Extensive aplasia cutis congenita associated with cephalocranial disproportion and brain extrusion The original version of this article unfortunately contained an error. The author apologizes for having communicated a wrong name: “Forbid Torkamand” should be in fact “Farbod Torkamand”. Given in this article is the correct author name.

Usefulness of Standardized A-Scan technique in distinguishing papilledema from pseudopapilledema

Spontaneous resolution of syringomyelia secondary to cranio-cervical junction stenosis in a patient with achondroplasia

Acknowledgment of contribution towards two published articles

GAPO syndrome with craniosynostosis and intracranial hypertension Abstract Background GAPO (growth retardation, alopecia, pseudoanodontia, and optic atrophy) as a rare genetic disorder includes growth retardation, alopecia, pseudoanodontia, and optic atrophy. It was reported to be associated with craniosynostosis and intracranial hypertension. Case Report A patient with such a rare disorder associated with multisuture craniosynostosis and headache is presented. Surgery has been done due to intracranial hypertension. Conclusions Abnormal intraoperative findings including sever pericranium and dural adhesions and extraordinary bleeding related to this syndrome are described.

The life and works of Giulio Cesare Casseri (1552–1616) , who was the pioneer neuroanatomist

Craniocerebral disproportion after decompressive craniectomy in infants: The hidden enemy of cranial repair? Abstract Introduction Cranioplasty aims at restoring the physiological integrity and volume of the skull. Any disproportion between the intracranial content and the volume of the container may favor the occurrence of complications. A classification of volume mismatches is proposed. A negative mismatch, consisting of intracranial content minor to skull volume, is well represented by the sinking flap. On the other side, a positive mismatch, consisting of intracranial content higher than skull volume, usually depends on CSF collection or hydrocephalus once the brain edema is regressed. Though, in children, this condition may result from physiological brain growth after decompressive craniectomy. Treatment algorithm based on this classification is presented. Illustrative case A 1-year-old boy with a severe traumatic brain injury underwent right decompressive craniectomy, evacuation of subdural hematoma, and dural expansion at another institution. After failure of autologous bone-assisted cranioplasty for infection, a helmet was recommended in order to postpone the cranial repair. Patient was admitted to our institution 3 years later. CT scan showed brain herniation through the cranial defect, associated to a condition of acquired craniocerebral disproportion, due to the condition of “open skull”. Augmented hydroxyapatite cranioplasty (CustomBone, Finceramica, Faenza, Italy) was performed in order to manage this rare condition of positive volume mismatch. Subsequent course was uneventful and no complication was recorded at 30-month follow-up. Conclusions This illustrative case highlights the possible occurrence of a positive structural mismatch between the skull and the intracranial content after decompressive craniectomy, thus configuring a condition of acquired craniocerebral disproportion, aside of other brain or CSF complications. We firstly recognize this condition in the literature and propose it as a possible factor affecting the outcome of cranioplasty in infants and young children.

Unusual radiological and histological presentation of a diffuse leptomeningeal glioneuronal tumor (DLGNT) in a 13-year-old girl Abstract Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are newly recognized as an entity in the 2016 revision of the WHO Classification of tumors of the central nervous system. They typically present as diffuse leptomeningeal infiltrates along the neuraxis with focal and superficial involvement of the parenchyma. Here, we report a DLGNT with unusual radiological and histological features. A 13-year-old girl presented with scoliosis and back pain. Magnetic resonance imaging demonstrated a syrinx from C2 to T11 and an intramedullary mass from T6 to T9–10. No leptomeningeal involvement was recognized. Histological examination of the gross total resection specimen revealed a low-grade neuroepithelial neoplasm predominantly infiltrating the spinal cord and only focally involving the leptomeninges. Chromosome microarray identified co-deletion of the short arm of chromosome 1 and the long arm of chromosome 19 as well as fusion of the KIAA1549 and BRAF genes. Next-generation sequencing demonstrated wild-type alleles at the mutational hotspots of IDH1 (R132) and IDH2 (R140 and R172). In contrast to most reported DLGNTs, the tumor described in this manuscript was characterized by a predominant parenchymal component and only minor leptomeningeal involvement both radiographically and histologically. Our case, therefore, expands the spectrum of radiological and histopathological features of this new entity. It also highlights the critical role of molecular genetic testing in establishing the diagnosis of DLGNT in unusual cases.

The ventriculo-cholecystic shunt: does CSF volume matter? Abstract Introduction The management of hydrocephalus in paediatric patients where the peritoneum has failed can be challenging. One option is to perform a ventriculo-cholecystic shunt. However, little is known about the capacity of the gall bladder to accommodate cerebrospinal fluid (CSF). Methods A retrospective case series was performed to include all paediatric patients who received a ventriculo-cholecystic shunt at a single centre, Sheffield Children’s Hospital. Results We identified three patients who had a ventriculo-cholecystic shunt inserted. The shunt survived past 1 year in two patients, who had pre-operative external ventricular drain (EVD) outputs of 8 and 10 ml/h respectively. One patient shunt failed at day four post-op due to distal dysfunction, his pre-operative EVD was over 30 ml/h. Conclusions When considering a patient for a ventriculo-cholecystic shunt, caution should be taken if a high CSF output is known, for example, as per an EVD measurement.

A novel method for safe and accurate placement of the rocker pins of head immobilization devices utilizing a digital caliber phantom: technical note Abstract Background Immobilization of the head and skull by head immobilization devices (HIDs) is a common practice in neurosurgery. A variety of complications and morbidities are associated with pinning the skull during application of HIDs. Objective Our aim is to describe a new technique that avoids repeated puncturing of the head and skull during application of HIDs and hence avoiding the potential complications resulting from multiple re-adjustments. Methods We used a pre-adjusted digital millimetric caliber (DMC) as a phantom for the two rocker pins of the HID to mimic and simulate the process of skull pinning. Localization and preparation of the accurate skull pinning sites are safely guided by the pinning phantom. Results The technique was applied in different neurosurgical approaches. The pinning phantom was utilized to accurately locate the suitable pinning sites. Contrary to the common practice, there was no need for repeated head and skull puncturing. Minimal manipulations of the head and neck are exerted in this approach as compared with the usual techniques. The head of the patient is allowed to be kept safe on the operating table until the final one-time confident skull pinning by the sterilized skull clamp pins. Conclusion The process of scalp and skull pinning of HIDs is technically demanding. The DMC utilized as a pinning phantom is a useful technique which provides safe and confident application of the skull rocker pins of HIDs allowing the neurosurgeon to avoid multiple puncturing of scalp and skull and minimizing manipulations of the head and cervical spine.