A Rare Case of Acute Onset of Hydrocephalus in a Nineteen-Year-Old Patient

A Rare Case of Acute Onset of in a Abstract Hydrocephalus is a rare disorder characterized by the accumulation of cerebrospinal fluid (CSF) inside the ventricles of the brain. The disease has multiple etiologies including, but not limited to, congenital malformations, neoplasms, and infections. The case discussed in this report pertains to a 19-year-old male who presented with acute hydrocephalus. Initial workups such as physical exams, CBC, chemistry panel, CT imaging and ECG were performed. Furthermore, initial treatment with Diamox and dexamethasone was started. The patient was then moved to the neurosurgery department at a tertiary hospital where an EVD was placed. Unfortunately, the patient had a complication during the EVD placement and developed an epidural hematoma and swelling in the posterior fossa a few days afterward. Furthermore, the patient was diagnosed with a Factor VII deficiency and started on replacement therapy. Despite medical treatment, the patient’s condition continued to deteriorate, and he passed away 44 days after admission into the tertiary hospital. Due to the patient’s age group as well as the presence of a concurrent clotting disorder, ascertaining the ideal treatment proved to be difficult. Therefore, a review of the case as well as meta-analysis of related articles may shine a light on how to not only diagnose the underlying causes of hydrocephalus but also discuss strategies that can be undertaken if comorbid conditions are present.


Introduction
The goal of this case report is to examine the atypical case of acute hydrocephalus in the patient. Using prior literature on the condition's pathogenesis, we aim to examine significant clinical findings which would guide prompt diagnosis and management of this debilitating condition. In most cases, management is precarious, so it is important to discuss the different strategies used in treating patients with hydrocephalus to enhance informed decisionmaking by physicians. Ultimately, the case report contributes to the limited pool of information regarding acute hydrocephalus in the adolescent population.
Hydrocephalus is broadly defined as a build-up of a significant amount of CSF in the intracerebral ventricles of the brain. The typical patient is either an older adult or an infant presenting with variable pathologies attributable to weakened structural support of the ventricular system [1]. The worldwide prevalence and unstable balance [4]. The broad nature of the symptoms may make the diagnosis difficult to ascertain in a patient, especially one who deviates from the typical demographic. Immediate diagnosis and treatment of hydrocephalus is necessary to reduce the onset of permanent disability and improve patient outcomes [4].
Several studies have been conducted to address the surgical management of hydrocephalus. One case series study observed instances of hydrocephalus specifically involving masses in the lateral ventricle. The study consisted of 11 cases of lateral ventricular lesions with 9 of the 11 cases presenting with preoperative hydrocephalus. All the participants had an atypical age of presentation with the mean age being 25 years old ranging from 6 to 45 years of age. The most common pathological lesion, resulting in hydrocephalus, was a neurocytoma observed in 27.3% of cases. This was followed by grade 1 meningioma making up 18.2% of cases. Although total resection was performed in all cases, four patients developed severe complications such as intraventricular bleeding and ventriculitis. Only two patients did not require an external ventricular drain (EVD) postoperatively to relieve the hydrocephalus with both being admitted for a grade 1 meningioma.
Two patients developed iatrogenic hydrocephalus because of the procedure with one patient requiring a ventricular shunt to relieve the symptoms. This study demonstrates the stringent precautions that must be taken in managing hydrocephalus through resections.
Complications can readily occur, and these procedures may not necessarily provide a safeguard against future instances of hydrocephalus [5].
Congenital mutations can also result in hydrocephalus.
Although most of these defects present early, in infancy, there are instances of where hydrocephalus develops later in life. One publication looked at 25 cases of late onset hydrocephalus with the age of the patients ranging from 5 to 64 years. All the patients had a diagnosis of aqueduct stenosis. The most common age group with the condition in this study was 40-64 years followed by 5-20 years.
The study had a predilection toward males with them making up 72% of the cases. In 80% of cases, a VP shunt resulted in marked improvement with the most common complication being subdural hematoma or post-surgical infection [6].
Furthermore, some studies have been done to examine the long-term effects of hydrocephalus in adolescents and children.
Another study analyzed the effects of shunt treatment for 138 patients of ages 15 years and younger who had hydrocephalus. The study found that the mortality rate was 21.7% with no differences in rate between the first and second decade. Furthermore, 81% of patients needed their shunt revised while 10% did not. In terms of employment, 56% of all patients were either students or working while 21% were unemployed. Lastly, while the health-related quality of life scores are lower than the general population, most patients reported living lives that are "comparable" to their friends and family [7]. The long term neurological or cognitive damages caused by hydrocephalus are worth noting especially for young patients. Detrimental lifelong neurological or cognitive effects may occur and can significantly impact career and lifestyle options.
Despite the inherent risk of surgical intervention in treating hydrocephalus, comorbidities may make treatment much more complicated. A dual case study looked at two infants with hydrocephalus concurrent with hemophilia A. The key decision that proved to be successful in treating both these patients was that their factor VIII deficiency was diagnosed before any surgical intervention was made. Because of this diagnosis, both infants were started on preoperative factor VIII replacement therapy for at least a week before surgery. The first case was given an EVD later replaced by a VP shunt while the second case presented with a Dandy Walker malformation and was given a VP shunt. The patients in both cases demonstrated remarkable recovery and were on track for hitting developmental milestones in a follow up appointment. Although hemophilia A can present much more severely than a factor VII deficiency, the general principles of preoperative management remain the same for treating hydrocephalus in patients with a comorbid clotting disorder [8]. A review of the following case should demonstrate how to diagnose and important considerations that need to be taken in treating hydrocephalus concurrent with a clotting disorder.

Methods
The information pertinent to the case was relayed to the research team by the site advisor who was involved in the treatment of the patient in the Emergency Department. Information was deidentified and deemed HIPAA-compliant prior to distribution to the research team for use in the development of the case report. After receiving the information, research questions were formed which guided the literature review. The first goal was to ascertain the rarity of the disease given the patient's age. The second goal was to analyze the pathology of acute hydrocephalus and apply it to the patient's presentation in the emergency department. The third goal was to use relevant literature to determine management strategies that may be used in the patient's case.
To understand the pathology of acute hydrocephalus, a literature review was conducted using the following medical databases: National Center for Biotechnology Information (NCBI); PLOS One; and Access Medicine. The following keywords were used in the search of pertinent information: Acute Hydrocephalus; young; prognosis; diagnosis; treatment; symptoms. After discussing

Discussion
The patient was a 19-year-old male with a history of depression and drug use who presented to the emergency room with dizziness along with unfocused vision for the last 2-3 weeks.
While waiting in the hospital, the patient suffered an acute episode of syncope resulting in facial bruising and neck pain with the extent of his head injuries being unclear. The patient denied nausea, vomiting, or headache associated with his dizziness. He had also not experienced any dyspnea, paresthesia, chest pain or abdominal pain. Furthermore, he did not have a loss of limb, bowel, or bladder function. Lastly, the patient denied thoughts of self-harm, or any hallucinations. Other than the neurological symptoms the patient reported, he stated that he contracted tetanus in 2018. Additional information on the patient's past medical history, family history, and social history was not declassified for use in this study so no definitive analysis can be made regarding these factors.
On physical examination, the patient was alert and oriented. He His lungs were clear to auscultation, and the patient had no abdominal tenderness. Outside of the tenderness in the periorbital area with bruising, he was normocephalic and atraumatic. On examination, there was no spinal tenderness, but his neck did have discomfort.
As part of the workup for the patient, a CBC with differential, chemistry panel, CT of the cervical area and ECG were ordered. The CBC test revealed leukocytosis at 13 cells/uL with 80.6% neutrophil and 10.3% lymphocyte relative count. Furthermore, the patient was found to have thrombocytosis at 555x103 cells/uL. The chemistry panel also detected some abnormalities regarding the patient. Calcium and total bilirubin level were elevated at 11.1mg/ dL and 1.7mg/dL respectively. Looking further into the bilirubin count, the patient was found to have an elevated direct bilirubin    In terms of prognosis for the patient, studies show that significant deleterious effects may occur in the long run if hydrocephalus is not treated promptly [5]. Therefore, it is imperative to find the underlying etiology that led to the occurrence of hydrocephalus within the patient. The most probable cause of noncommunicating hydrocephalus within the patient's age demographic is a ventricular lesion [5]. Nevertheless, no specific tumors were identified on the CT or the MRI making the presence of an obstructive neoplasm unlikely. A potential differential diagnosis can be made for idiopathic cerebral aqueduct stenosis based on the CT imaging indicating dilation of the first three ventricles but not the fourth cerebral ventricle.
Other differentials can also be explored based on the initial imaging of the patient. For instance, the early radiologic finding of ependymal lining enhancement, extensive to the level of T9, is highly suspect of ventriculitis. However, multiple pathologies could explain this event. Although a shunt may be able to explain this finding, leakage of the CSF, as well as meningitis are other possible causes [3]. While the discovery preceded surgical intervention in this patient, ventriculitis may also occur in response to invasive surgical procedures which manage hydrocephalus [4]. Additional analysis of the CT performed on the head, and cervical spine demonstrates changes in neuroanatomy, such as dilated ventricles, resulting from high intracranial pressure. These findings can additionally point towards potential causes of the hydrocephalus.
For example, in noncommunicating hydrocephalus caused by spinal cord edema, swelling that extends to at least two vertebral segments above an injured portion is a diagnostic finding [9].
Furthermore, the absence of extra-axial fluid collection is used to rule out potential hydrocephalus ex-vacuo [10].
In the case of the patient, hydrocephalus continued following the placement of an external ventricular drain. The persistence of the hydrocephalus suggests either unmanageable severity of the extent of the hydrocephalus, or complications related to the Factor VII deficiency. The low D-dimer on the patient's CBC is probably indicative of the Factor VII deficiency which he was diagnosed with a week after admission. The most common manifestations of this condition are easy bruising, gum and nose bleeds [11]. Life threatening complications are rare but usually involve bleeds in the central nervous or gastrointestinal systems [11]. While it is unknown how severe the patient's deficiency was, it can be safe to say that invasive operations in the brain would only increase his risk of hemorrhage [12].
The decision of whether or not to place a ventricular shunt depends on the assessment by the neurological care team.13 A ventricular shunt is contraindicated if there is active infection of the CSF or the obstruction area, coagulopathy, high CSF protein, and blood in CSF [12]. The most significant complications associated with surgical treatment of hydrocephalus is hemorrhage [13].
EVD's are associated with lower rates of hemorrhage as opposed to VP shunts with the most prevalent risk factor being the use of perioperative antiplatelet medications [13]. Although it is unknown what medications the patient was given by the neurosurgical team, the patient's undiagnosed Factor VII deficiency may have resulted in an increased risk of bleeding during the procedure [12].
It is likely that the epidural hematoma was a complication of the operation. Since the only treatment of an epidural hematoma is surgery, the patient may have been at an even greater risk of further hemorrhage due to the craniotomy. This risk is evidenced by the fact that within the next week, the patient had to undergo a craniectomy due to compression of the posterior fossa due to severe dilation

Conclusion
The presence of hydrocephalus within the patient is uncharacteristic due to his age. Although the presence of a ventricular lesion is most likely, there were no obstructive masses seen on imaging. The elevated intracranial pressure is likely due to a cerebral aqueduct stenosis judging from the pattern seen on the

Conflicts of Interests
No conflicts of interest are indicated in this case report.