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Meng-Fai Kuo, Yihsin Tsai, Wen-Ming Hsu, Ruei-Sheng Chen, Yong-Kwang Tu and Huei-Shyong Wang

Object

Vertebral defects, anal atresia, cardiovascular anomalies, tracheoesophageal fistulas (TEFs), renal anomalies, and limb defects (most often of the radius) are commonly associated and known collectively by the acronym VACTERL. The authors studied these nonrandomly associated birth defects to determine if a further relationship exists between VACTERL association and the presence of a tethered spinal cord (TSC).

Methods

From 2001 to 2004, 12 patients with VACTERL association who were treated operatively by a single pediatric surgeon underwent magnetic resonance (MR) imaging to evaluate the intraspinal abnormalities that may cause tethering. Three patients were excluded from the study due to complications after surgery for TEF. Coincidentally, these three patients did not have imperforate ani. In the remaining nine patients, seven had associated urogenital anomalies, and six of these also had high-type imperforate ani. Five of the six patients and the one patient with low-type imperforate anus and a urogenital anomaly were found to have TSCs. In the remaining two patients without urogenital anomalies there was a high-type imperforate anus without a TSC in one patient and a low-type imperforate anus with a TSC in the other. All seven patients with TSCs underwent successful untethering. The lesions contributing to TSC included terminal filum lipomas (TFLs) in five patients, an intramedullary ependymal cyst in one patient, and a lipomeningomyelocele in another patient.

Conclusions

The authors found that in patients with VACTERL association there was a high incidence of TSC (seven of nine patients) if an imperforate anus was present as one of the anomalies. In patients with VACTERL association and urogenital anomalies, the incidence of TSC was even higher (86%). Five of the seven cases of TSC in the present study were caused by a TFL, a lesion that can be easily and safely managed surgically. The authors conclude that MR imaging is essential for ruling out the possibility of a TSC in patients with VACTERL association combined with urogenital anomalies or an imperforate anus.

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Meng-Fai Kuo, Huei-Shyong Wang, Quang-Ting Kuo, Chia-Tung Shun, Hey-Chi Hsu, Shih-Hong Yang and Ray-Hwang Yuan

Object

Stathmin, an important cytosolic phosphoprotein, is involved in cell proliferation and motility. This study was performed to elucidate the role of stathmin in the progression of medulloblastoma.

Methods

The expression of stathmin protein was examined by immunohistochemical staining of tumor sections obtained in 17 consecutive patients with medulloblastoma who underwent resection between 1995 and 2005. Four patients were excluded because they were either lost to follow-up or underwent biopsy sampling only, leaving a total of 13 patients in the study. The stathmin expression was scored according to the immunoreactive fraction of tumor cells, and the level was correlated with various clinicopathological factors.

Results

The expression level of stathmin protein was ≤ 10% in 9 patients, 11–50% in 1, and > 50% in 3. No staining was seen in the tissues adjacent to the tumors. For comparison, the authors grouped the expression level of stathmin into high (> 50%) and low (≤ 50%). It was found that patients with high expression of stathmin had more frequent tumor dissemination at the time of resection or soon after total excision of the tumor (p = 0.0035), and hence experienced a fulminant course with lower patient survival (p < 0.0001), with an average survival period of 6.7 months (range 2–10 months). The expression level of stathmin did not correlate with patient age, sex, CSF cytological findings, use of adjuvant therapies, Ki 67 index, or risk classification of the tumors according to previously described categories in the literature.

Conclusions

High stathmin expression correlates with tumor dissemination, is an important prognostic factor of medulloblastoma, and may serve as a useful marker for more intensive adjuvant therapies.

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Shin-Joe Yeh, Sung-Chun Tang, Li-Kai Tsai, Chung-Wei Lee, Ya-Fang Chen, Hon-Man Liu, Shih-Hung Yang, Yu-Lin Hsieh, Meng-Fai Kuo and Jiann-Shing Jeng

OBJECTIVE

Pediatric and adult patients with moyamoya disease experience similar clinical benefits from indirect revascularization surgeries, but there are still debates about age-related angiographic differences of the collaterals established after surgery. The goal of this study was to assess age-related differences on ultrasonography before and after indirect revascularization surgeries in moyamoya patients, focusing on some ultrasonographic parameters known to be correlated with the collaterals supplied by the external carotid artery (ECA).

METHODS

The authors prospectively included moyamoya patients (50 and 26 hemispheres in pediatric and adult patients, respectively) who would undergo indirect revascularization surgery. Before surgery and at 1, 3, and 6 months after surgery, the patients underwent ultrasonographic examinations. The ultrasonographic parameters included peak-systolic velocity (PSV), end-diastolic velocity (EDV), resistance index (RI), and flow volume (FV) measured in the ECA, superficial temporal artery (STA), and internal carotid artery on the operated side. The mean values, absolute changes, and percentage changes of these parameters were compared between the pediatric and adult patients. Logistic regression analysis was used to clarify the determinants affecting postoperative EDV changes in the STA.

RESULTS

Before surgery, the adult patients had mean higher EDV and lower RI in the STA and ECA than the pediatric group (all p < 0.05). After surgery, the pediatric patients had greater changes (absolute and percentage changes) in the PSV, EDV, RI, and FV in the STA and ECA (all p < 0.05). The factors affecting postoperative EDV changes in the STA at 6 months were age (p = 0.006) and size of the revascularization area (i.e., revascularization in more than the temporal region vs within the temporal region; p = 0.009). Pediatric patients who received revascularization procedures in more than the temporal region had higher velocities (PSV and EDV) in the STA than those who received revascularization within the temporal region (p < 0.05 at 1–6 months), but such differences were not observed in the adult group.

CONCLUSIONS

The greater changes of these parameters in the STA and ECA in pediatric patients than in adults after indirect revascularization surgeries indicated that pediatric patients might have a greater increase of collaterals postoperatively than adults. Pediatric patients who undergo revascularization in more than the temporal region might have more collaterals than those who undergo revascularization within the temporal region.