No abstract

Bile duct ligation caused a threefold elevation of not only hepatic but also intestinal alkaline phosphatase. The increase was transient in intestinal mucosa and peaked at 12 h. Hepatic phosphatase levels reached a plateau by 36 h. Intraperitoneal injection of bile from ligated animals into normal rats produced qualitatively similar results. Studies were performed to further characterize the induction of phosphatase activity in both tissues. The addition of bile from ligated animals to organ explants of liver and intestine produced a two- and threefold rise in activity, respectively. Therefore, neural and hormonal factors are not essential in producing this induction. The increased activity produced in vitro in both tissues was due to heat-stable and dialyzable factor(s) and was blocked by inhibitors of protein synthesis. In the intestine activity occurred over the entire brush border as demonstrated by electron microscopic histochemistry. In the liver, increased activity was noted over the entire plasma membrane and was not localized to the canalicular membrane. Tunicamycin treatment of liver and intestinal explants markedly suppressed the induced phosphatase activity. Simultaneous treatment with protease inhibitors restored some of the phosphatase activity. These findings suggest that glycosylation of the alkaline phosphatase might provide at least partial protection from proteolysis.

OBJECTIVEChondrodysplasia punctata (CDP), a rare skeletal dysplasia, can lead to cervical spine instability and deformity. However, an optimal neurosurgical intervention has yet to be established. Thus, a retrospective study was conducted to assess the efficacy of various surgical interventions for children with CDP.METHODSThe authors retrospectively reviewed 9 cases of CDP in which cervical decompression with or without posterior fusion was performed between April 2007 and May 2016. Patient demographics, preoperative clinical conditions, radiographic findings, surgical procedures, and the postoperative course were analyzed in detail.RESULTSA total of 12 operations were carried out in 9 patients (8 male, 1 female) during the study period. The patients’ ages at the initial surgery ranged from 2 months to 2 years. Seven of the children had CDPX1, 1 had CDPX2, and 1 had tibia-metacarpal type CDP (CDP-TM). The lesion occurred at the craniovertebral junction (CVJ) in 7 cases and involved a subaxial deformity in 2 cases. The initial surgery was C-1 laminectomy with occipitocervical fusion (OCF) followed by halo external fixation in 5 cases, OCF alone in 1 case, and C-1 laminectomy alone in 3 cases. Three children required additional surgery. In one of these cases, a staged operation was required because the patient’s head was too small to attach a halo ring at the time of the initial procedure (C-1 laminectomy). In another case, OCF was performed 11 months after C-1 laminectomy because of intramedullary signal change on serial MRI, although the child remained asymptomatic. In the third case, additional posterior fusion was performed 17 months after an initial laminectomy and OCF due to newly developed cervical dislocation caudal to the original fusion. This last patient required a third operation 9 months after the second because of deep wound infection. Surgery improved the motor function of all 7 children with CDPX1, but 3 children who had already suffered respiratory failure preoperatively required continued respiratory support. At the time of this report, 7 of the 9 children were alive and in stable condition. One child died due to restrictive respiratory insufficiency, and another died in an accident unrelated to CDP.CONCLUSIONSSurgical decompression with or without fusion for CVJ and subaxial cervical lesions in infants and toddlers with CDP generally saves lives and increases the likelihood of motor function recovery. However, in this case series the patients’ preoperative condition had a strong effect on postoperative respiratory function. The surgery was not straightforward, and a second operation was required in some cases. Nevertheless, the findings indicate that early surgical intervention for CDP with cervical involvement is feasible, suggesting that the role of neurosurgery should be reevaluated.

In the present study, we found that endoscopic fenestration of intraventricular arachnoid cysts in the body to trigone is a safe procedure with a satisfactory outcome. In our limited experience, there are two anatomic backgrounds; velum interpositum cistern and quadrigeminal cistern. Differentiation can be possible by neuroimagings, especially those obtained after surgery.

OBJECTIVESacrococcygeal dimples in neonates and infants are of uncertain pathological import. Previously they were believed to be rarely associated with intraspinal anomalies. Recent studies using MRI, however, revealed that 6%–7% of pediatric cases of sacrococcygeal dimples were associated with anatomical tethered spinal cord (TSC). Because the prevalence of tethered cord syndrome is still unclear, there is no consensus among pediatric neurosurgeons on the management of children with sacrococcygeal dimples. The authors performed an analysis of MRI and urodynamic studies to validate their management strategy for pediatric cases of sacrococcygeal dimples.METHODSA total of 103 Japanese children (49 male and 54 female, median age 4 months, range 8 days–83 months) with sacrococcygeal dimples who were referred to the Division of Pediatric Neurosurgery between 2013 and 2015 were included in this study. The lumbosacral region of all the patients was investigated using MRI. Anatomical TSC was defined as a condition in which the caudal end of the conus medullaris is lower than the inferior border of the L2–3 intervertebral disc. Patients with minor spinal anomalies (e.g., anatomical TSC, filum lipoma, thickened filum, or filar cyst) underwent further urodynamic studies to ascertain the presence of neurogenic bladder (NGB). In this study, the presence of NGB without anatomical TSC but with other minor spinal anomalies was defined as “functional TSC.” The prevalence of anatomical and functional TSC was investigated. The association of the following cutaneous findings with spinal anomalies was also assessed: 1) depth of the dimple, 2) deviation of the gluteal fold, and 3) other skin abnormalities (e.g., discoloration, angioma, or abnormal hair).RESULTSThe children were classified into 4 groups: Group 1, patients with anatomical TSC; Group 2, patients with functional TSC; Group 3, patients without anatomical or functional TSC but with other minor spinal anomalies; and Group 4, patients with no spinal anomaly. There were 6 patients (5.8%) in Group 1, 8 patients (7.8%) in Group 2, 10 patients (9.7%) in Group 3, and 79 patients (76.7%) in Group 4. Twenty-four patients (23.3%; Groups 1, 2, and 3) showed MRI abnormalities, including filum lipoma (14 cases), filar cysts (5 cases), thickened filum (2 cases), and anatomical TSC without other spinal anomalies (3 cases). Untethering of the spinal cord was indicated for 14 patients (13.6%; Groups 1 and 2) with anatomical and functional TSCs. Preoperative NGB was found in 12 patients and improved postoperatively in 7 (58.3%). None of the associated lumbosacral skin findings predicted the presence of underlying spinal anomalies.CONCLUSIONSThe prevalence of tethered cord syndrome among children with sacrococcygeal dimples was, for the first time, revealed to be higher than previously thought. MRI and supplemental urodynamic studies may be indicated for children with sacrococcygeal dimples to identify patients with symptomatic TSC.