Secondary metopic craniosynostosis after posterior cranial decompression in cloverleaf skull deformity.

Cloverleaf skull deformity or Kleeblattschadel syndrome is a severe condition where multiple cranial sutures are absent and prematurely fused, leading to a trilobate head shape. The remaining open sutures or fontanelles compensate for rapid brain expansion, while the constricted fused calvarium restricts brain growth and results in increased intracranial pressure. Recent data show that early posterior cranial and foramen magnum decompression positively affects infants with cloverleaf skulls. However, long-term sequelae are still rarely discussed. We hereby report a child who developed secondary metopic craniosynostosis after posterior cranial decompression, which required a front-orbital advancement and cranial remodelling as a definitive procedure.

Re-imagining early cloverleaf skull deformity management from front to back approach-30 years on.

The cloverleaf skull deformity remains among the most complicated craniofacial conditions to successfully manage. Many cases achieve largely unsatisfactory outcomes due to the requirement for frequent reoperation on the cranial vault and failure to deal with all the elements of the craniofaciostenosis in a timely fashion. Early cranial vault surgery without addressing the cranial base deformity and its attendant cerebrospinal fluid flow changes is invariably challenging and disappointing. A recent focus on the expansion of the posterior cranial vault as a primary procedure with the greater volume change allows a delay in fronto-orbital advancement and reduced need for repeat surgery. We herein describe three cases of complex multisuture craniosynostosis with cloverleaf skull deformity who underwent neonatal posterior cranial vault decompression along with foramen magnum decompression. Our report examines the safety and rationale for this pre-emptive surgical approach to simultaneously deal with the cranial vault and craniocervical junction abnormalities and thus change the early trajectory of these complex cases.

Metopic and Sagittal Craniosynostosis in Williams Syndrome.

Craniosynostosis has been previously reported in patients with Williams syndrome. Due to the associated significant cardiovascular anomalies, with an attendant increased risk of death under anaesthesia, most patients have been managed conservatively. Here we report the multidisciplinary approach in a 12-month-old female infant with Williams syndrome who has metopic and sagittal craniosynostosis. The child successfully underwent calvarial remodelling procedures, with the clinical outcome demonstrating dramatically improved global development after surgery.

Multisuture craniosynostosis: a case report of unusual presentation of chromosome 14q32 deletion.

Multisuture craniosynostosis is associated with a number of syndromes and underlying gene mutations. It is rarely caused by chromosome disorders. For the management, multisuture craniosynostosis raises concerns about abnormal head shape and risks of increased intracranial pressure in affected patients. Calvarial reconstruction to reshape the skull shape and expand the intracranial volume plays an essential role in correcting particular problems. Here, we report a 2-month-old female infant presenting with low birth weight, abnormal head shape, dysmorphic facies and pinnae, hypotonia, and feeding difficulty. Three-dimensional computed tomographic scans revealed left unicoronal and sagittal synostoses. Chromosome microarray analysis revealed de novo chromosome 14q32.12-q32.31 deletion. Among the deleted genes, YY1 and BCL11B are the most likely candidate genes causing craniosynostosis. Some clinical features of the patient are similar to Temple syndrome indicating that the deleted region is paternal in origin. In summary, this is a rare case of chromosome 14q32 deletion with multisuture craniosynostosis. We also report the multidisciplinary management and clinical outcomes after early cranial vault remodelling procedures.

Proboscis Lateralis With Basal Encephalocele: A Report of Clinical Management and Reconstructive Approach.

Proboscis lateralis is a rare craniofacial anomaly in which a rudimentary nasal appendage arises at the medial canthal area. The severity depends on organ involvement, including eyes, nose, cleft lip/palate, and/or concomitant intracranial anomalies. Here, we present a child with proboscis lateralis and associated trans-ethmoidal encephalocele. We suggest doing the preoperative CT and/or MRI to rule out associated intracranial anomalies and reliably preoperative planning tools. Moreover, we proposed an alternative nasal reconstructive technique using a composite graft from the proboscis mass at the same time as encephalocele repair with promising results.

Dorsal Longitudinal T-Myelotomy (Bischof II Technique): A Useful, Antiquated Procedure for the Treatment of Intractable Spastic Paraplegia.

OBJECTIVE: Dorsal longitudinal T-myelotomy is a long-established operation to treat severe spastic paraplegia. The present study aimed to report this surgical technique and investigate the efficacy of T-myelotomy for spasticity relief. METHODS: All cases undergoing T-myelotomy for treatment of intractable spastic paraplegia during 2009-2017 were included. The severity of spasticity was evaluated with the Modified Ashworth Scale, Penn Spasm Frequency Scale, Adductor Tone Rating Scale, degree of passive range of motion, and occurrence of abdominal muscle spasms. Other clinical assessments included deep tendon reflex assessed by the National Institute of Neurological Disorders and Stroke scale, Babinski sign, healing of decubitus ulcers, and ambulatory status. RESULTS: Fourteen patients with a mean age of 39.3 ± 13.4 years were included. The 7 patients with abdominal muscle spasms before surgery had no spasms after surgery. The Babinski sign was absent in all cases after surgery. Unhealed pressure ulcers in all 9 cases were healed after surgery. All 4 patients with a preoperative bed-bound condition were able to ambulate with a wheelchair. A statistically significant improvement in mean Modified Ashworth Scale score, degree of passive range of motion, and National Institute of Neurological Disorders and Stroke scale score was found in the subgroup and overall analyses. There was also a statistically significant improvement in the Penn Spasm Frequency Scale and Adductor Tone Rating Scale scores. CONCLUSIONS: Dorsal longitudinal T-myelotomy remains an effective option for the treatment of intractable spastic paraplegia. It is suitable for, and may be an alternative to, intrathecal baclofen therapy for patients with complete spinal cord lesion or patients without hope of regaining motor function.

Comparison of efficacy between dorsal root entry zone lesioning and selective dorsal rhizotomy for spasticity of cerebral origin.

BACKGROUND: Severe spasticity adversely affects patient functional status and caregiving. No previous study has compared efficacy between dorsal root entry zone lesioning (DREZL) and selective dorsal rhizotomy (SDR) for reduction of spasticity. This study aimed to investigate the efficacy of DREZL and SDR for attenuating spasticity, and to compare efficacy between these two methods. METHODS: All patients who underwent DREZL, SDR, or both for treatment of intractable spasticity caused by cerebral pathology at Siriraj Hospital during 2009 to 2016 were recruited. Severity of spasticity was assessed using Modified Ashworth Scale (MAS) and Adductor Tone Rating Scale (ATRS). Ambulatory status was also evaluated. RESULTS: Fifteen patients (13 males) with a mean age of 30.3 ± 17.5 years were included. Eight, six, and one patient underwent DREZL, SDR, and combined cervical DREZL and lumbosacral SDR, respectively. Eight of ten patients with preoperative bed-bound status had postoperative improvement in ambulatory status. Spasticity was significantly reduced in the DREZL group (p < 0.001), the SDR group (p < 0.001), and in overall analysis (p < 0.001). SDR was effective in both pediatric and adult spasticity patients. A significantly greater reduction in spasticity as assessed by MAS score (p < 0.001) and ATRS score (p = 0.015) was found in the DREZL group. Transient lower limb weakness was found in a patient who underwent SDR. CONCLUSIONS: DREZL is more effective for reducing spasticity, but is more destructive than SDR. DREZL should be preferred for bed-ridden patients, and SDR for ambulatory patients. Both operations are helpful for improving ambulatory status. Gait improvement was observed only in patients who underwent SDR. Adult patients with spasticity of cerebral origin benefit from SDR.

Predictors of meningioma consistency: A study in 243 consecutive cases.

BACKGROUND: Meningioma is a common neoplasm primarily arising in the central nervous system. Its consistency is considered to be one of the critical prognostic factors for determining surgical resectability. The present study endeavored to investigate predictive factors associated with the tumor consistency. METHODS: Two hundred and forty-three consecutive participants who underwent resective surgery of meningioma were examined. The authors designed an objective grading system for meningioma consistency and utilized it for assessing consistency among all cases. We focused on the relationship between preoperative tumor characteristics on neuroimaging studies and the consistency. RESULTS: The tumor attributes on T2-weighted image (T2WI) and fluid attenuated inversion recovery (FLAIR) image were significantly correlated with the tumor consistency (p = 0.004 and 0.045, respectively). The hypointense tumors on both MRI sequences tended to be hard, whereas the tumors showing hypersignal intensity were associated with soft consistency. There was no correlation between the consistency and age, gender, duration of neurologic symptoms, tumor location, size, calcification, cystic portion, en plague appearance, tumor-brain contact interface expressed by cerebrospinal fluid (CSF) cleft, perilesional vasogenic edema, bony status, features on T1-weighted image (T1WI) and pattern of contrast enhancement. In multiple logistic regression analysis, the tumor characteristics on T2WI and FLAIR image were independent factors significantly correlated with the tumor consistency (p = 0.005 and 0.041, respectively). The tumor consistency was also correlated with operative radicalness as evaluated by the Simpson criteria. CONCLUSIONS: Signal intensity on T2WI and FLAIR image can be used for insinuating meningioma consistency. Presurgical prediction of the consistency is highly valuable in operative planning, particularly in arduous cases.