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Jothy Kandasamy and Mark Souweidane

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John A. Emelifeonwu, Drahoslav Sokol, Pasquale Gallo, Jothy Kandasamy and Chandrasekaran Kaliaperumal

The authors report a case of a child with hypothalamic-origin pilocytic astrocytoma and hydrocephalus, which was refractory to treatment with a ventriculoperitoneal shunt due to high CSF protein content. With parental education, the child's hydrocephalus was managed long-term in the community with a long-tunnelled external ventricular drain, which was maintained by his parents. To the authors' knowledge this is the first report of this management option as a long-term measure. No harm has come to the patient. The authors propose long-term, long-tunnelled external ventricular drain as a viable treatment option for such patients.

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Gianluca Agresta, Drahoslav Sokol, Chandrasekaran Kaliaperumal, Jothy Kandasamy and Pasquale Gallo

Neurenteric cysts (NCs) are rare congenital lesions with epithelial mucin-secreting walls. They can occur anywhere along the neural axis, and an intrinsic midbrain cyst is extraordinary. Surgical management may pose a challenge due to the location of the lesion and adhesion of the cyst wall to the surrounding brainstem. The authors describe the first case of pediatric NC that was treated successfully with intracystic interferon-α (IFN-α).

A 16-month-old baby girl presented with a 2-week history of progressive croup, vomiting, and swallowing difficulty. MRI revealed a 1.8-cm cystic intrinsic lesion in the pontomedullary region. She initially underwent posterior fossa craniotomy and drainage of the cyst under intraoperative neurophysiology monitoring. Three weeks following the procedure, her symptoms recurred, and follow-up MRI demonstrated cystic recurrence. She underwent repeat aspiration of the cyst and biopsy of the cyst wall, and INF-α-2b was injected into the cystic cavity. Her symptoms improved and completely resolved after 5 months. A 9-month follow-up brain MRI study showed complete resolution of the NC. Intracystic IFN-α injection after cystic content aspiration may be a safe treatment option for the management of intrinsic brainstem NCs. Long-term clinical and radiological follow-up is recommended.

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Michael D. Jenkinson, Caroline Hayhurst, Mohammed Al-Jumaily, Jothy Kandasamy, Simon Clark and Conor L. Mallucci

Object

Endoscopic third ventriculostomy (ETV) is the treatment of choice for hydrocephalus, but the outcome is dependent on the cause of this disorder, and the procedure remains principally the preserve of pediatric neurosurgeons. The role of ETV in adult patients with hydrocephalus was therefore investigated.

Methods

One hundred ninety adult patients underwent ETV for hydrocephalus. Cases were defined as primary ETV (newly diagnosed, without a previously placed shunt) and secondary ETV (performed for shunt malfunctions due to infection or mechanical blockage). Causes of hydrocephalus included tumor, long-standing overt ventriculomegaly (LOVA), Chiari malformation Types I and II (CM-I and -II), aqueduct stenosis, spina bifida, and intraventricular hemorrhage (IVH). Successful ETV was defined as resolution of symptoms with shunt independence. Operative complications and ETV failure rate were investigated according to the causes of hydrocephalus and between the primary and secondary ETV groups.

Results

In the primary group, ETV was successful in 107 (83%) of 129 patients, including those with tumors (52 of 66), LOVA (21 of 24), CM-I (11 of 11 cases), CM-II (8 of 9), aqueduct stenosis (8 of 9), and IVH (2 of 2). In the secondary group, ETV was successful in 41 (67%) of 61 patients and was equally successful in cases of mechanical shunt malfunction (35 of 52 patients) and infected shunt malfunction (6 of 9 patients). The median time to ETV failure was 1.7 months in the primary group and 0.5 months in the secondary group. The majority of ETV failures occurred within the first 3 months, and thereafter, the Kaplan-Meier survival curves plateaued. There were no procedure-related deaths, and complications were seen in only 5.8% of cases.

Conclusions

The success rate of ETVs in adults is comparable, if not better, than in children. In addition to the well-defined role of ETV in the treatment of hydrocephalus caused by tumors and aqueduct stenosis, ETV may also have a role in the management of CM-I, LOVA, persistent shunt infection, and IVH resistant to other CSF diversion procedures.

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Nii K. Addo, Sheila Javadpour, Jothy Kandasamy, Paul Sillifant, Paul May and Ajay Sinha

Object

The association of Chiari malformation Type I (CM-I) with syndromic craniosynostosis (SC) in children is well established. Central sleep apnea (CSA) may subsequently occur. However, sleep studies performed in these patients have been focused mainly on assessing the severity of obstructive sleep apnea. Therefore, the incidence and management of CSA in these patients remains poorly defined. Authors of this study aimed to assess the efficacy of foramen magnum decompression (FMD) in resolving CSA, initially detected incidentally, in a small cohort of patients with CM-I and SC.

Methods

The clinical data for 5 children who underwent FMD for CSA at Alder Hey Children's Hospital between December 2007 and December 2009 were retrospectively analyzed. Outcomes were evaluated with respect to FMDs by utilizing pre- and postdecompression sleep studies. Of the 5 patients, 2 had Crouzon syndrome and 3 had Pfeiffer syndrome.

Results

Patient age at the time of surgery ranged from 1.1 to 12.6 years (median 4.1 years). The median postoperative follow-up was 3.6 years. Sleep studies revealed that 2 children experienced a > 80% reduction in CSAs at 1.5 and 21 months after decompression. The remaining 3 children experienced a > 60% reduction in CSAs when reevaluated between 2 and 10 months after decompression. The associated central apnea index improved for all patients.

Conclusions

Findings suggested that FMD is an effective treatment modality for improving CSA in patients with SC and associated CM-I. The use of multimodal polysomnography technology may improve the evaluation and management of these patients.

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Caroline Hayhurst, Tjemme Beems, Michael D. Jenkinson, Patricia Byrne, Simon Clark, Jothy Kandasamy, John Goodden, Rishi D.S. Nandoe Tewarie and Conor L. Mallucci

Object

As many as 40% of shunts fail in the first year, mainly due to proximal obstruction. The role of catheter position on failure rates has not been clearly demonstrated. The authors conducted a prospective cohort study of navigated shunt placement compared with standard blind shunt placement at 3 European centers to assess the effect on shunt failure rates.

Methods

All adult and pediatric patients undergoing de novo ventriculoperitoneal shunt placement were included (patients with slit ventricles were excluded). The first cohort underwent standard shunt placement using anatomical landmarks. All centers subsequently adopted electromagnetic (EM) navigation for routine shunt placements, forming the second cohort. Catheter position was graded on postoperative CT in both groups using a 3-point scale developed for this study: (1) optimal position free-floating in CSF; (2) touching choroid or ventricular wall; or (3) intraparenchymal. Episodes and type of shunt revision were recorded. Early shunt failure was defined as that occurring within 30 days of surgery. Patients with shunts were followed-up for 12 months in the standard group, for a median of 6 months in the EM-navigated group, or until shunt failure.

Results

A total of 75 patients were included in the study, 41 with standard shunts and 34 with EM-navigated shunts. Seventy-four percent of navigated shunts were Grade 1 compared with 37% of the standard shunts (p = 0.001, chi-square test). There were no Grade 3 placements in the navigated group, but 8 in the standard group, and 75% of these failed. Early shunt failure occurred in 9 patients in the standard group and in 2 in the navigated group, reducing the early revision rate from 22 to 5.9% (p = 0.048, Fisher exact test). Early shunt failures were due to proximal obstruction in 78% of standard shunts (7 of 9) and in 50% of EM-navigated shunts (1 of 2).

Conclusions

Noninvasive EM image guidance in shunt surgery reduces poor shunt placement, resulting in a significant decrease in the early shunt revision rate.