Intracranial hypertension and empty Sella from adrenal adenoma and excessive and prolonged steroid usage: a case report

Background There is only one documented case of intracranial hypertension (IH) and empty sella from cortisol-producing adrenal adenoma so far. And IH and empty sella caused by long-term exogenous hypercortisolism has never been reported before. The purpose of this case report is to alert clinicians to glucocorticoid-induced IH. Case presentation We present retrospectively a 50-year-old woman with cortisol-secreting adrenal adenoma, who progressed to intractable intracranial hypertension and a markedly expanded empty sella due to improper treatment. In 2011, the patient presented with hypertension, lack of cortisol circadian rhythm, low ACTH, a left adrenal adenoma and a partial empty sella, but did not receive low-dose dexamethasone suppression test (LDDST) and 24-h urinary cortisol. In 2014, she exhibited truncal obesity, raised cortisol, LDDST non-suppression, high urinary free cortisol and low ACTH, proving her cortisol-producing adrenal adenoma. She was simultaneously diagnosed with unexplained IH because of papilledema and elevated intracranial pressure, and her partial empty sella changed to a complete empty sella. In 2015, she underwent adrenal adenoma resection. From 2015 to 2018, she kept taking dexamethasone at least 2 mg daily without her doctors’ consent. During this period, she developed transient cerebrospinal fluid rhinorrhea, and her empty sella further worsened. After switching to low dose hydrocortisone, her papilledema disappeared completely, but optic atrophy has become irreversible. Conclusions The patient seems to be just an extreme case, but it may reveal and illustrate a general phenomenon: Both cortisol-producing adrenal adenoma and long-term exogenous hypercortisolism could cause varying degrees of elevated intracranial pressure and empty sella. Clinicians should remain vigilant for this phenomenon in patients with cortisol-producing adrenal adenoma or excessive and prolonged steroid usage and give them corresponding examinations to identify this complication.

Esquizencefalia bilateral gigante de labio abierto

Schizencephaly is a rare congenital brain malformation characterized by clefts in the cerebral cortex, it is classified in Type I (open lip) and Type II (close-lip). Patients with schizencephaly present seizures, hydrocephalus, motor and mental deficits. Ultrasound is used for in-utero and newborns patients’ diagnosis, and MRI or CT for already born patients. The management of schizencephaly is conservative, with rehabilitation in motor or mental deficits, medication or surgery for seizures and shunt in hydrocephalus with increased intracranial pressure. In the literature, only few giant bilateral cases have been reported. We report a case of giant bilateral open lip schizencephaly, in a 10-day old male patient, presenting with mild hypotonia and no seizures. This case is rare because the relatively benign features compared to other reported cases.

Paradoxical Brain Herniation after Cranioplasty: Secondary Sunken Flap Syndrome

Decompressive craniectomy is a life-saving procedure done for innumerable etiologies. Though, not a technically demanding procedure, it has its own complications. Among many, sinking flap syndrome or syndrome of the trephined or paradoxical herniation of brain is frequently underestimated. It results from the pressure difference between the atmospheric pressure and the intracranial pressure causing the brain to shift inward at the craniectomy site. This can present with either nonspecific symptoms leading to delay in diagnosis or acute neurological deterioration, memory disturbances, weakness, confusion, lethargy, and sometimes death if not treated. Cranioplasty is a time validated procedure used to treat paradoxical brain herniation with good and early neurological recovery. We, here in, are going to describe a case report in which the paradoxical herniation occurred after cranioplasty which has not been described in literature.

Acetazolamide modulates intracranial pressure directly by its action on the cerebrospinal fluid secretion apparatus

Elevated intracranial pressure (ICP) is observed in many neurological pathologies, e.g. hydrocephalus and stroke. This condition is routinely relieved with neurosurgical approaches, since effective and targeted pharmacological tools are still lacking. The carbonic anhydrase inhibitor, acetazolamide (AZE), may be employed to treat elevated ICP. However, its effectiveness is questioned, its location of action unresolved, and its tolerability low. Here, we employed in vivo and ex vivo approaches to reveal the efficacy and mode of action of AZE in the rat brain. The drug effectively reduced the ICP, irrespective of the mode of drug administration and level of anaesthesia. The effect occurred via a direct action on the choroid plexus and an associated decrease in cerebrospinal fluid secretion, and not indirectly via the systemic action of AZE on renal and vascular processes. Upon a single administration, the reduced ICP endured for approximately 10 h post-AZE delivery with no long-term changes of brain water content or choroidal transporter expression. However, a persistent reduction of ICP was secured with repeated AZE administrations throughout the day. Future specific targeting of choroidal carbonic anhydrases may limit the systemic side effects, and therefore enhance the treatment tolerability and effectiveness in select patient groups experiencing elevated ICP.

Change in the optic nerve sheath diameter after deflation of a pneumatic tourniquet: a prospective observational study

Applying a pneumatic tourniquet provides surgeons with a bloodless surgical field. However, application of the tourniquet induces various physiological changes. We evaluated the effect of tourniquet deflation on the intracranial pressure by using ultrasonography to measure the optic nerve sheath diameter (ONSD) in patients undergoing lower limb surgery. The ONSD was measured in 20 patients at five time points: after anesthetic induction (T0) and immediately before (T1), immediately after (T2), 5 min after (T3), and 10 min after tourniquet deflation (T4). Hemodynamic and respiratory variables were recorded. The ONSD showed significant differences at each point (P < 0.001). The ONSDs at T2 and T3 were significantly greater than that at T1 (P = 0.0007 and < 0.0001, respectively). The change in the end-tidal carbon dioxide partial pressure (EtCO2) was similar to the change in the ONSD. The change in the ONSD was significantly correlated with the change in the EtCO2 after tourniquet deflation (r = 0.484, P = 0.030). In conclusion, the ONSD, as an indicator of intracranial pressure, increased after tourniquet deflation in patients undergoing lower limb surgery. This was correlated with an increased EtCO2 and arterial carbon dioxide partial pressure.Trial registration: ClinicalTrials.gov (NCT03782077).

Intraoperative Measurement of Intracranial Pressure During Cranial Vault Remodeling in Children with Craniosynostosis

Purpose In this study, we analyzed the utility of intracranial pressure (ICP) monitoring intraoperatively for deciding height reduction and need for cerebrospinal fluid (CSF) diversion during cranial vault remodeling in children with multisutural craniosynostosis (CS). Methods This is a retrospective observational study of children who underwent surgery for CS and ICP monitoring during surgery. The ICP was monitored using an external ventricular drainage catheter. The ICP monitoring was continued during the entire procedure. Results A total of 28 (19 boys) children with the involvement of two or more sutures underwent ICP monitoring during surgery. The commonest pattern of suture involvement was bicoronal seen in 16 (57.1%) children followed by pancraniosynostoses in eight (28.6%) cases. The mean opening ICP was 23 mm Hg, which dropped to 10.9 mm Hg after craniotomy. The ICP increased transiently to 19.5 mm Hg after height reduction, and the mean ICP at closure was 16.2 mm Hg. The ICP recordings helped in undoing the height reduction in two children and ventriculoperitoneal shunt after surgery in two children. Conclusions Intraoperative monitoring of ICP helps in deciding the type of cranial vault remodeling and the need for CSF diversion after surgery.

Hypertonic Saline Compared to Mannitol for the Management of Elevated Intracranial Pressure in Traumatic Brain Injury: A Meta-Analysis

Background: We performed a meta-analysis to evaluate the effect of hypertonic saline compared to mannitol for the management of elevated intracranial pressure in traumatic brain injury.Methods: A systematic literature search up to July 2021 was performed and 17 studies included 1,392 subjects with traumatic brain injury at the start of the study; 708 of them were administered hypertonic saline and 684 were given mannitol. They were reporting relationships between the effects of hypertonic saline compared to mannitol for the management of elevated intracranial pressure in traumatic brain injury. We calculated the odds ratio (OR) and mean difference (MD) with 95% confidence intervals (CIs) to assess the effect of hypertonic saline compared to mannitol for the management of elevated intracranial pressure in traumatic brain injury using the dichotomous or continuous method with a random or fixed-effect model.Results: Hypertonic saline had significantly lower treatment failure (OR, 0.38; 95% CI, 0.15–0.98, p = 0.04), lower intracranial pressure 30–60 mins after infusion termination (MD, −1.12; 95% CI, −2.11 to −0.12, p = 0.03), and higher cerebral perfusion pressure 30–60 mins after infusion termination (MD, 5.25; 95% CI, 3.59–6.91, p &lt; 0.001) compared to mannitol in subjects with traumatic brain injury.However, hypertonic saline had no significant effect on favorable outcome (OR, 1.61; 95% CI, 1.01–2.58, p = 0.05), mortality (OR, 0.59; 95% CI, 0.34–1.02, p = 0.06), intracranial pressure 90–120 mins after infusion termination (MD, −0.90; 95% CI, −3.21–1.41, p = 0.45), cerebral perfusion pressure 90–120 mins after infusion termination (MD, 4.28; 95% CI, −0.16–8.72, p = 0.06), and duration of elevated intracranial pressure per day (MD, 2.20; 95% CI, −5.44–1.05, p = 0.18) compared to mannitol in subjects with traumatic brain injury.Conclusions: Hypertonic saline had significantly lower treatment failure, lower intracranial pressure 30–60 mins after infusion termination, and higher cerebral perfusion pressure 30–60 mins after infusion termination compared to mannitol in subjects with traumatic brain injury. However, hypertonic saline had no significant effect on the favorable outcome, mortality, intracranial pressure 90–120 mins after infusion termination, cerebral perfusion pressure 90–120 mins after infusion termination, and duration of elevated intracranial pressure per day compared to mannitol in subjects with traumatic brain injury. Further studies are required to validate these findings.

Anesthetic Management in Patient with Suspected Brain Abscess, Atrium Septal Defect, and Eisenmenger Syndrome

Introduction: Patients with congenital heart disease especially with systemic shunting between systemic and pulmonary circulation often develop pulmonary hypertension and left-to-right shunt (Eisenmenger syndrome) if left untreated. These patients are at risk of developing spontaneous brain abscess due to brain infarction caused by polycythemia, impaired immune function, and loss of lung phagocytosis. Such patients were often admitted to the emergency room with signs of increased intracranial pressure (ICP), and needed specific consideration during surgery. Case: a 31-year old female diagnosed with intracranial space occupying lesion (SOL) due to suspected brain abscess with concurrent heart defects (atrial septal defect / ASD and Eisenmenger syndrome) was consulted to the operating theatre for emergency burrhole aspiration. The surgery was performed for an hour and the postoperatively the patient was admitted to the intensive care unit (ICU). Conclusion: perioperative management of patients with brain abscess and concurrent ASD and Eisenmenger syndrome consists of preoperative management, methods of anesthesia, monitoring, and interventions to prevent the worsening of left-to-right shunt and increasing intracranial pressure. These managements consist of optimal pain management, perioperative oxygen therapy, and prevention of precipitating factor that increases left-to-right shunting.

Perioperative Anesthetic Management in Preterm 12 Days Infants with Giant Meningoencephalocele at Mid Occipital and Secondary Trigonocephaly

Introduction: A meningoencephalocele is herniation of neural element along with meninges through a congenital defect in cranium. The incidence of encephalocele is approximately 1/5000 live births; occipital encephalocele is more common in females than males. It is called as giant meningoencephalocele when the head is smaller than the meningoencephalocele. These giant meningoencephaloceles harbor a large amount of cerebrospinal fluid (CSF) and brain tissue, so there occur various surgical challenges and anesthetic challenges in positioning and intubation. Case: A 12 days neonate was consulted to the neurosurgery department with complaints of large swelling over the back of head and difficulty in feeding. She was diagnosed with ventriculomegaly and meningoencephalocele since 32-33 pregnancy. The swelling was small at the time of birth, but it gradually increased in size. The child was born by section caesarean because of fetal distress and meningoenchepalocele. The neonate current weight was 3.195 grams with Post Conceptional Age (PCA) 35-36 weeks. On examination, the patient large spherical swelling was present over occipital region and there was no head control. The patient was active, conscious with no impression of focal neurological deficit. Systemic examination was unremarkable. The head circumference was 30 cm and circumference of occipital swelling was 40 cm. Potential problems in this patient include preoperative preparation and optimization of general condition, difficulty in positioning the patient, difficult airway (intubation), periodic apnea and potential hemodynamic disturbances and a sudden decrease in intracranial pressure during cele resection. Conclusion: Perioperative management in this case started from preoperative to postoperative evaluation. Preoperative preparation in anticipation of airway difficulties and communication with the operator is very important. Appropriate anesthetic techniques should aim to maintain stable hemodynamics and oxygenation and prevent a sudden increase or decrease in intracranial pressure.