Characteristics and management of mirror syndrome: a systematic review (1956-2016).

OBJECTIVES: To describe the clinical features of mirror syndrome and to correlate the effects of different treatments with the fetal outcomes. DATA SOURCES: Online search up to May 2016 was conducted in the PubMed, Embase (Ovid platform) and clinicalTrials.gov without restrictions of language, date or journal. Only papers providing both fetal and maternal presentations and outcomes were included. RESULTS: The study included 74 papers (n=111), with an additional two patients diagnosed at our center (n=113). The mean gestational age at diagnosis was 27 weeks±30 days (16-39 weeks). Whether early or late gestational age at diagnosis, and whether mother and fetus show symptoms simultaneously or on different dates, has insignificant impact on fetal outcome (P=0.06 and P=0.46, respectively). Edema (84%) followed by hypertension (60.1%) were the leading maternal findings. Fetal hydrops (94.7%) and placental edema (62.8%) were the commonest sonographic features. Procedures correcting fetal hydrops/anemia in utero as well as labor induction were the only treatment options correlated with improved fetal survival (χ2 analysis, P=0.01 and Fisher's exact test, P=0.02; respectively). The overall rate of fetal/neonatal mortality was 67.26%. CONCLUSION: The gestational age at diagnosis and sequence of presentation have insignificant impact on fetal outcome. Improved fetal survival was associated with procedural interventions that correct fetal hydrops as well as labor induction.

Rates of cerebrospinal fluid leak and pseudomeningocele formation after posterior fossa craniotomy versus craniectomy: A systematic review and meta-analysis.

Background: Postoperative cerebrospinal fluid (CSF) leak is a major concern after posterior fossa surgery with significant clinical implications. It has been postulated that replacing the bone flap, performing a craniotomy, would reinforce the surgical closure and decrease rates of CSF leak. This systematic review and meta-analysis compared the rate of CSF leak after posterior fossa craniotomies versus craniectomies. Methods: Three databases were searched for English studies comparing the primary outcome, rate of CSF leak, after adult posterior fossa craniotomies versus craniectomies. Secondary outcomes included the rate of postoperative pseudomeningocele formation, CSF leak and pseudomeningocele formation, CSF diversion, revision surgery, and infection. Pooled estimates and relative risks for dichotomous outcomes were calculated using Review Manager 5.4, with corresponding 95% confidence intervals (CIs). Results: A total of 1250 patients (635 craniotomies and 615 craniectomies), from nine studies, were included in the final analysis. Even though rates of CSF leak favored craniotomies, the difference did not reach statistical significance in our pooled analysis (Risk Ratio: 0.71, 95% Confidence Interval: 0.45-1.14, p-value = 0.15, Heterogeneity I-squared = 0%). On the other hand, comparing the rates of pseudomeningocele formation and CSF leak, as a combined outcome, or pseudomeningocele formation only showed a significant difference favoring craniotomies. The quality of evidence in this meta-analysis was graded as having a high risk of bias based on the risk of bias in non-randomized studies - of exposure criteria. Conclusion: Based on evidence with high risk of bias, rates of postoperative CSF leak and pseudomeningocele formation favored posterior fossa craniotomies over craniectomies. Further research with more robust methodology is required to validate these findings.

Case Report: Endovascular Treatment of a Giant Distal PICA Aneurysm in Association With a Cerebellar AVM: A Report on Treatment Considerations and a Literature Review.

Background: Distal posterior inferior cerebellar artery (PICA) aneurysms are uncommon intracranial vascular lesions. The coincidence of these aneurysms and Arteriovenous malformation (AVM) is even more rare. Since 1956, a total of 57 cases of distal PICA aneurysms associated with AVM have been reported with clear and adequate description. None of these reports describe a giant prenidal aneurysm at this particular location. The paucity of natural history data as well as lack of consensus about treatment strategies in such cases present a significant challenge that requires an individualized management approach. Case Description: A 68-year-old male presented with recurrent episodes of nausea and vomiting precipitated by physical exertion and change of head position. An MRI of the brain demonstrated a giant partially thrombosed right posterior inferior cerebellar artery (PICA) aneurysm with mass effect on the floor of the fourth ventricle. A conventional cerebral angiogram revealed a giant (3.1 x 3.1 x 2.8cm) distal right PICA pre-nidal aneurysm with two smaller distal PICA aneurysms. An AVM (Spetzler-Martin Grade 1) supplied by the right PICA as well as the right superior cerebellar artery (SCA) was also identified on cerebral angiography (not seen on an MRI). Endovascular coil embolization with parent vessel sacrifice was performed to occlude the giant aneurysm. Due to the asymptomatic nature, low risk of rupture, and the patient's age, AVM treatment was deferred. Conclusion: This paper presents the first case of a giant PICA aneurysm associated with cerebellar AVM. For PICA aneurysm-AVM complexes, meticulous evaluation of the morphology, associated anatomy, and comparative risk analysis for both lesions are key for treatment planning. Distal PICA aneurysms can be treated safely with parent vessel occlusion, particularly in the case of prenidal aneurysms.

Surgical Management of Isolated Fourth Ventricular Hydrocephalus Associated with Injury to the Guillain-Mollaret Triangle.

BACKGROUND: The occurrence of isolated fourth ventricle and injury to the Guillain-Mollaret triangle in the setting of posterior fossa ependymoma represents a new association. In this case report, we discuss the clinical, theoretical, and therapeutic aspects of this problem. We describe a lateral transcerebellar trajectory and shunt valve configuration for safe fourth ventricle shunting in a patient with prior posterior fossa surgery. CASE DESCRIPTION: A 45-year-old woman underwent subtotal resection of a fourth ventricle ependymoma (World Health Organization grade III) followed by radiation therapy to control the residual tumor. Her course was complicated by a cerebral abscess and subsequent communicating hydrocephalus, for which she received a lateral ventriculoperitoneal shunt. After placement of the lateral ventricle shunt, there was a progressive increase in the volume of the fourth ventricle over the next 2 years, from 2.5 to 12.0 mL. She developed palatal myoclonus, hand incoordination, bilateral foot numbness, and progressive ataxia. Neuroimaging also revealed hypertrophic degeneration of the inferior olivary nuclei bilaterally. The isolated fourth ventricle was treated by a separate fourth ventriculoperitoneal shunt inserted through a lateral transcerebellar trajectory. A programmable variable pressure valve was implemented. CONCLUSIONS: Development of an isolated fourth ventricle and injury to the Guillain-Mollaret triangle in the setting of fourth ventricular ependymoma is a newly encountered complication. Choice of treatment modality and timing of intervention should be carefully considered on a case-by-case basis. The data presented in this report may assist in the selection of surgical treatment for isolated fourth ventricle.

Pure Endovascular Management of an Arteriovenous Malformation and an Aneurysm Both Supplied by Anterio-Inferior Cerebellar Artery: A Case Report and a Review of Literature.

BACKGROUND AND IMPORTANCE: The tendency of posterior fossa arteriovenous malformations (pfAVM) to develop associated aneurysms (AA) is a well-known phenomenon with an increased total risk of rupture. Most pfAVM and AA develop in the territory of the posterior inferior cerebellar artery while the involvement of the anterior inferior cerebellar artery (AICA) is extremely rare. We describe an unusual case of an arteriovenous malformation (AVM) supplied by the AICA with a "proximal" AA. This unique combination of vascular lesions has been reported in only four cases so far, limiting the available experience that can safely guide the therapeutic intervention. CLINICAL PRESENTATION: This study describes a 59-year-old female presented with a subarachnoid hemorrhage, Hunt and Hess grade 4. Angiography demonstrated an AVM fed mainly by the right AICA and draining superficially into the transverse sinus (Spetzler-Martin grade II). In addition, there was a ruptured proximal AICA aneurysm. An endovascular approach was chosen to coil the aneurysm and obliterate the AVM using ONYX in a multi-staged process. The patient recovered well without residual deficit at 6-month follow-up. CONCLUSION: To the best of our knowledge, this is the first report describing a proximal AICA aneurysm and AVM treated by endovascular means. The outcome was very good, considering the technically demanding location. All previously reported cases with exactly similar lesions were managed surgically, with inconclusive outcomes. The data presented in this study are meant to help in decision-making process for similar cases till more data are available.

A delayed presentation of homozygous protein C deficiency in a series of children: a report on two molecular defects.

Pediatric emergency visits with purpura fulminans should raise the suspicion of hereditary homozygous protein C deficiency even beyond the neonatal age. The absence of this classical finding does not role the diagnosis out as atypical presentation with isolated intraocular bleeding was observed. Premarital counseling should be offered when family history suggests.

Peters Anomaly in Twins: A Case Report of a Rare Incident with Novel Comorbidities.

INTRODUCTION: Peters anomaly is a rare developmental malformation involving the anterior segment of the eye, which culminates in amblyopia or congenital blindness. Multiple ocular and/or systemic malformations have been observed with this anomaly, and novel comorbidities continue to be reported. CASE PRESENTATION: The probands were monozygotic twin boys (twin I and twin II) born to consanguineous parents at 36 weeks of gestation. Coarse facial features and deep-seated eyes were noted at birth. At 6 months, ophthalmic examination revealed that both twins were unable to blink in response to light, or to fixate and follow a moving object. Both twins had prominent horizontal nystagmus. Slit-lamp examination demonstrated varying degrees of central leukoma (corneal opacity) associated with iridocorneal adhesion, which is characteristic of type I Peters anomaly. No cataractous changes were observed. Normal intraocular pressure and disorganized retina were observed. Pupillary abnormalities included bilaterally underdeveloped pupils and bilateral absence of pupils was noted. Ocular MRI showed bilateral microphthalmia and optic nerve hypoplasia, with a small optic chiasm in both twins. At this age, the diagnosis of Peters anomaly was made. At 16 months of age, both twins developed deep venous thrombosis and purpuric skin lesions. Investigations revealed a hereditary thrombophilia secondary to a homozygous mutation causing protein C deficiency, which is a rare thrombotic condition. Ocular ultrasonography revealed bilateral vitreous hemorrhaging linked to altered coagulation. One twin developed bilateral inguinal hernia and cryptorchidism. CONCLUSION: The novel concordance of Peters anomaly in these monozygotic twins sharing a mutation in PROC gene provides further evidence that this anomaly has a genetic basis. Hypoplasia of the optic nerves and optic chiasm, along with severe protein C deficiency and bilateral absence of the pupils, are associated comorbidities that have not previously been reported with this anomaly.