSF leak.

Endoscopic balloon dilation as an adjunct to extended endoscopic approaches to the skull base.

Cholesterol granulomas (CGs) are benign, expanding cystic lesions surrounded by a thick fibrous capsule and filled with fluid, formed by the degradation of blood elements. The goal of surgery is to open the granuloma widely, creating a well-drained cavity. The endonasal endoscopic approach for this extradural lesion is a minimal access method for surgical removal or fenestration. The role of balloon dilation in creating a wide fenestration has not been previously described. The authors describe a patient with a recurrent petrous apex CG who underwent an endoscopic, endonasal, transmaxillary transpterygoid approach to the petrous apex. A balloon sinuplasty catheter was used to dilate the surgical fenestration to maintain continued patency. The authors report on their first experience using balloon dilation combined with endoscopic drainage of the petrous apex. The excellent surgical outcome of this minimally invasive technique holds promise for future endonasal approaches to the middle cranial fossa.

Use of a side-cutting aspiration device for resection of tumors during endoscopic endonasal approaches.

OBJECT: Accessing intra- and extradural tumors via an endonasal approach requires working safely in a relatively narrow area with unobstructed visibility. The authors describe their experience to highlight the utility of a side-cutting aspiration device for endoscopic endonasal resection of skull base tumors. METHODS: The authors used this device in 13 nonconsecutive endoscopic endonasal procedures for different skull base tumors (8 pituitary macroadenomas, 2 craniopharyngiomas, 1 chordoma, 1 recurrent ependymoma, and 1 lymphoma). Illustrative cases and video are presented to demonstrate its use. RESULTS: The instrument was easy to use and effective in the removal of the lesions presented in this series. In 10 patients (77%), gross-total resection was possible; in the other 3 patients (23%), more than 80% of the tumor was resected. No collateral tissue damage or any other complication resulted from device-related debulking or aspiration. CONCLUSIONS: The side-cutting tissue resector is a safe, easy to use, and effective tool for internal debulking and extracapsular dissection of nonvascularized tumors that are too firm for bimanual suction or blunt ring curette dissection. It is particularly useful when working through a deep and narrow corridor such as is encountered in endoscopic endonasal skull base surgery.

A look at Mayan artificial cranial deformation practices: morphological and cultural aspects.

Induced deformation of the cranial vault is one form of permanent alteration of the body that has been performed by human beings from the beginning of history as a way of differentiating from others. These procedures have been observed in different cultures, but were particularly widespread in Mesoamerica. The authors examined and reviewed the historical and anthropological literature of intentional deformation practices in Mayan culture. The Mayans performed different types of cranial deformations and used different techniques and instruments to deform children's heads. The most remarkable morphological alteration is seen in the flattening of the frontal bone. Some archeological investigations link deformation types with specific periods. This article provides a glance at the cultural environment of the Mayans and demonstrates the heterogeneity of this interesting cultural phenomenon, which has changed over time.

Choreoacanthocytosis in a Mexican family.

BACKGROUND: Choreoacanthocytosis (CHAC) (Online Mendelian Inheritance in Man accession No. 200150) is a hereditary neurodegenerative syndrome characterized by movement disorders, cognitive decline, myopathy, behavioral changes, and acanthocytosis and is caused by mutations in the VPS13A gene. OBJECTIVE: To describe the cases of 2 Mexican women with clinical and molecular characteristics compatible with CHAC. DESIGN: Case reports. Patients Choreoacanthocytosis was identified in 2 Mexican mestizo sisters with healthy consanguineous parents. Clinical manifestations began at different ages. RESULTS: The onset of signs and symptoms of CHAC in the proband was at age 32 years and was characterized by balancing problems followed by chorea, compulsive lip and tongue biting with buccolingual self-mutilation, dysarthria, dysphagia, and weight loss. The first clinical manifestations in the proband's sister occurred at age 45 years and included multiple motor and verbal tics, with coprolalia, followed by lip and tongue biting, self-mutilation, and chorea. The clinical findings in both sisters were remarkable for acanthocytosis that developed late, when neurologic changes were already evident. Mutation screening of the VPS13A gene revealed homozygosity for the frameshift mutation c.3556_3557dupAC in exon 33. Currently, the proband's sister, in whom neurologic defects developed 13 years after onset of CHAC in the proband, is the least affected. CONCLUSIONS: The same mutation of the VPS13A gene can be expressed differently in the same family. This observation confirms the notion that there is considerable heterogeneity in the clinical manifestation of CHAC.