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Mazhar Husain, Deepak K. Jha, Sunil Agrawal, Nuzhat Husain and Rakesh K. Gupta

Object. The instrumentation for endoscopic discectomy continues to evolve to allow for acceptable clinical outcomes and expanding applications. The authors describe their experience in using a conical working tube equipped with a guide for angular entry of the telescope to perform endoscopic discectomy in patients with lumbar disc herniation.

Methods. Fifty-one patients (38 men and 13 women) with herniated lumbar discs underwent endoscopic lumbar surgery during the past 2.5 years at the authors' institutions. A conical working tube was inserted over sequential coaxial dilators via a muscle-splitting approach. Conventional neurosurgical instruments were used in conjunction with an angled insertion telescope. Endoscopic discectomy was performed at the L1–2 (one case), L3–4 (two cases), L4–5 (32 cases), and L5—S1 (18 cases) levels. The surgical approach was bilateral in two patients: bilateral L4–5 in one, and right L4–5 and left L5—S1 in the other. The remaining patient suffered adjacent two-level (right-sided L4–5 and L5—S1) herniations. Outcome was assessed at a mean of 11 months after surgery by using modified Macnab criteria.

Outcomes were excellent in 46 (90%), fair in three (6%), and poor in two patients (4%). Complications occurred in four patients and included a dural tear in one case, postoperative neurological deterioration in two, and discitis in two; in two of these patients open surgical exploration was required.

Conclusions. A separate angled entry of the telescope shortens the effective length of the working sheath and creates a better working space, thereby allowing greater instrument maneuverability and ability to use conventional neurosurgical instruments. In addition, use of this telescope in other endoscopic procedures reduces overall cost of instrumentation and treatment, and results are comparable to those reported in association with microscopic lumbar discectomy.

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Deepak Kumar Gupta and Ashok K. Mahapatra

Object

A terminal myelocystocele is a rare form of spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are herniated through a posterior spina bifida. In their study of 17 cases, the authors attempt to develop treatment guidelines for patients suffering from this condition.

Methods

Seventeen patients (age range 2 months–5 years) underwent surgery during a 7-year period. Surprisingly, nine of 17 patients had no neurological deficits. Among these nine asymptomatic cases, three patients had a dermal sinus and two had a skin dimple, whereas one had congenital talipes equinovarus deformity (CTEV). Among the eight patients with neurological deficits, seven had lower-limb weakness (two had complete paraplegia), and one patient had urinary incontinence and constipation. Four patients each had a dermal sinus and CTEV; interestingly, one infant had an accessory phallus. Radiologically, eight patients had pure myelocystoceles, and a diagnosis of lipomyelocystocele was made in nine instances on the basis of magnetic resonance images. All patients underwent surgery. The meningocele sacs were excised, and the bands tethering the myelocystocele sac to the meningocele sacs were incised, with as little damage to the roots as possible. Two patients experienced postoperative CSF leakage (one required reexploration), and two others had subcutaneous CSF collection. No patient had deterioration of the neurological condition after surgery.

Conclusions

The authors present the largest study of patients with terminal myelocystocele to date and emphasize that the possibility of this condition must be kept in mind when addressing all congenital lumbosacral masses. The authors assert that myelocystoceles and lipomyelocystoceles should be kept in the same category and recommend early surgery in all cases.

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Ashok K. Mahapatra and Deepak Kumar Gupta

Object

Split cord malformations (SCMs) are rare anomalies of the spinal cord. A total of 254 cases of SCM were treated surgically at the authors' center during a period of 16 years.

Methods

Patients' demographic profiles, imaging studies, operative details, complications, and surgical outcomes were evaluated retrospectively. A new classification based on intraoperative findings is proposed.

The mean age of the patients was 7.3 years (female/male 1.5:1). Type I SCM was seen in 156 patients (61.4%) and 98 patients (38.6%) had Type II SCM. Skin stigmata were present in 153 cases (60%); hypertrichosis, being the most common, was seen in 82 cases (32.3%). Asymmetrical lower-limb weakness and sphincter disturbances were present in 173 (68.1%) and 73 (33%) cases, respectively. Of the symptomatic cases, 39% (68 of 173) showed improvement in motor power, 57.9% (33 of 57) experienced sensory improvement, and 27.3% (20 of 73) regained continence. None of the 38 patients in the asymptomatic group had postoperative neurological deterioration. The neurological status was unchanged in 63% of the cases. A new subclassification of Type I SCM is proposed, based on intraoperative location of a bone spur causing the split, which may have a bearing on surgical dissection and outcome. Based on the authors' experience with 25 cases of Type I SCM, they have classified the disorder into four subtypes: Type Ia, bone spur located in the center with duplicated cord above and below the spur (12 cases); Type Ib, bone spur at the superior pole with no space above it (four cases); Type Ic, bone spur at the lower pole with large duplicated cord above (three cases); and Type Id, bone spur straddling the bifurcation with no space above or below the spur (six cases). The risk of injury to the hemicords is highest in the Id subtype (four of six patients in this group deteriorated neurologically in the present series, whereas none with subtypes Ia–c worsened).

Conclusions

The authors present the largest series on SCMs so far reported in the world literature The risk of neurological deficits developing increases with age; hence, all patients with SCM should be surgically treated prophylactically even if they are asymptomatic. This new classification is easy to use and remember and takes into account the use of intraoperative findings that may have a bearing on surgical outcome.

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Shaila Gowda, Fortino Salazar, William E. Bingaman, Prakash Kotagal, Deepak L. Lachhwani, Ajay Gupta, Stephen Davis, Julie Niezgoda and Elaine Wyllie

Object

Few data are available concerning efficacy and safety of surgery for catastrophic epilepsy in the first 6 months of life.

Methods

The authors retrospectively analyzed epilepsy surgeries in 15 infants ranging in age from 1.5 to 6 months (median 4 months) and weight from 4 to 10 kg (median 7 kg) who underwent anatomical (4 patients) or functional (7 patients) hemispherectomy, or frontal (1 patient), frontoparietal (2 patients), or parietooccipital (1 patient) resection for life-threatening catastrophic epilepsy due to malformation of cortical development.

Results

No patient died. Intraoperative complications included an acute ischemic infarction with hemiparesis in our youngest, smallest infant. The most frequent complication was blood loss requiring transfusion, which was encountered in every case. The estimated blood loss was 3–214% (median 63%) of the total blood volume. At maximum follow-up of 6–121 months (median 60 months), 46% were seizure free.

Conclusions

Epilepsy surgery may be effective in young infants as it is in older children. However, intraoperative blood loss and risk of permanent postoperative neurological deficits present significant challenges.

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Elia M. Pestana Knight, Tobias Loddenkemper, Deepak Lachhwani, Prakash Kotagal, Elaine Wyllie, William Bingaman and Ajay Gupta

Object

The aim of this study was to identify the reasons for and predictors of no resection of the epileptogenic zone in children with epilepsy who had undergone long-term invasive subdural grid electroencephalography (SDG-EEG) evaluation.

Methods

The authors retrospectively reviewed the consecutive medical records of children (< 19 years of age) who had undergone SDG-EEG evaluation over a 7-year period (1997–2004). To determine the predictors of no resection, the authors obtained the clinical characteristics and imaging and EEG findings of children who had no resection after long-term invasive SDG-EEG evaluation and compared these data with those in a group of children who did undergo resection. They describe the indications for SDG-EEG evaluation and the reasons for no resection in these patients.

Results

Of 66 children who underwent SDG-EEG evaluation, 9 (13.6%) did not undergo subsequent resection (no-resection group; 6 males). Of these 9 patients, 6 (66.7%) had normal neurological examinations and 5 (55.6%) had normal findings on brain MR imaging. Scalp video EEG localized epilepsy to the left hemisphere in 6 of the 9 patients and to the right hemisphere in 2; it was nonlocalizable in 1 of the 9 patients. Indications for SDG-EEG in the no-resection group were ictal onset zone (IOZ) localization (9 of 9 patients), motor cortex localization (5 of 9 patients), and language area localization (4 of 9 patients). Reasons for no resection after SDG-EEG evaluation were the lack of a well-defined IOZ in 5 of 9 patients (4 multifocal IOZs and 1 nonlocalizable IOZ) and anticipated new permanent postoperative neurological deficits in 7 of 9 patients (3 motor, 2 language, and 2 motor and language deficits). Comparison with the resection group (57 patients) demonstrated that postictal Todd paralysis in the dominant hand was the only variable seen more commonly (χ2 = 4.781, p = 0.029) in the no-resection group (2 [22.2%] of 9 vs 2 [3.5%] of 57 patients). The no-resection group had a larger number of SDG electrode contacts (mean 126. 5 ± 26.98) as compared with the resection group (100.56 ± 25.52; p = 0.010). There were no significant differences in the demographic data, seizure characteristics, scalp and invasive EEG findings, and imaging variables between the resection and no-resection groups.

Conclusions

Children who did not undergo resection of the epileptogenic zone after SDG-EEG evaluation were likely to have normal neurological examinations without preexisting neurological deficits, a high probability of a new unacceptable permanent neurological deficit following resection, or multifocal or nonlocalizable IOZs. In comparison with the group that underwent resection after SDG-EEG, a history of Todd paralysis in the dominant hand and arm was the only predictor of no resection following SDG-EEG evaluation. Data in this study will help to better select pediatric patients for SDG-EEG and to counsel families prior to epilepsy surgery.

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Amey Savardekar, Manjul Tripathi, Deepak Bansal, Kim Vaiphei and Sunil K. Gupta

Langerhans cell histiocytosis (LCH) of the CNS is a rare entity, known to involve primarily the hypothalamicpituitary region, with the clinical hallmark of diabetes insipidus. There have been a few reports of CNS LCH involving the brainstem as intraparenchymal enhancing lesions, but this has never been the presenting complaint of LCH. The authors report on a 7-year-old boy who presented with right cerebellopontine syndrome, in whom a well-defined, solid, enhancing lesion in the brainstem was diagnosed. Clinicoradiological differential diagnosis included glioma and tuberculosis. Biopsy revealed atypical histiocytes positive for CD68, CD1a, and S100 protein; these are the diagnostic features of LCH on histopathological examination. The rapid growth of the lesion was controlled with a chemotherapeutic regimen of cladribine.

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Akash Mishra, Deepak Agrawal, Deepak Gupta, Sumit Sinha, Guru D. Satyarthee and Pankaj K. Singh

OBJECT

Spondyloptosis represents the most severe form of spondylolisthesis, which usually follows high-energy trauma. Few reports exist on this specific condition, and the largest series published to date consists of only 5 patients. In the present study the authors report the clinical observations and outcomes in a cohort of 20 patients admitted to a regional trauma center for severe injuries including spondyloptosis.

METHODS

The authors performed a retrospective chart review of patients admitted with spondyloptosis at their department over a 5-year period (March 2008–March 2013). Clinical, radiological, and operative details were reviewed for all patients.

RESULTS

In total, 20 patients with spondyloptosis were treated during the period reviewed. The mean age of the patients was 27 years (range 12–45 years), and 17 patients were male (2 boys and 15 men) and 3 were women. Fall from height (45%) and road traffic accidents (35%) were the most common causes of the spinal injuries. The grading of the American Spinal Injury Association (ASIA) was used to assess the severity of spinal cord injury, which for all patients was ASIA Grade A at the time of admission. In 11 patients (55%), the thoracolumbar junction (T10–L2) was involved in the injury, followed by the dorsal region (T1–9) in 7 patients (35%); 1 patient (5%) had lumbar and 1 patient (5%) sacral spondyloptosis. In 19 patients (95%), spondyloptosis was treated surgically, involving the posterior route in all cases. In 7 patients (37%), corpectomy was performed. None of the patients showed improvement in neurological deficits. The mean follow-up length was 37.5 months (range 3–60 months), and 5 patients died in the follow-up period from complications due to formation of bedsores (decubitus ulcers).

CONCLUSIONS

To the authors' best knowledge, this study was the largest of its kind on traumatic spondyloptosis. Its results illustrate the challenges of treating patients with this condition. Despite deformity correction of the spine and early mobilization of patients, traumatic spondyloptosis led to high morbidity and mortality rates because the patients lacked access to rehabilitation facilities postoperatively.