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Shyamal C. Bir, Piyush Kalakoti, Christina Notarianni and Anil Nanda

In the late 18th and early 19th centuries, Dr. John Howship, a pioneering British surgeon, described the clinical features and pathophysiology of various surgical disorders of the human body. His critical contributions to pediatric neurosurgery came in 1816 when he first described the features of an important childhood condition following head trauma, what he referred to as parietal bone absorption. This condition as depicted by Dr. Howship was soon to be christened by later scholars as traumatic cephalhydrocele, traumatic meningocele, leptomeningeal cyst, meningocele spuria, fibrosing osteitis, cerebrocranial erosion, and growing skull fracture. Nevertheless, the basic features of the condition as observed by Dr. Howship were virtually identical to the characteristics of the above-mentioned disorders. This article describes the life and accomplishments of Dr. Howship and his contributions to the current understanding of growing skull fracture.

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Shyamal C. Bir, Sudheer Ambekar, Christina Notarianni and Anil Nanda

In the 19th century, Dr. Odilon Marc Lannelongue was a pioneering French surgeon who introduced a surgical technique for the treatment of craniosynostosis. In 1890, Dr. Lannelongue performed correction of sagittal synostosis by strip craniectomy. From his procedure, multiple techniques have been developed and endorsed for this condition, ranging from simple suturectomies to extensive calvarial vault remodeling. In addition, even today, endoscopically aided strip craniectomy is performed as a surgical treatment of craniosynostosis. This article describes the life and works of the surgeon who revolutionized the management of craniosynostosis.

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Prasad Vannemreddy, Christina Notarianni, Krishna Yanamandra, Dawn Napper and Joseph Bocchini Jr

Object

Studies have shown decreased levels of nitric oxide (NO), the product of endothelial NO synthase (eNOS) gene activity, in infants with respiratory conditions and intraventricular hemorrhage (IVH). The authors evaluated the association of the eNOS gene promoter polymorphism T-786C with the cause of these conditions (respiratory conditions and IVH) in premature infants.

Methods

Blood samples from 124 African American premature infants were studied. The DNA was isolated and microplate polymerase chain reaction–restriction fragment length polymorphism assay was performed. Genotypes were scored as: TT homozygotes with 140 bp and 40 bp; CC homozygotes with 90 bp, 50 bp, and 40 bp; and TC heterozygotes with 140 bp, 90 bp, 50 bp, and 40 bp. Genotypes were stratified according to ethnicity, preterm status, and prematurity conditions.

Results

The mutant allele -786C was present in 15.3% of premature infants with respiratory distress syndrome, bronchopulmonary dysplasia, and IVH, compared with 7.25% in those premature infants without these conditions. A significant 2-fold increase of the mutant allele in patients compared with controls (p = 0.04, OR 2.3) reveals that the eNOS -786C allele could be a significant risk factor in the origin of respiratory conditions and IVH in premature infants.

Conclusions

These results suggest that the mutant eNOS -786C allele is a significant risk factor in the origin of respiratory and IVH diseases, probably mediating an insufficient supply of endogenous NO in premature infants.

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Christina Notarianni, Prasad Vannemreddy, Gloria Caldito, Papireddy Bollam, Esther Wylen, Brian Willis and Anil Nanda

Object

Hydrocephalus is a notorious neurosurgical disease that carries the adage “once a shunt always a shunt.” This study was conducted to review the treatment results of pediatric hydrocephalus.

Methods

Pediatric patients who underwent ventriculoperitoneal shunt surgery over the past 14 years were reviewed for shunt revisions. Variables studied included age at shunt placement, revision, or replacement; programmable shunts; infection; obstruction; and diagnosis (congenital, posthemorrhagic, craniospinal dysraphism, and others including trauma, tumors, and infection). Multiple regression analysis methods were used to determine independent risk factors for shunt failure and the number of shunt revisions. The Kaplan-Meier method of survival analysis was used to compare etiologies on the 5-year survival (revision-free) rate and the median 5-year survival time.

Results

A total of 253 patients were studied with an almost equal sex distribution. There were 92 patients with congenital hydrocephalus, 69 with posthemorrhagic hydrocephalus, 48 with craniospinal dysraphism, and 44 with other causes. Programmable shunts were used in 73 patients (other types of shunts were used in 180 patients). A total of 197 patients (78%) underwent revision surgeries due to shunt failures. The mortality rate was 1.6%. Age at first revision, the 5-year survival rate, and the median 5-year survival time were significantly less for both posthemorrhagic and craniospinal dysraphism than for either the congenital or “other” group (p < 0.05). The failure rate and number of revisions were not significantly reduced with programmable shunts compared with either pressure-controlled or no-valve shunts (p > 0.5).

Conclusions

Posthemorrhagic hydrocephalus and craniospinal dysraphism hydrocephalus had significantly earlier revisions than congenital and other etiologies. Programmable systems did not reduce the failure rate or the average number of shunts revisions.

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Prashant Chittiboina, Helena Pasieka, Ashish Sonig, Papireddy Bollam, Christina Notarianni, Brian K. Willis and Anil Nanda

Object

Cerebrospinal fluid shunts in patients with posthemorrhagic hydrocephalus are prone to failure, with some patients at risk for multiple failures. The objective of this study was to identify factors leading to multiple failures.

Methods

The authors performed a retrospective analysis of cases of posthemorrhagic hydrocephalus requiring neurosurgical intervention between 1982 and 2010.

Results

In the 109 cases analyzed, 54% of the patients were male, their mean birth weight was 1223 g, and their mean head circumference 25.75 cm. The mean duration of follow-up was 6 years, and 9 patients died. Grade III intraventricular hemorrhage was seen in 47.7% and Grade IV in 43.1%. Initial use of a ventricular access device was needed in 65 patients (59.6%), but permanent CSF shunting was needed in 104 (95.4%). A total of 454 surgical procedures were performed, including 304 shunt revisions in 78 patients (71.6%). Detailed surgical notes were available for 261 of these procedures, and of these, 51% were proximal revisions, 13% distal revisions, and 17% total shunt revisions. Revision rates were not affected by catheter type, patient sex, presence of congenital anomalies, or type of hydrocephalus. Age of less than 30 days at the initial procedure was associated with decreased survival of the first shunt. Regression analysis revealed that lower estimated gestational age (EGA) and obstructive hydrocephalus were significant predictors of multiple shunt revisions.

Conclusions

We found a high rate of need for permanent CSF shunts (95.4%) in patients with posthemorrhagic hydrocephalus. Shunt revision was required in 71.6% of patients, with those with lower birth weight and EGA at a higher risk for revisions.

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Shyamal C. Bir, Devi Prasad Patra, Tanmoy K. Maiti, Hai Sun, Bharat Guthikonda, Christina Notarianni and Anil Nanda

OBJECTIVE

Adult-onset hydrocephalus is not commonly discussed in the literature, especially regarding its demographic distribution. In contrast to pediatric hydrocephalus, which is related to a primary CSF pathway defect, its development in adults is often secondary to other pathologies. In this study, the authors investigated the epidemiology of adult-onset hydrocephalus as it pertains to different etiologies and in reference to age, sex, and race distributions.

METHODS

The authors retrospectively reviewed the clinical notes of 2001 patients with adult-onset hydrocephalus who presented to Louisiana State University Health Sciences Center within a 25-year span. Significant differences between the groups were analyzed by a chi-square test; p < 0.05 was considered significant.

RESULTS

The overall mean (± SEM) incidence of adult hydrocephalus in this population was 77 ± 30 per year, with a significant increase in incidence in the past decade (55 ± 3 [1990–2003] vs 102 ± 6 [2004–2015]; p < 0.0001). Hydrocephalus in a majority of the patients had a vascular etiology (45.5%) or was a result of a tumor (30.2%). The incidence of hydrocephalus in different age groups varied according to various pathologies. The incidence was significantly higher in males with normal-pressure hydrocephalus (p = 0.03) or head injury (p = 0.01) and higher in females with pseudotumor cerebri (p < 0.0001). In addition, the overall incidence of hydrocephalus was significantly higher in Caucasian patients (p = 0.0002) than in those of any other race.

CONCLUSIONS

Knowledge of the demographic variations in adult-onset hydrocephalus is helpful in achieving better risk stratification and better managing the disease in patients. For general applicability, these results should be validated in a large-scale meta-analysis based on a national population database.

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Devi Prasad Patra, Shyamal C. Bir, Tanmoy K. Maiti, Piyush Kalakoti, Hugo Cuellar, Bharat Guthikonda, Hai Sun, Christina Notarianni and Anil Nanda

OBJECTIVE

Despite significant advances in the medical field and shunt technology, shunt malfunction remains a nightmare of pediatric neurosurgeons. In this setting, the ability to preoperatively predict the probability of shunt malfunction is quite compelling. The authors have compared the preoperative radiological findings in obstructive hydrocephalus and the subsequent clinical course of the patient to determine any association with overall shunt outcome.

METHODS

This retrospective study included all pediatric patients (age < 18 years) who had undergone ventriculoperitoneal shunt insertion for obstructive hydrocephalus. Linear measurements were taken from pre- and postoperative CT or MRI studies to calculate different indices and ratios including Evans' index, frontal horn index (FHI), occipital horn index (OHI), frontooccipital horn ratio (FOHR), and frontooccipital horn index ratio (FOIR). Other morphological features such as bi- or triventriculomegaly, right-left ventricular symmetry, and periventricular lucency (PVL) were also noted. The primary clinical outcomes that were reviewed included the need for shunt revision, time interval to first shunt revision, frequency of shunt revisions, and revision-free survival.

RESULTS

A total of 121 patients were eligible for the analysis. Nearly half of the patients (47.9%) required shunt revision. The presence of PVL was associated with lower revision rates than those in others (39.4% vs 58.2%, p = 0.03). None of the preoperative radiological indices or ratios showed any correlation with shunt revision. Nearly half of the patients with shunt revision required early revision (< 90 days of primary surgery). The reduction in the FOHR was high in patients who required early shunt revision (20.16% in patients with early shunt revision vs 6.4% in patients with late shunt revision, p = 0.009). Nearly half of the patients (48.3%) requiring shunt revision ultimately needed more than one revision procedure. Greater occipital horn dilation on preoperative images was associated with a lower frequency of shunt revision, as dictated by a high OHI and a low FOIR in patients with a single shunt revision as compared with those in patients who required multiple shunt revisions (p = 0.029 and 0.009, respectively). The mean follow-up was 49.9 months. Age was a significant factor affecting shunt revision–free survival. Patients younger than 6 months of age had significantly less revision-free survival than the patients older than 6 months (median survival of 10.1 vs 94.1 months, p = 0.004).

CONCLUSIONS

Preoperative radiological linear indices and ratios do not predict the likelihood of subsequent shunt malfunction. However, patients who required early shunt revision tended to have greater reductions in ventricular volumes on postoperative images. Therefore a greater reduction in ventricular volume is not actually desirable, and a ventricular volume high enough to reduce intracranial pressure is instead to be aimed at for long-term shunt compliance.