Joon-Ki Kang

Shunt-independent surgical treatment of middle cranial fossa arachnoid cysts in children

Abstractu2002The best operative intervention for children with arachnoid cysts remains the subject of controversy. Recent reports stress that craniotomy for cyst fenestration is associated with a low incidence of morbidity and mortality and may leave the child shunt-independent. Among a total of 66 intracranial arachnoid cysts operated on in the authors’ department from 1985 to 1997, 44 cases (67%) were located in the middle cranial fossa. A higher incidence in the first decade of life (53 cases) and a marked male predominance (45 cases) were recognized. Headache, cranial deformities, symptoms of raised intracranial pressure, and seizures constituted the most frequent features of the clinical presentation. To determine which treatment provides the greatest benefit with the lowest incidence of complications, the records of the 44 patients with arachnoid cysts in the middle cranial fossa were reviewed. The mean age of these patients was 4.6 years (range 0–16 years). Different types of initial surgical procedures were performed. In 33 patients with middle cranial fossa arachnoid cysts (MCFAC) the initial surgery took the form of craniotomy with excision of the cyst walls and fenestration into the basal cisterns. Shunting procedures were performed in 9 patients: cysto-peritoneal shunts (CPS) were placed in 4 patients and ventriculo-peritoneal shunts (VPS), in 3 patients, and cyst excision was performed in addition to CPS in 2 patients. Excision of the cyst membrane alone without fenestration was performed in 2 patients. The initial treatment was successful in terms of reduced symptoms and decreased cyst size, with no additional treatment needed for the cyst, in 79% (26/33) of patients who had undergone excision of the cyst walls and fenestration into the basal cisterns, compared with 66% (6/9) of patients who had undergone shunting procedures. Cyst membrane excision was not successful in any of the patients who underwent this procedure alone. No significant difference in morbidity was noted between these different treatment options. On follow-up CT scan and MRI, cysts of types I and II (Galassi classification) exhibited a steady tendency to reduction or obliteration. These results confirm that radical excision of the outer and inner membranes of the cyst wall with fenestration into the basal cistern is a safe and effective shunt-independent procedure for MCFAC, especially for those of types I and II.

Brain tumors diagnosed in the first year of life in five Far-Eastern countries

A statistical survey is presented of brain tumors diagnosed in the first year of life (from five Far-Eastern countries) in relation to the racial differences in tumor types, congenital factors, and general clinical features. Of the 307 cases collected, 262 were verified histologically, and astrocytomas comprised 23.3%, medulloblastomas 17.2%, ependymomas 11.1%, choroid plexus papillomas 10.7%, teratomas 8.4%, primitive neuroectodermal tumors 4.2%, meningiomas 2.3%, and others 22.9%. There were statistically significant racial differences in comparison with the worldwide survey done by the International Society for Pediatric Neurosurgery Education Committee (1987) on the same subject. In the Far-Eastern population, medulloblastoma and teratoma were more common (P<0.05), whereas astrocytoma was less frequent (P<0.01) than reported in the worldwide survey. The malformative factors were suggested in 18 cases in which various associated congential anomalies were observed. Vascular anomalous lesions, mostly in the extracranial organs, were most common, comprising 61.1% of the associated malformations. Hereditary factors were less commonly demonstrated in these tumors than were anomalies in the major congenital central nervous system. Among the 307 cases, there was one instance (0.3%) of nearly identical tumors occurring in twin brothers. The specific clinical manifestations of brain tumors involving the immature brain were again apparent in this survey, as were the poor survival rates and poor functional prognosis.

Combined direct and indirect reconstructive vascular surgery on the fronto-parieto-occipital region in Moyamoya disease

Between January 1992 and December 1995, eight patients with Moyamoya disease, aged from 2 to 39 years, underwent encephalo-duro-arterio-myo-synangiosis (EDAMS) on the frontal region, superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis combined with encephalo-myo-synangiosis (EMS) on the parietal region and encephaloduro-arterio-synangiosis (EDAS) on the occipital region using the frontal and parietal branch of the STA and the occipital artery, respectively. The development of postoperative collateral formation was assessed by carotid angiography and the improvement of clinical symptoms was evaluated for over 1 year after the bypass surgery. Of the 13 sides which underwent EDAMS and STA-MCA anastomosis with EMS, 11 sides resulted in extensive revascularization on the frontoparietal region and two sides showed localized collaterals, whereas EDAS on the occipital region demonstrated extensive and localized revascularization in each four sides and no evidence of revascularization in two sides among ten sides which underwent the EDAS. The clinical improvement due to the combined reconstructive surgery was very excellent in the reduction of the incidence of transient ischemic attacks (TIA) and reversible ischemic neurologic deficits (RIND).

Surgical Decompression of Supratentorial Arachnoid Cysts in Pediatric Patients Younger than One Year

Background: There has been much debate about the optimal surgical management of arachnoid cysts in infants younger than 1 year of age. The authors present the clinical and neuroimaging findings in pediatric patients with supratentorial arachnoid cysts treated by surgical decompression. Methods: A retrospective chart review was conducted to identify all pediatric patients who had been diagnosed with arachnoid cysts since 2003. Five infants with symptomatic supratentorial arachnoid cysts underwent surgery at Kangnam St. Mary’s Hospital. Results: The initial surgery in 4 patients involved a craniotomy with the excision of the cyst wall and fenestration into the basal cisterns. Three of the four patients needed additional procedures, such as cyst-peritoneal or subdural-peritoneal shunting due to failure of cyst resolution. A shunting procedure was performed as initial therapy in 1 patient who presented with increased intracranial pressure. Conclusion: Our strategy for approaching arachnoid cysts in pediatric patients has been to diagnose and treat the cysts as early as possible and to avoid the complications of shunts whenever possible. However, open craniotomy with excision and fenestration of the cyst is associated with a relatively high failure rate in pediatric patients younger than 1 year of age with supratentorial arachnoid cysts.

Radicular Compression by Intraspinal Epidural Gas Bubble Occurred in Distant Two Levels after Lumbar Microdiscectomy

The authors report a case of symptomatic epidural gas accumulation 2 weeks after the multi-level lumbar surgery, causing postoperative recurrent radiculopathy. The accumulation of epidural gas compressing the dural sac and nerve root was demonstrated by CT and MRI at the distant two levels, L3-4 and L5-S1, where vacuum in disc space was observed preoperatively and both laminectomy and discectomy had been done. However, postoperative air was not identified at L4-5 level where only laminectomy had been done in same surgical field, which suggested the relationship between postoperative epidural gas and the manipulation of disc structure. Conservative treatment and needle aspiration was performed, but not effective to relieve patients symptoms. The patient underwent revision surgery to remove the gaseous cyst. Her leg pain was improved after the second operation.

Management of craniopharyngioma in children: the present and future

Abstract Although controversy still exists regarding the optimum treatment of craniopharyngiomas, all neurosurgeons agree that total removal of the tumor is desirable if it can be done safely. However, successful radical excision of craniopharyngiomas remains one of the most difficult procedures in the era of microsurgery. Early enthusiasms for attempts at curative surgery were tempered, however, by the eventual realization that not only had this tumor not always been completely removed but the patients were often left severely damaged. Recognition of the morbidity of treatment led to a vogue for more conservative methods of treatment cyst aspiration and radiosurgery. The authors would like to discuss the management of childhood craniopharyngiomas for not only the present situation—the situation of todays surgery and hormonal therapy—but also for the future—new surgical strategies, new anatomical approaches, prevention of recurrence, cystic instillations, radiotherapy, and maintaining acceptable functional state hormonally and psychologically. What has been learned from our experience that might help us in the future to achieve the ultimate goal of decompressing the visual apparatus and preventing tumor recurrence at the same time as preserving normal hypothalamic and pituitary functions? In the management of childhood craniopharyngiomas, finally, we would like to list a series of questions to further increase our knowledge of the behavior of these difficult tumors: 1. Should we acknowledge that some childhood craniopharyngiomas are surgically incurable if an unacceptable disability in terms of hypothalamic damage is to be avoided? 2. Can such tumors be identified before surgical treatment is attempted? 3. Can the response of an individual tumor to radiotherapy be predicted? 4. Will the use of stereotactically focused external fractionated radiotherapy allow us to reduce the age (prevention of damaging the childs cognitive behavior)? 5. Is there a role for radiosurgery? 6. What is the role of intracyst instillation? 7. How can surgeons choose the most appropriate surgical approaches, based on the size and location of the tumors? 8. How long should endocrinological follow-up be continued before a child can be considered cured?

Thoracolumbar teratoma associated with meningomyelocele: common aetiology or coincidence?

This commentary refers to the article http://dx.doi.org/10.1007/s00381-002-0586-z tive yolk sac. However, a dysembryogenic origin of intraspinal teratoma is suggested by following investigations. First, there may be an association between the dysraphic process and teratomas [3]. Second, recent embryological investigations have demonstrated the pluripotential nature of the developing caudal spinal cord, which can potentially form all three germ layers [4]. Third, a dysembryogenic origin has recently been proposed for other neoplasms that typically occur in the midline. Another viewpoint is that a neoplastic transformation of heterotopic primordial elements may be the result of the overgrowth of neural tissue due to interaction between intrauterine teratogenic factors and familial predisposition [1]. Another hypothesis is that neural tube defects are produced by an inadequate supply of nutrients to the rapidly growing neural folds. These vascular abnormalities are considered to be the primary malformations that lead to neural tube defects [5]. Mutated genes and/or deficiency of inductive cellular mechanism during embryogenesis in combination with the pluripotential cell mass of the spinal cord may lead to the development of this rare association [2]. I suggest that these teratomas are a product of abnormal development of the pluripotential embryonic spinal cord mesenchyme, especially the caudal cell mass. The fact that each teratoma arose in an area of known aberrant embryological development also indicates that the origin of each teratoma is a part of the same dysembryogenic process. In this reported case of neonatal intraspinal teratoma associated with meningomyelocele, the work-up studies, step-bystep management of repair of the meningomyelocele, removal of the tumour and shunt surgery were rationally adequate and successfully attained a good outcome.