#Leptomeningeal cyst

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Juniper Publishers-Open Access Journal of Head Neck & Spine Surgery

A Rare Case of Traumatic Leptomeningeal Cyst in Adult: Case Report

Authored by Zheng-ming Zhu

Abstract

Roux-en-Y choledochojejunostomy is a common bile duct reconstruction operation, which has a good surgical effect, and has a low chance of bile leakage and bile duct stenosis. The main complications are bleeding, infection, anastomotic leakage and stress ulcer, but unclosed mesenteric foramen of small intestine after Roux-en-Y choledochojejunostomy is rare. Therefore, in this report, we reviewed a case of the clinical data, imaging findings, and surgical status of a patient with unclosed mesenteric foramen of small intestine of ten years after Roux-en-Y choledochojejunostomy and hope to provide a case reference for clinicians.

Keywords: Roux-en-Y choledochojejunostomy; Unclosed mesenteric foramen; Surgery; Suture

Introduction

Roux-en-Y choledochojejunostomy has different complications in clinical [1,2], and it is rare to have a proximal unclosed mesenteric hiatus. In this case, the patient had an intermittent colic episode, which was considered as the possibility of intestinal volvulus by CT examination. The small intestinal volvulus was diagnosed by postoperative exploration, and the unclosed mesenteric hiatus of the small intestine was also found. Therefore, through this case report, it is hope that can help clinical diagnosis and treatment in this area.

General clinical data and CT findings of this patient

A 51-year-old female patient, recently the lower abdomen is painful for 2 days, and was admitted to the hospital for half a day with increased pain. This patient had a history of open choledochectomy, and Roux-en-Y choledochojejunostomy for more than 10 years. Examination revealed that the patient’s abdomen was soft, tenderness in the lower abdomen, no obvious rebound pain, and active bowel sounds. T: 36. 8℃, R: 19 /min, P: 78 /min, BP: 130 /80 mmHg. Abdominal CT examination showed that multiple dilatation of the intrahepatic bile duct, a large low-density shadow and a lower-density shadow could be seen in the left lobe of the liver, the position of the gallbladder fossa not shown the gallbladder, the density of the surrounding tissue was vague, and a small amount of free gas could be seen, the proximal small intestine stenosis and the thickened peritoneal adhesions could be seen (Figure 1). The image of intestinalvolvulus and mesenteric hiatus not closed.after Roux-en-Y choledochojejunostomy. A and B: Lamellar low-density shadows and the lower liquid density shadows can be seen in the left lobe of the liver, surrounding tissues are blurred, gas density shadows can also be seen, and dilatation of the intrahepatic and external bile ducts can be seen. C: Circular stenosis can be seen in the upper segment of the small intestine the density of intestinal wall is uniform. These images suggest that it may be intestinal volvulus, but the mesenteric hiatus cannot be found.

Operation situation

The patient was anesthetized and placed on the operating table, and then routinely the abdomen was disinfected, and the sterile sheet was paved. An incision was made in the midline of the abdomen, and then the abdominal wall, rectus abdominis, and rectus sheath was opened in order, and entered the abdominal cavity. The bowel was detected, it was found that the bowel at the proximal anastomosis of the upper segment of jejunum was twisted, resulted in intestinal stenosis and intestinal wall edema, and further exploration revealed that the unclosed mesenteric hiatus near the site of intestinal stenosis (Figure 2). After the reduction of intestinal volvulus, the mesenteric hiatus was sutured and closed, and then further detected whether there are any abnormalities in other intestines. Subsequently, the abdominal incision was sutured, and the wound was covered with sterile gauze. The bleeding and anastomotic leakage were not found during the operation. The map of obstruction of upper segment of small intestine complicated with mesenteric hiatus not closed. A: The site of volvulus of the small intestine, located the below anastomosis and the upper segment of the jejunum. B: The site of intestinal volvulus has been restored and shows the site of choledochojejunostomy. C and D: The site of the proximal mesenteric hiatus of the small intestine has shown and the mesenteric foramen is closed by stitching.

Discussion

Roux-en-Y choledochojejunostomy is to cut off and close the distal end of the jejunum about 15 cm from the duodenum jejunum, retain the broken suture for traction, and jejunumjejunostomy is performed at a distance of 55 cm from the jejunum [3]. It is one of the commonly used surgical methods in general surgery []. The main indications are as follows [4-6]:  

a) Benign stricture of bile duct.

b) Some diseases requiring extrahepatic bile duct reconstruction (such as, choledochal cyst, choledochal malignant tumor or pancreatectomy, etc).  

c) Liver transplantation is not suitable for end-to-end biliary anastomosis.

d) Common bile duct stricture caused by trauma, surgery or malignancy.

e) Distal common bile duct obstruction caused by malignant tumors of pancreas, duodenum and bile duct, and incarcerated stones. Roux-en-Y choledochojejunostomy has become an important surgical method for the treatment of biliary diseases, which can significantly improve biliary obstruction and relieve symptoms [7].  

 However, there are certain contraindications, mainly those with intrahepatic stenosis or stones above the common bile duct that have not been treated [8]. The long-term complications of the Roux-en-Y choledochojejunostomy are mainly bleeding, infection, anastomosis and leakage, and stress ulcers [9,10]. The main cause of postoperative bleeding is the peeling surface bleeding and the instability of vascular ligation during operation, while the coagulation factor or fibrinogen deficiency is also part of the cause of bleeding [11]. The principle for the treatment of postoperative bleeding is through blood transfusion, transfusion and correction of blood coagulation. If the vital signs can remain stable, the patients can continue to be treated conservatively, however, if the vital signs are unstable, it is necessary to reexplore in time to stop bleeding [12]. Patients can be cured by suing antibiotics after simple infection however, retrograde biliary infections are often secondary to anastomotic stenosis and stone formation [13]. The main causes of anastomotic stenosis and biliary fistula are the high position of the resected bile duct and small anastomosis diameter. Poor technique during operation, ischemia of the bile duct wall and scar contraction can also lead to anastomotic stenosis, which can further lead to poor bile drainage and intestinal reflux, cause reflux cholangitis and aggravate symptoms. Large caliber choledochojejunostomy or the use of a silicone tube to support the drainage tube can effectively prevent the development of the postoperative anastomosis stenosis and biliary fistula [14,15]. In this report, we reviewed a patient with Roux-en-Y choledochojejunostomy, due to this patient has a long postoperative time, we only considered the possibility of small bowel torsion before surgery. Fortunately, the location of the unclosed mesenteric hiatus in the small intestine was close to the intestinal torsion, so it was found during surgery. If neglected, serious consequences were brought to the patient after surgery. Therefore, we reported the clinical symptoms, CT imaging manifestations and surgical conditions of the patients accordingly, and as a reference for clinicians.

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Pathology of the Nervous System: What makes the nervous system special?

Understanding how the nervous system reacts to injury

When attempting to understand how the nervous system of animals react to injury, we must note some important points about what makes this system special when it comes to disease.

1. The central nervous system has low resistance to infection and injury. i.e. it is easily hurt!

2. Cells of the central nervous system vary in their susceptibility to injury. NEURONS (these are your brain cells) are the most susceptible, followed by oligodendroglia (companion cells of neurons which help to form myelin to insulate neurons - important for fast transmission), astrocytes (repair cells), microglia (immune cells) and blood vessels.

3. Neurons have limited energy stores thus if blood flow to the brain were to stop, this can be lethal! 4. Neurons cannot regenerate once damaged. 

5. Nerve fibres in the central nervous system have little to no regenerative capacity. However, those of the peripheral nervous system can regenerate under certain conditions.

6. Wounds deep in the central nervous system heal by proliferation of astrocytes’ processes (i.e. they become bigger, more numerous). In contrast, wounds which are superficial (i.e. on the surface) or those that extend throughout the meninges (these are your protective coverings of your brain) heal by deposition of collagen by fibroblasts. 

Some basic terminology:

Encephalitis - Inflammation (-itis) of the brain (enceph-)

Encephalopathy - Degenerative (-pathy) disease process of the brain

Leukoencephalitis - Inflammation of the white matter (”leuko”-) of the brain

Malacia - Softening of CNS tissue, usually the result of necrosis

Myelitis - Inflammation of the spinal cord (mye-)

Myelopathy - Degenerative disease process of the spinal cord (mye-)

Neuronophagia - Accumulation of microglial cells (-phagia) around a dead neuron

Polioencephalomalacia - Softening/necrosis of the grey matter (polio-) of the brain

Polioenaphalomyelitis - Inflammation of the grey matter of the brain AND spinal cord

Poliomyelomalacia - Softening (necrosis) of the grey matter of the spinal cord

Radiculoneuritis (polyradiculoneuritis) - Inflammation of a spinal nerve rootlet 

Satellitosis - Accumulation of oligodendroglia around neuronal cell bodies

Congenital Malformations

These are abnormalities of the nervous system which animals are BORN WITH. 

Some common ones include:

1. Cerebellar hypoplasia

The term “hypoplasia” means an incomplete development or under-development of a tissue or organ. The “cerebellum” refers to a structure which is located at the back of the brain. Thus, cerebellar hypoplasia means an incomplete development/under-development of the cerebellum. The cerebellum is important for movement and co-ordination.

Cerebellar hypoplasia can be caused by certain viruses such as parvovirus and pestivirus. 

These viruses (an example being FELINE PANLEUKOPAENIA VIRUS) can infect a pregnant female, and then enter and target neuroblast cell precursors. These are responsible for the development of the cerebellum. Thus, as these precursors are infected by viruses, the development of the cerebellum is affected, resulting in cerebellar hypoplasia. 

Affected animals have trouble moving properly as a result. 

2. Hydrocephalus

This abnormality refers to the abnormal accumulation of cerebral spinal fluid (the fluid in your brain which provides it with nutrients) in the cranial cavity. 

This can either be congenital (born with - especially in brachycephalic puppies such as bulldogs), acquired (which is almost always secondary to obstruction to the flow) or caused by a viral infection (leading to stenosis i.e. “stop” of the flow through the mesencephalic duct, which means that the fluid cannot be drained away).

Thus, accumulation of this fluid would cause an enlarged brain! 

This is a serious, life-threatening condition as the excess CSF in the brain can put pressure on sensitive brain tissues, leading to brain damage.

Infectious Diseases of the Central Nervous System

These can either be bacterial, viral, or parasitic. 

Bacterial infections:

These can also be called “pyogenic” infections. The term “pyogenic” means involving the formation of pus.

1. Abscess 

Sometimes, abscesses can be formed within the central nervous system in response to a bacterial infection. An “abscess” is the immune system’s attempt to “wall off” an infection.

These can either be from a hematogenous spread (blood flow) from other sites, direct extension, trauma, or direct incidental injection (also called “iatrogenic”).

2. Neonatal bacterial suppurative meningitis

“Suppurative” as in “pus formation”, “meningitis” as in inflammation (”-itis”) of the meninges (the protective coverings of the brain). 

Grossly, you can see cloudiness and opacity of the meninges. 

3. Listeriosis

This is an interesting one. The disease is caused by the bacteria called Listeria monocytogenes. Animals are at risk of contracting the disease if they are fed contaminated silage. 

The bacteria enters the mouth of the animal and invade the trigeminal nerve, which allows it to travel up axons to the brain. It likes to localise in the brainstem. 

The bacterial infection can cause three main syndromes in ruminants like cows:

- Infection of the pregnant uterus leading to abortions and stillbirths.

- Septicaemia (i.e. blood poisoning with the bacteria)

- Meningoencephalitis (i.e. inflammation of the brain and meninges). 

Neurological signs include depression, head tilt, head pressing, and circling. This is because infected animals have trouble co-ordinating their movements.

4. Infectious thrombotic meningoencephalitis 

This disease is caused by the bacteria Histophilus somni. Interestingly enough, the bacteria forms part of the NORMAL flora of the bovine genital and upper respiratory tract!

Calves are infected by cows in the first months of life, and disseminate the infection in feedlots. 

The bacteria can attack vessels, causing vasculitis (inflammation of blood vessels) leading to thrombus formation (i.e. blood clots). The formation of these blood clots can stop blood flow to the brain. The bacteria can also cause a septicaemia which may result in acute death or may cause subacute or chronic fatal or non-fatal disease.

Clinical signs include ataxia, circling and head pressing.  

Viral infections:

1. Rabies

When a rabid animal bites another, the virus is transferred from the infected animal’s saliva into the victim’s monocytes around the wound. The virus then invades the neuromuscular junction (the space between a nerve innervating a particular muscle group - i.e. to allow muscle contraction) and ascend to the central nervous system. It then replicates there, before spreading to major “exit portals” such as adrenal glands, nasal mucosa, and salivary glands.

The virus causes non-suppurative (i.e. non-pus forming) encephalomyelitis, ganglioneuritis and parotid adenitis.

2. Canine distemper virus

Canine distemper is a type of paramyxovirus which affects multiple organ systems of dogs such as the respiratory, gastrointestinal and central nervous system, including conjunctival membranes of the eye.

The virus is able to spread to the brain from the bloodstream, affecting astrocytes and microglia. Astrocytes are “repair cells” of the central nervous system and microglia are “immune cells” of the CNS. When these are affected, they cannot carry out their functions i.e. inability to repair the nervous system nor fight off infections. The virus also affects oligodendrocytes, leading to abnormal myelin formation.

As a result, affected dogs may present with sneezing, coughing, and thick mucus coming from the eyes. They may also have fever, depression, diarrhoea and vomiting. CNS signs develop 1-3 weeks after systemic signs, or may occur after a subclinical infection. 

Dogs with adequate humoral and cell-mediated immunity may recover within 14 days. However, dogs with intermediate or poor immunity may develop the severe disease symptoms described above.

3. Feline infectious peritonitis

Feline infectious peritonitis is caused by a feline corona virus. 

There are two types:

The effusive form is characterized by serositis (inflammation of serous membranes),  fluid accumulation in the abdominal cavity and varying degrees of pyogranulomatous inflammation. 

The non-effusive form is characterised by leptomeningitis, chorioependymitis, focal encephalomyelitis and ophthalmitis. 

Parasitic infections:

1. Equine protozoal myelonencephalitis

This disease is caused by the protozoa sarcocystis neurona. 

Prevalence is low, but affected horses present with ataxia, limb weakness, lameness and seizures. 

Gross lesions are only present in severe cases. 

2. Neosporosis

This disease is caused by an apicomplexan parasite and its effects on animals depends on if the host is a cow or a dog. 

In a cow (bovine), neosporosis can cause abortions. Cattle can become infected by ingesting sporulated oocyst-contaminated food, water, or soil. However, the principal route for infection in cattle is transplacental. 

In dogs, neosporosis causes neuromuscular disease. Dogs can become infected by eating tissues contaminated with cysts. Once infected, they then shed oocysts in faeces. 

Young dogs in utero are most severely affected; resulting in muscle atrophy, dysphagia and heart failure. Clinical signs include ataxia, head tilt, hind limb paresis, rigidity and progressive paralysis.

3. Toxoplasmosis

This is a disease which affects cats. Cats can become infected by ingesting tissues contaminated with tissue cysts. Infected cats shed oocysts in their faeces. 

The encephalitic form of toxoplasmosis is most likely to occur in immunosuppressed dogs and cats.

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Biomed Grid | Primary Intracranial Germinoma: Case Report and Review of Literature

Introduction

Primary intracranial germinomas (PIGs) are rare malignant brain tumors that represent approximately 0.2% to 1.7% of all primary intracranial tumors (1). The usual anatomical locations of these tumors are pineal, parapineal and suprasellar regions. The less common sites include intrasellar region, fourth ventricle, occipital region and cauda equine.

Case Representation

Total surgical resection of cerebral germ cell tumors GCTs is limited by the deep anatomical location of these tumors. Therefore, treatment is mainly based on chemotherapy and craniospinal irradiation. Advances in diagnostic imaging, surgical and radiotherapy and the adjunction of chemotherapy have significantly improved the outcome of patients with these tumors.

Figure 1a, b, c:MRI T1-weighted postcontrast views: tumorous expansion of solid-cystic nature in the pineal region (arrows), penetrating the 3rd ventricle with compressed aqueduct of Silvius and 3-ventricular hydrocephalus.

We report the case of a 23-years-old male with an unremarkable past medical history who was admitted in the department of medical oncology at Hassan II University Hospital for headache rebellious to symptomatic treatment; associated to diplopia. Neurological examination did not reveal any neurological deficit. Cerebral Computed tomography (CT) scan showed an expansive process of the pineal area measuring 35.5*44*52.5mm with double component containing calcifications. A subsequent gadolinium-enhanced magnetic resonance imaging (MRI) revealed a bulky expansive process of the pineal area, isointense T2 and T1 compared to gray substance (Figure 1a-1c ). The lesion measured 4*4*4.6cm and caused considerable masse effect and biventricular hydrocephalus. These neuroradiological features evoked a germinoma of the pineal area. Tumor markers were assessed at the moment of diagnosis and showed a normal value of alpha-fetoprotein (AFP) and a slightly high level of beta-human chorionic gonadotropin (b-HCG) at 7.87 IU/L (n=0-5 IU/L). A lumbar puncture was performed, and the cytology of the cerebrospinal fluid confirmed the diagnosis of the germinoma (Figure 2a). Patient underwent a medullar MRI and thoracic scan that did not show metastases (Figure 2b).

Figure 2a, The tumor cells have irregular nuclei and nuclei (*100).

Figure 2b,Presence of large tumor cells mixed with small lymphocytes (*400).

The case was discussed in a multidisciplinary meeting and the decision was to start neoadjuvant chemotherapy followed by radiotherapy. After normal blood exams and normal cardiac and respiratory functions, the patient received the chemotherapy regimen BEP based on: Etoposide 100mg/m² IV (5 days), Cisplatin 20mg/m² IV (5days) and Bleomycin 30 units IV weekly on Days 1,8, and 15.

The evolution was marked by a clear clinical benefit from the first cycle of chemotherapy with complete resolution of diplopia and headaches. After the third cycle, the patient was admitted for worsening of performance status, with febrile pancytopenia without any identified infection at microbiological exams. He received antiobitherapy based on piperacillin/tazobactam with transfusion, but the evolution was unfortunately marked by septic complications with rapid worsening of vital functions leading to the death of patient.

Discussion

Germ cell tumors (GCTs) arise almost exclusively from midline axis. The intracranial location interests mainly pineal gland and the suprasellar area with pineal tumors that occur nearly twice as often as suprasellar GCTs. Intracranial GCTs can also arise in other intracranial areas such as the cerebral hemisphere, basal ganglia, thalamus, and cerebellum [1,2]. These tumors are usually pure germinomas and they are located in both pineal and supra-sellar sites in 5 to 10% of cases [33,4]. Tumor seeding or multiple tumor nodules along the lateral and third ventricles are observed in about 10% of patients. Intracranial GCT is diagnosed mainly in young patients especially during the second decade of life, with a median age at diagnosis of 10 to 12 years; and a male predominance (sex ratio between 2:1 and 3:1), especially for tumors of the pineal region [3,4].

Many molecular alterations were found, by analyzing molecular pathogenesis of intracranial GCTs such as isochromosome 12p [5,6] and gain-of-function mutations of KIT [7,8]. Next generation sequencing (NGS) was used in an international collaborative study, to analyze 62 intracranial GCTs and revealed several alterations that may therapeutically targeted. Overall, 53% of tumors harbored somatic mutations in at least one of the genes involved in the KIT/ RAS or AKT/mTOR signalling pathways [9].

Clinically, the symptoms depend on the location of the tumor. Obstructive hydrocephalus is a usual consequence of pineal tumors in addition to symptoms related to high intracranial pressure such as headache, vomiting, papilledema and somnolence in 25 to 50% of cases. Other signs might be seen including ataxia and behavioral changes. Neuro-opthalmologic abnormalities such as paralysis of upward gaze and convergence are present in up to 50% of cases [10]. Delays in diagnosis are common, especially endocrinal symptoms that may occur with delays of more than 12 months and are associated with higher rate of metastatic disease [11] but generally the endocrinopathies are rarely associated with pineal tumors at diagnosis [12].

Diagnosis of intracranial GCTs can be retained based on increased level of tumor markers including secreted into the cerebrospinal fluid (CSF) and serum, as well as by the presence of histochemical markers on tumor cells. The markers including beta-hCG and AFP are measured in the CSF and serum. Additional immunohistochemical analysis may confirm the diagnosis by using placental alkaline phosphatase (PLAP) and c-Kit on tumor cells.

Intracranial GCTs may be divided into two groups: “secreting” and “nonsecreting” tumors. Secreting tumors are commonly defined as tumors with CSF AFP >10 microg/L and/or a CSF beta-hCG level >50 int. unit/L. Secreting GCTs are more more aggressive with poorer outcomes. Pure germinomas generally are negative for AFP and beta-hCG levels in both CSF and serum and only a minority of germinomas present a high level of beta-hCG levels in the CSF and/ or serum [13,14].

Radiologically, magnetic resonance imaging (MRI) is the best imaging tool for more accurate diagnosis and for staging. Otherwise, the computed tomography (CT) also has high sensitivity to detect suprasellar and pineal GCTs. On MRI, intracranial GCTs present an iso-intense appearance or hypo-intense on T1 sequences and hyperintense on T2 sequences. These tumors typically show homogeneous enhancement with gadolinium or heterogeneous enhancement if cysts are present. Imaging characteristics of the histologic subtypes are similar, and MRIs cannot reliably distinguish germinomas from NGGCTs [15,16]. It is imperative to complete with MRI of the entire spine in order to stage adequately the intracranial GCTs, because 10 to 15% of cases may have leptomeningeal spread [3,12].

Measurement of AFP and beta-hCG in the CSF is more sensitive than serum levels in detecting abnormalities. However, discordance between serum and CSF tumor marker results has been observed, and both of them should be measured especially if no contraindication to lumbar puncture in addition to its usefulness for cytology. In case of contraindication to lumbar puncture, then tumor markers from ventricular CSF may be considered for diagnosis [14].

Histologically, the pathological proof is needed to establish a definitive diagnosis of an intracranial GCT and to ascertain the histologic subtype. A tissue sample should be obtained especially in case of normal CSF and serum AFP and beta-hCG to distinguish between pure germinoma or mature teratoma and other benign and malignant lesions, including pineal primitive neuroectodermal tumor (PNET), ependymoma (pineal location), craniopharyngioma, Langerham cell histiocytosis (suprasellar location), low-grade glioma, hamartoma, or metastatic disease from extra-cranial tumors unless surgery cannot be performed safely [17].

Patients with positive CSF cytology are considered as metastatic intracranial GCTs despite the normality of the spine MRI and must receive craniospinal irradiation (CSI). For patients with a suspected intracranial GCT, surgery for the confirmation of histologic diagnosis is strongly recommended in the absence of contraindications. Surgery is also indicated in case of neuro-surgical complication such as obstructive hydrocephalus from a pineal mass or for acute visual deterioration from a suprasellar mass [18]. When there is discordance between the tissue histological diagnosis and the CSF and/or serum markers, the treatment should be based upon the result that is associated with the most malignant histology and worst prognosis.

The gross total resection of localized germinomas is generally not recommended because of post-surgical complications and because pure germinomas are very radiosensitive [17]. Most contemporary series have reported exciting results with radiation therapy alone with long-term progression free survival (PFS) rates superior to 90% for patients with pure germinomas [19,20]. The historical schema of radiotherapy was 36 Gy craniospinal irradiation (CSI) and a boost to the primary tumor for a total of 50 to 54 Gy. Recent studies showed that whole-brain or whole-ventricle irradiation in patients with localized germinomas resulted in a spinal failure rate of less than 10 percent [17,21-23]. Therefore, whole-ventricle RT with additional boost to the tumor therefore became the new standard in the treatment of localized germinoma [24]. Several clinical researches are working on reducing both the dose and volume of radiation, without compromising the excellent survival rate to overlook the neurocognitive and endocrine commplications of radiotherapy. Germinomas are also highly chemo sensitive. Therefore, recent studies have evaluated the addition of chemotherapy to reduced dose of RT in order to minimize late complications [24,25].

Platinum-based chemotherapy regimens have showna high level of activity against extracranial GCTs in children. As in adults with advanced GCTs, the most widely used combinations are bleomycin, etoposide, and either cisplatin (BEP) or carboplatin [26]

The excellent results obtained among children and adults with extracranial GCTs, have led to the exploration of neoadjuvant chemotherapy in patients with localized intracranial germinomas, followed by a reduced dose and volume of RT, in an effort to minimize toxicity. Several series have shown excellent tumor response to chemotherapy suggesting that neoadjuvant chemotherapy allows for the reduction of both the dose and volume of RT in patients with localized germinomas without compromising PFS [26,27]. While it is clear that the addition of chemotherapy may allow reduction of radiation volume, larger studies with longer follow-up have shown that further elimination of whole-ventricle RT increases the tumor recurrence rate [24,25,28].

In a series of 60 patients with localized germinomas, neoadjuvant chemotherapy followed by 40 Gy focal RT to the tumors resulted in eight-year EFS of 83%. Eight of 10 recurrences occurred outside the RT field, in the periventricular area [25].

In the SIOP CNS GCT 96 prospective nonrandomized study, 190 patients with localized germinomas received either chemotherapy plus 40 Gy focal RT or 24 Gy CSI with a 16 Gy tumor boost without chemotherapy [28,29]. The five-year EFS for patients receiving chemotherapy and focal RT was less than for those receiving RT to a larger field without chemotherapy (88 versus 94 percent). In the patients who received chemotherapy plus focal RT, six of seven recurrences (86 percent) were ventricular, either alone or in combination with local tumor recurrence. In the patients who received CSI, all four relapses were at the original tumor site

A preliminary report of a phase II study from the Japanese CNS GCT Study Group described outcomes in 123 patients with localized germinomas, most of whom were treated with chemotherapy followed by focal RT [24]. The recurrence rate was higher in patients who received chemotherapy plus focal RT compared to those who received chemotherapy plus whole ventricle RT (28 versus 6 percent). As discussed above, the current standard of care for radiation alone (without neoadjuvant chemotherapy) in localized germinomas is 21 to 24 Gy to the whole ventricle and an additional boost to the tumor for a total dose of 40 to 45 Gy. Despite the promise of neoadjuvant chemotherapy, further reduction of the total RT dose below 40 Gy and/or elimination of whole-ventricle RT should only be done within the context of a prospective randomized trial

The current COG trial for localized germinomas is examining the efficacy of reducing the dose of whole ventricle irradiation (to 18 Gy) and local tumor boost (to 12 Gy) in patients whose tumors had complete responses to chemotherapy and compare to historical outcomes.

Although nearly all patients with localized germinomas respond to chemotherapy, treatment with chemotherapy alone has resulted in unacceptable tumor recurrence rates. In two series that included a total of 64 patients with pure germinomas, recurrent disease eventually developed in 48 and 58 percent, respectively [30,31]. The Third International CNS Germ Cell Tumor Study also confirmed that a chemotherapy-only approach led to inferior event-free survival compared to radiation-containing regimens [32].

In our case, the patient had an expansive process of the pineal area, the lesion measured 4*4*4.6cm and caused considerable masse effect and biventricular hydrocephalus.

The case was discussed in a multidisciplinary meeting and the decision was to start a neoadjuvant chemotherapy followed by radiotherapy. Our patient was received three cycles of chemotherapy and the evolution was marked by a clear clinical benefit from the first cycle of chemotherapy, but the patient died after septic complications with febrile pancytopenia.

Conclusion

Primary intracranial germinomas are rare malignant brain tumors and diagnostically very challenging. Recognition of this rare form of localization of GCT by imaging is important in addition to confirmation with tumor markers, cerebrospinal fluid cytology and stereotactic biopsy if needed. Treatment is based on multimodal therapy including mainly radiotherapy and chemotherapy. Further molecular research is required in order to better understand these tumors and develop new drugs.

Read More About this Article: https://biomedgrid.com/fulltext/volume5/primary-intracranial-germinoma-case-report-and-review-of-literature.000923.php

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Hypomelanosis of Ito with Partial Motor Seizure and Hemimegaloencephaly: Case Report

Authored by  Amal Y. Kentab*

Abstract

The term hypomelanosis of Ito [HI] is applied to individuals with skin hypopigmentation along the lines of Blaschko. Even though originally described as a purely cutaneous disease, subsequent reports have included a 33% to 94% association with multiple extracutaneous manifestations mostly of the central nervous and musculoskeletal systems leading to frequent characterization as a neurocutaneous disorder. A boy with constellation of multiple congenital anomalies including facial dysmorphism, skin hypopigmentation, musculoskeletal, and nervous system abnormalities in the form of hypotonia and mild mental retardation suggestive of [HI] presented with partial motor seizures and found to have hemimegalencephaly [HME] on MRI of the brain.

Keywords: Hypomelanosis ito (HI); Cortical dysplasia; Neurocutaneous syndrome; Cerebral malformation.

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Introduction

Hypomelanosis of I to [HI] or Incontinentia pigmenti achromians [OMIM no. 146150]. Ito first introduced the syndrome 1951 [1]. It is a rare neurocutaneous syndrome that involves mainly skin and nervous system symptoms in 75 % of cases and may be associated with multiple organ systems involvement including the head and face, eyes (microphthalmia, cataracts, optic atrophy, and retinal detachment), teeth, cleft lip and palate, heart (tetralogy of Fallot), kidneys, musculoskeletal (hemihypertrophy), and reproductive systems (precocious puberty) [2-4]. The incidence and prevalence of [HI] has been reported to be1/7540 births and 1/82,000 individuals, respectively [4]. McKusick`s catalogue of inherited diseases lists HI as an autosomal dominant disorder, although evidence for this mode of inheritance, or indeed for any genetic etiology, is inconclusive [2]. Frequency is equal among males and females. It is caused by Nonheritable mutation, and it have been reported frequently in association of various chromosomal abnormalities, including mosaicism for aneuploidy or unblanaced translocations, mosaic trisomy 18, ring chromosome 22, and translocations involving the X chromosome. There is no clear genotype - phenotype correlation [2]. Only a few cases of HI have been reported with partial motor seizures in association with hemimegalencephaly [HME]. In order to highlight on this rare neurocutaneous disorder, this report describes such association in a 10 year old boy with skin hypopigmentation, craniofacial and musculosketetal abnormalities.

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Case Report

A 10 -year-old male, left handed, 6th grade student was evaluated in our tertiary care neurology clinic for afebrile complex partial motor seizure with 2nd generalization, started one month prior to presentation. He had frequent episodes of sudden left-sided eye deviation and facial twitching with secondary left-sided unilateral and generalized tonic-clonic seizures. Each episodes last for less than 3 minutes, preceded by dizziness, pain in the right eye with blurred vision and headache, end spontaneously after an episode of vomiting , headache , and postictal sleep without loss of sphincter control . Seizures were controlled with levetiracetam (Keppra). He was a product uneventful pregnancy delivered by spontaneous vaginal delivery at 36 weeks gestational age, readmitted on 2nd day, and operated for intestinal intussusception. Past history included a slower cognitive and language development in comparison to other siblings, skin hypopigmentation noted at 6 months of age, bronchial asthma, abnormal dentation with frequent dental caries, and tonsillectoy-adenectomy operation for frequent tonsillitis.

His parents were consanguineous and there was a history of seizure disorder in two sons of paternal uncle from twin pregnancy. Microophthalmia in two sisters of paternal ante. Reported multiple infantile deaths of both parental, and maternal side with obscured reasons. His mother had cafe`-au lit spot and hypopigmentation on her thighs. On examination, he had macrocephaly, short stature, dysmorphic features in the form of high prominent forehead, low set and posteriorly rotated prominent ears, orbital hypertelorism, epicanthal fold, depressed nasal bridge, anteverted nostrils, high arched palate, tooth spacing with abnormality in size and shape, micrognathia, broad fingers with archanodactyly, mild clinodatyly of 5th finger, broad nails, a gap between big toe and the second one, syndactly between the 2nd and the 3rd toes on the right foot, mild scoliosis (deviation to the left) with prominent lordosis, joint hyperlaxity and bilateral pes plans , with mild hemi hypertrophy of the right side of the body , involving mainly the face and upper extremity.

Hypopigmented areas (whorl-like and streaks) noted on the anterior and the lateral aspects of both legs extending to involve the left shine anteriorly and another similar one but with small size seen at the right shoulder (Figure 1). His neurological examination revealed poor short-term memory and cognitive delay, especially in terms of general knowledge, the ability to read and mathematical concepts and mild right hemiparesis. His initial basic laboratory work-up was normal apart from mild anemia, and low vitamin D 62 nmol/l (NR 75-120). Full metabolic screen thyroid function test, full chromosomal analysis, microarray CGH showed and Cardiac evaluation by electrocardiogram (ECG), echocardiogram (Echo) and Abdominal ultrasound were unrevealing. Ophthalmological evaluation showed mild sclerocornea, pigmented iris, and bilateral flat retinal astrocytoma with retinal pigmentary changes. Skeletal survey revealed mild osteoporosis and mildscoliosis.

Electroencephalogram (EEG) showed background asymmetry with slow theta wave activity noted on the left hemisphere with occasional sharp waves seen predominately on the left hemisphere. Computed tomographic (CT) scan of the brain showed no space occupying lesion, but left hemimegaloencephaly with dilated left ventricle. Magnetic resonance imaging scan of the brain (MRI) revealed similar findings with small arachnoid cyst at left middle fossa with no evidence of neuronal migration disorders or cortical dysplasia (Figure 2). Magnetic resonance angiography (MRA) was normal with no evidence of vessel stenosis or picture similar to moyamoya disease.

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Discussion

The cutaneous features of HI include multiple hypopigmented streaks or patches that found in 100% of affected individuals and are either present at birth or emerge in infancy. These lesions follow Blaschko lines (skin lines that form specific patterns over the trunk and extremities, such as a V shape over the back and linear lines over the limbs and are best detected under ultraviolet light in light - skinned children. In contrary to incontinentiapigmenti, these lesions, are not preceded by inflammatory or degenerative changes. (Table 1) The extent of the skin lesions do not correlate strongly with neurologic involvement. An important differential diagnosis is Tuberus Sclerosis Complex, where the hypopigmented patch (ash leave spot which is usually oval in shape with regular border) appears during the 1st year of life mainly on trunk or extremities, and as the child approaching puberty other skin manifestations appear like adenoma sebaceum, on the face, shagreen patch on the back and periungual fibroma [3,4].

(Table 1) Neurologic manifestations include intellectual impairment with mild to severe mental deficiency (IQ<70) that is usually seen in 57% while only 20% of patients have an IQ above 85% with reported poor school performance in (40-60%). Seizures that include generalized tonic or tonic-clonic, complex partial, myoclonic seizures or infantile spasm with onset early in the first year of life, with variable response to anticonvulsant drugs . Behavior disorders that include autism, Asperger`s syndrome, self-injurious behavior and severe sleep problems especially in the first 3-5 years of age [3,4]. Macrocephaly or microcephaly may be seen, with the former being more common. Other associated findings include, a non-progressive speech delay, muscular hypotonia or hypertonia, hyperkinesias, nystagmus, ataxia, and neurosensory deafness. MRI in more than half of patients may show either cerebral or cerebellar hypoplasia and various other malformations of cortical development including hemimegalencephaly, lissencephaly, pachygyria, and heterotopia. White matter abnormalities of either cystic like lesions or delayed myelination. Other brain abnormalities include, focal or generalized brain atrophy, basal ganglia lesion, or intracranial vascular abnormalities such as A-V malformation, Moyamoya disease, leptomeningeal angioma [2]. The patient in this report had clinical picture suggestive of HI (skin hypopigmentation, facial dysmorphism, eye and dental abnormalities, joint hypermobility, skeletal abnormalities ie. short stature, scoliosis, osteoporosis, mild learning disability, partial motor seizures), mild right hemiparesis and HME with no other associated neuronal migration disorder on brain MRI which has been previously reported [5-9].

Hemimegalencephaly is a major, but rare congenital hamartomatous malformation of the brain [10], characterized by enlargement of all or parts of a cerebral hemisphere and frequently associated with cortical focal or diffuse neuronal migration defects, such as polymicrogyria, pachygyria, or heterotopias. HEM affects all ethnic groups and both genders equally.

Its etiology remains unknown. It has been suggested that it results from some type of acquired unilateral hemispheric insult in the mid to late second trimester of pregnancy [11] that affect the genetically programmed process that establishes symmetry as well as the development of neuroepithelial lineage and cellular growth occurring at an earlier stage of neuroblast migration [12]. It does not follow a Mendelian pattern of inheritance and usually occurs sporadically. It is widely believed that a single or multiple gene mutations contribute to this process.

It typically presents with a triad of intractable epilepsy, psychomotor delay and hemiparesis. Some patients show entire brain asymmetry, hemifacial hypertrophy, or hemicorporal hypertrophy. Other associated features may include macrocephaly with cranial asymmetry, and behavioral disabilities. Epilepsy is usually of early onset and intractable. Those with later-onset epilepsy are more likely to have mild motor deficit like the patient in this report, or normal motor function. The EEG is abnormal in all cases of HME. In the neonatal period, suppression burst pattern, followed by hypsarrhythmia and later, focal seizure activity may be seen. Functional or anatomical hemispherectomyis recommended for early - onset pharmacoresistant epilepsy as improvement of either the motor function level or intellectual development was seen in most patients post-surgery [13,14].

It is often an isolated disorder, but may be syndromic associated with several diseases or neurocutaneous syndromes, such as epidermal nevus syndrome, tuberous sclerosis complex (TSC), linear sebaceous nevus syndrome, Klippel - Trenaunay syndrome (KTS), Proteus syndrome (PS), hypomelanosis of ito (HI), neurofibromatosis , Sotos syndrome and Alexander [10,15]. It is worthy to mentioned that usually there is no differences in clinical symptoms in regards to the laterality (Right or left) or the type (isolated or syndromic) of hemimegalencephaly [16].

MR Imaging is the investigation of choice for diagnosis, though ultrasound and CT will show the abnormalities. As in our patient, increased size and altered shape of the ventricle is usual. The cortex is broader (dysplastic) than normal, neuroblast migratory anomalies are frequently seen, the most common being heterotopias. [11], as well as white-matter abnormalities (high signal intensity in T2W image) [17]. Since the disease does not follow a Mendelian pattern of inheritance, chances of recurrence are rare and there is usually no family history of other affected individuals. Prenatal diagnosis may be suspected on the basis of fetal ultrasound [18] or MRI (diffusion - weighted images) [19].

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Conclusion

Combination of a triad of intractable epilepsy, psychomotor delay and hemiparesis in the presence of macrocephaly and / or skin hypopigmentation should prompt consideration of HEM . MRI is the imaging of choice for diagnosis of HEM. HEM should prompt a search for other syndromic diagnosis such as HI.

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Acknowledgment

This work was supported by the College of Medicine Research Center, Deanship of Scientific Research, King Saud University.

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[mri-radiology-blog]

#intradiploic postraumatic #arachnoid #cyst 👇🇺🇸 -------- Внутрикостная/диплоическая арахноидальная киста характеризуется накоплением ЦСЖ в мешке, покрытие которого выстилается арахноидной мембраной, расположенной в диплоическом пространстве. Чрезвычайно редкое явление, как правило после травмы у детей. Врезультате которой происходит перелом внутренней пластинки. Иногда используют различную номенклатуру для описания этого поражения в литературе, которое включает внутридиплоическую фистулу ЦСЖ, #внутрикостную #лептоменингиальную кисту, посттравматическую #арахноидальную кисту. ----- 🇺🇸Intradiploic arachnoid cysts are characterized by accumulation of CSF in a sac, whose covering is lined by arachnoid membrane situated within the diplopic space, and have exceedingly rare occurrence. Different nomenclature used to describe this lesion in the literature includes intradiploic CSF fistula, #intraosseous #leptomeningeal cyst, traumatic and posttraumatic arachnoid cyst,intradiploic arachnoid cyst (IAC). By Satish Kumar Verma, Guru Dutta Satyarthee et al. Department of Neurosurgery, All India Institute of Medical Sciences. ------ #radiopaedia

[itsmedicinesfakianakis]

Glymphatic MRI in idiopathic normal pressure hydrocephalus

Abstract

The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer’s disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In idiopathic normal pressure hydrocephalus, we found delayed enhancement (P < 0.05) and decreased clearance of gadobutrol (P < 0.05) at the Sylvian fissure. Parenchymal (glymphatic) enhancement peaked overnight in both study groups, possibly indicating a crucial role of sleep, and was larger in normal pressure hydrocephalus patients (P < 0.05 at inferior frontal gyrus). We interpret decreased gadobutrol clearance from the subarachnoid space, along with persisting enhancement in brain parenchyma, as signs of reduced glymphatic clearance in idiopathic normal hydrocephalus, and hypothesize that reduced glymphatic function is instrumental for dementia in this disease. The study shows promise for glymphatic magnetic resonance imaging as a method to assess human brain metabolic function and renders a potential for contrast enhanced brain extravascular space imaging. from # All Medicine by Alexandros G. Sfakianakis via alkiviadis.1961 on Inoreader http://ift.tt/2vaupPN from OtoRhinoLaryngology - Alexandros G. Sfakianakis via Alexandros G.Sfakianakis on Inoreader http://ift.tt/2vMEJBa

[ourhaileydavies]

Juniper Publishers-Open Access Journal of Head Neck & Spine Surgery

A Rare Case of Traumatic Leptomeningeal Cyst in Adult: Case Report

Authored by Aneesh KM

Abstract

Traumatic leptomeningeal cysts are a rare complication of a childhood skull fracture. Clinical manifestations of a childhood trauma are very rare in adults and usually presents as a nontender subcutaneous mass with progressive neurological deficit and seizures.

Keywords: Leptomeningeal cyst; Adult; Trauma; Seizures; Skull fracture

Abbrevations: CT: Computed Tomography; MRI: Magnetic Resonance Imaging; CSF: Cerebrospinal Fluid; T1WI: T1-Weighted Images; T2WI: T2-Weighted Images

Case Report

A 28-year-old male presenting with a gradually increasing scalp swelling in the left parietal region over a long period and seizures. The patient was conscious. On physical examination, there was a cystic swelling over the left parietal prominence. The swelling was compressible but non-tender and non-pulsatile. There was a history of head injury during infancy (Figure 1-5).

A non-contrast enhanced head computed tomography (CT) examination was performed on a multidetector CT (Lightspeed ultra, GE Medical Systems) and demonstrated a large calvarial defect in the left parietal region with irregular and beveled margins. An adjacent CSF density cystic lesion of size 42x41mm noted in the left high parietal lobe. The cyst was seen communicating with the subarachnoid space and also seen extending though the calvarial defect. Mild widening of sulcal spaces and hypodense areas also noted in the surrounding brain parenchyma due to encephalomalacia. The cystic lesion was seen closely abutting the left lateral ventricle with focal dilatation of the ventricle. But there was no communication of the cyst with the ventricle. Corrective surgery was done. The intraoperative and postoperative period was uneventful.

Discussion

Growing skull fractures usually occur due to severe head trauma during the first three years of life, particularly in infancy. Incidence reported is only.05 to.1% of skull fracture in childhood [1,2]. Cause for growing skull fractures is multifactorial but the main factor is tear in the dura mater. The pulsatile force of CSF and pressure of growing brain will cause cerebral or subarachnoid herniation through the dural tear which causes the fracture in the thin skull to enlarge. This interposition of tissue prevents osteoblasts from migrating, inhibiting fracture healing. The resorption of the adjacent bone by the continuous pressure from tissue herniation through the bone gap adds to the progression of the fracture line (Tables 1-2).

Table abbreviations: CT = Computed Tomography, MRI = Magnetic Resonance Imaging, CSF = Cerebrospinal fluid, T1WI= T1-weighted images, T2WI= T2-weighted images.

Table abbreviations: CT = Computed Tomography, MRI = Magnetic Resonance Imaging, CSF = Cerebrospinal fluid, T1WI= T1-weighted images, T2WI= T2-weighted images.

The brain extrusion may be present shortly after diastatic linear fracture in neonates and young infants [3] resulting in focal dilatation of the lateral ventricle near the growing fracture. This focal dilatation may be seen in adults which is also seen in this case. This focal dilatation is reversible and may normalize after surgical repair [4]. Cranial defects never increase if the underlying dura is intact. Leptomeningeal cyst never occurs if the dura is intact.

Another risk factor is severity of underlying trauma. A linear fracture associated with hemorrhagic contusion of subjacent brain suggests a trauma significant enough to cause dural laceration. Cystic changes at the growing fracture site may be because of cystic encephalomalacia. Post traumatic aneurysms and subdural hematomas have also been reported to accompany growing skull fractures [6,7]. Though most patients show damage to underlying brain, this finding is not a prerequisite for the development of growing skull fractures [8].

These skull fractures after reaching maximum extent will cease to grow and remain stable throughout adulthood [2,5].

A depressed fracture usually does not become a growing fracture [9] but a linear fracture extending from a depressed one can become one [10].

A fracture with a diastasis of >4mm may be considered at risk of developing a growing skull fracture [3,11,12]. But a post traumatic diastasis of a cranial suture is an unusual site for a growing fracture. Growing fractures can even be seen in usually in linear fractures in thin areas of skull base associated with dural laceration, for e.g.: Orbital roof, ethmoid plate, frontal sinus.

These fractures commonly present as a progressive, scalp mass that appears sometime after head trauma sustained during infancy. There may seizures and hemiparesis, but an asymptomatic palpable mass may be the sole sign. The usual site is the parietal region. A growing fracture at the skull base may present with ocular proptosis or CSF rhinorrhea or otorrhea.

A plain radiograph may show a fracture line that crosses a coronal or lambdoid suture, but it is usually limited to a parietal bone [13]. CT or MRI demonstrates a cystic lesion near the fracture site communicating with the subarachnoid spaces and extending though the bony defect. Margins of bony defect may be beveled or irregular. Adjacent brain parenchyma usually shows mild encephalomalacia changes and focal atrophy. Gliosis may also see in the adjacent brain parenchyma. On CT scan gliosis is seen as hypodense areas. On MRI gliosis is seen as hypointense T1 and hyperintense T2 signals.

Because of neurological deterioration and of seizure disorder surgical correction of growing fractures is recommended.  

 Even though traumatic leptomeningeal cyst is rare in adults, it should be considered in the differential diagnosis of intracranial cystic lesions with adjacent calvarial defects.

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[mri-radiology-blog]

#intradiploic postraumatic #arachnoid #cyst 👇🇺🇸 -------- Внутрикостная/диплоическая арахноидальная киста характеризуется накоплением ЦСЖ в мешке, покрытие которого выстилается арахноидной мембраной, расположенной в диплоическом пространстве. Чрезвычайно редкое явление, как правило после травмы у детей. Врезультате которой происходит перелом внутренней пластинки. Иногда используют различную номенклатуру для описания этого поражения в литературе, которое включает внутридиплоическую фистулу ЦСЖ, #внутрикостную #лептоменингиальную кисту, посттравматическую #арахноидальную кисту. ----- 🇺🇸Intradiploic arachnoid cysts are characterized by accumulation of CSF in a sac, whose covering is lined by arachnoid membrane situated within the diplopic space, and have exceedingly rare occurrence. Different nomenclature used to describe this lesion in the literature includes intradiploic CSF fistula, #intraosseous #leptomeningeal cyst, traumatic and posttraumatic arachnoid cyst,intradiploic arachnoid cyst (IAC). By Satish Kumar Verma, Guru Dutta Satyarthee et al. Department of Neurosurgery, All India Institute of Medical Sciences. ------ #radiopaedia

[ourhaileydavies]

A Rare Case of Traumatic Leptomeningeal Cyst in Adult: Case Report-Juniper publishers

Abstract

Roux-en-Y choledochojejunostomy is a common bile duct reconstruction operation, which has a good surgical effect, and has a low chance of bile leakage and bile duct stenosis. The main complications are bleeding, infection, anastomotic leakage and stress ulcer, but unclosed mesenteric foramen of small intestine after Roux-en-Y choledochojejunostomy is rare. Therefore, in this report, we reviewed a case of the clinical data, imaging findings, and surgical status of a patient with unclosed mesenteric foramen of small intestine of ten years after Roux-en-Y choledochojejunostomy and hope to provide a case reference for clinicians.

Keywords: Roux-en-Y choledochojejunostomy Unclosed mesenteric foramen Surgery suture

Introduction

Roux-en-Y choledochojejunostomy has different complications in clinical [1,2], and it is rare to have a proximal unclosed mesenteric hiatus. In this case, the patient had an intermittent colic episode, which was considered as the possibility of intestinal volvulus by CT examination. The small intestinal volvulus was diagnosed by postoperative exploration, and the unclosed mesenteric hiatus of the small intestine was also found. Therefore, through this case report, it is hope that can help clinical diagnosis and treatment in this area.

General clinical data and CT findings of this patient

A 51-year-old female patient, recently the lower abdomen is painful for 2 days, and was admitted to the hospital for half a day with increased pain. This patient had a history of open choledochectomy, and Roux-en-Y choledochojejunostomy for more than 10 years. Examination revealed that the patient’s abdomen was soft, tenderness in the lower abdomen, no obvious rebound pain, and active bowel sounds. T: 36. 8℃, R: 19 /min, P: 78 /min, BP: 130 /80 mmHg. Abdominal CT examination showed that multiple dilatation of the intrahepatic bile duct, a large low-density shadow and a lower-density shadow could be seen in the left lobe of the liver, the position of the gallbladder fossa not shown the gallbladder, the density of the surrounding tissue was vague, and a small amount of free gas could be seen, the proximal small intestine stenosis and the thickened peritoneal adhesions could be seen (Figure 1). The image of intestinalvolvulus and mesenteric hiatus not closed.after Roux-en-Y choledochojejunostomy. A and B: Lamellar low-density shadows and the lower liquid density shadows can be seen in the left lobe of the liver, surrounding tissues are blurred, gas density shadows can also be seen, and dilatation of the intrahepatic and external bile ducts can be seen. C: Circular stenosis can be seen in the upper segment of the small intestine the density of intestinal wall is uniform. These images suggest that it may be intestinal volvulus, but the mesenteric hiatus cannot be found.

Operation situation

The patient was anesthetized and placed on the operating table, and then routinely the abdomen was disinfected, and the sterile sheet was paved. An incision was made in the midline of the abdomen, and then the abdominal wall, rectus abdominis, and rectus sheath was opened in order, and entered the abdominal cavity. The bowel was detected, it was found that the bowel at the proximal anastomosis of the upper segment of jejunum was twisted, resulted in intestinal stenosis and intestinal wall edema, and further exploration revealed that the unclosed mesenteric hiatus near the site of intestinal stenosis (Figure 2). After the reduction of intestinal volvulus, the mesenteric hiatus was sutured and closed, and then further detected whether there are any abnormalities in other intestines. Subsequently, the abdominal incision was sutured, and the wound was covered with sterile gauze. The bleeding and anastomotic leakage were not found during the operation. The map of obstruction of upper segment of small intestine complicated with mesenteric hiatus not closed. A: The site of volvulus of the small intestine, located the below anastomosis and the upper segment of the jejunum. B: The site of intestinal volvulus has been restored and shows the site of choledochojejunostomy. C and D: The site of the proximal mesenteric hiatus of the small intestine has shown and the mesenteric foramen is closed by stitching.

Discussion

Roux-en-Y choledochojejunostomy is to cut off and close the distal end of the jejunum about 15 cm from the duodenum jejunum, retain the broken suture for traction, and jejunumjejunostomy is performed at a distance of 55 cm from the jejunum [3]. It is one of the commonly used surgical methods in general surgery []. The main indications are as follows [4-6]:

a) Benign stricture of bile duct.

b) Some diseases requiring extrahepatic bile duct reconstruction (such as, choledochal cyst, choledochal malignant tumor or pancreatectomy, etc).

c) Liver transplantation is not suitable for end-to-end biliary anastomosis.

d) Common bile duct stricture caused by trauma, surgery or malignancy.

e) Distal common bile duct obstruction caused by malignant tumors of pancreas, duodenum and bile duct, and incarcerated stones. Roux-en-Y choledochojejunostomy has become an important surgical method for the treatment of biliary diseases, which can significantly improve biliary obstruction and relieve symptoms [7].

However, there are certain contraindications, mainly those with intrahepatic stenosis or stones above the common bile duct that have not been treated [8]. The long-term complications of the Roux-en-Y choledochojejunostomy are mainly bleeding, infection, anastomosis and leakage, and stress ulcers [9,10]. The main cause of postoperative bleeding is the peeling surface bleeding and the instability of vascular ligation during operation, while the coagulation factor or fibrinogen deficiency is also part of the cause of bleeding [11]. The principle for the treatment of postoperative bleeding is through blood transfusion, transfusion and correction of blood coagulation. If the vital signs can remain stable, the patients can continue to be treated conservatively, however, if the vital signs are unstable, it is necessary to reexplore in time to stop bleeding [12]. Patients can be cured by suing antibiotics after simple infection however, retrograde biliary infections are often secondary to anastomotic stenosis and stone formation [13]. The main causes of anastomotic stenosis and biliary fistula are the high position of the resected bile duct and small anastomosis diameter. Poor technique during operation, ischemia of the bile duct wall and scar contraction can also lead to anastomotic stenosis, which can further lead to poor bile drainage and intestinal reflux, cause reflux cholangitis and aggravate symptoms. Large caliber choledochojejunostomy or the use of a silicone tube to support the drainage tube can effectively prevent the development of the postoperative anastomosis stenosis and biliary fistula [14,15]. In this report, we reviewed a patient with Roux-en-Y choledochojejunostomy, due to this patient has a long postoperative time, we only considered the possibility of small bowel torsion before surgery. Fortunately, the location of the unclosed mesenteric hiatus in the small intestine was close to the intestinal torsion, so it was found during surgery. If neglected, serious consequences were brought to the patient after surgery. Therefore, we reported the clinical symptoms, CT imaging manifestations and surgical conditions of the patients accordingly, and as a reference for clinicians.

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[ourhaileydavies]

A Rare Case of Traumatic Leptomeningeal Cyst in Adult: Case Report-Juniper publishers

Abstract

Traumatic leptomeningeal cysts are a rare complication of a childhood skull fracture. Clinical manifestations of a childhood trauma are very rare in adults and usually presents as a nontender subcutaneous mass with progressive neurological deficit and seizures.

Keywords: Leptomeningeal cyst; Adult; Trauma; Seizures; Skull fracture

Abbrevations: CT: Computed Tomography; MRI: Magnetic Resonance Imaging; CSF: Cerebrospinal Fluid; T1WI: T1-Weighted Images; T2WI: T2-Weighted Images

Case Report

A 28-year-old male presenting with a gradually increasing scalp swelling in the left parietal region over a long period and seizures. The patient was conscious. On physical examination, there was a cystic swelling over the left parietal prominence. The swelling was compressible but non-tender and non-pulsatile. There was a history of head injury during infancy (Figure 1-5).

A non-contrast enhanced head computed tomography (CT) examination was performed on a multidetector CT (Lightspeed ultra, GE Medical Systems) and demonstrated a large calvarial defect in the left parietal region with irregular and beveled margins. An adjacent CSF density cystic lesion of size 42x41mm noted in the left high parietal lobe. The cyst was seen communicating with the subarachnoid space and also seen extending though the calvarial defect. Mild widening of sulcal spaces and hypodense areas also noted in the surrounding brain parenchyma due to encephalomalacia. The cystic lesion was seen closely abutting the left lateral ventricle with focal dilatation of the ventricle. But there was no communication of the cyst with the ventricle. Corrective surgery was done. The intraoperative and postoperative period was uneventful.

Discussion

Growing skull fractures usually occur due to severe head trauma during the first three years of life, particularly in infancy. Incidence reported is only.05 to.1% of skull fracture in childhood [1,2]. Cause for growing skull fractures is multifactorial but the main factor is tear in the dura mater. The pulsatile force of CSF and pressure of growing brain will cause cerebral or subarachnoid herniation through the dural tear which causes the fracture in the thin skull to enlarge. This interposition of tissue prevents osteoblasts from migrating, inhibiting fracture healing. The resorption of the adjacent bone by the continuous pressure from tissue herniation through the bone gap adds to the progression of the fracture line (Tables 1-2). Table abbreviations: CT = Computed Tomography, MRI = Magnetic Resonance Imaging, CSF = Cerebrospinal fluid, T1WI= T1-weighted images, T2WI= T2-weighted images. Table abbreviations: CT = Computed Tomography, MRI = Magnetic Resonance Imaging, CSF = Cerebrospinal fluid, T1WI= T1-weighted images, T2WI= T2-weighted images.

The brain extrusion may be present shortly after diastatic linear fracture in neonates and young infants [3] resulting in focal dilatation of the lateral ventricle near the growing fracture. This focal dilatation may be seen in adults which is also seen in this case. This focal dilatation is reversible and may normalize after surgical repair [4]. Cranial defects never increase if the underlying dura is intact. Leptomeningeal cyst never occurs if the dura is intact.

Another risk factor is severity of underlying trauma. A linear fracture associated with hemorrhagic contusion of subjacent brain suggests a trauma significant enough to cause dural laceration. Cystic changes at the growing fracture site may be because of cystic encephalomalacia. Post traumatic aneurysms and subdural hematomas have also been reported to accompany growing skull fractures [6,7]. Though most patients show damage to underlying brain, this finding is not a prerequisite for the development of growing skull fractures [8].

These skull fractures after reaching maximum extent will cease to grow and remain stable throughout adulthood [2,5].

A depressed fracture usually does not become a growing fracture [9] but a linear fracture extending from a depressed one can become one [10].

A fracture with a diastasis of >4mm may be considered at risk of developing a growing skull fracture [3,11,12]. But a post traumatic diastasis of a cranial suture is an unusual site for a growing fracture. Growing fractures can even be seen in usually in linear fractures in thin areas of skull base associated with dural laceration, for e.g.: Orbital roof, ethmoid plate, frontal sinus.

These fractures commonly present as a progressive, scalp mass that appears sometime after head trauma sustained during infancy. There may seizures and hemiparesis, but an asymptomatic palpable mass may be the sole sign. The usual site is the parietal region. A growing fracture at the skull base may present with ocular proptosis or CSF rhinorrhea or otorrhea.

A plain radiograph may show a fracture line that crosses a coronal or lambdoid suture, but it is usually limited to a parietal bone [13]. CT or MRI demonstrates a cystic lesion near the fracture site communicating with the subarachnoid spaces and extending though the bony defect. Margins of bony defect may be beveled or irregular. Adjacent brain parenchyma usually shows mild encephalomalacia changes and focal atrophy. Gliosis may also see in the adjacent brain parenchyma. On CT scan gliosis is seen as hypodense areas. On MRI gliosis is seen as hypointense T1 and hyperintense T2 signals.

Because of neurological deterioration and of seizure disorder surgical correction of growing fractures is recommended.

Even though traumatic leptomeningeal cyst is rare in adults, it should be considered in the differential diagnosis of intracranial cystic lesions with adjacent calvarial defects.

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[itsmedicinesfakianakis]

Glymphatic MRI in idiopathic normal pressure hydrocephalus

Abstract

The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer’s disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In idiopathic normal pressure hydrocephalus, we found delayed enhancement (P < 0.05) and decreased clearance of gadobutrol (P < 0.05) at the Sylvian fissure. Parenchymal (glymphatic) enhancement peaked overnight in both study groups, possibly indicating a crucial role of sleep, and was larger in normal pressure hydrocephalus patients (P < 0.05 at inferior frontal gyrus). We interpret decreased gadobutrol clearance from the subarachnoid space, along with persisting enhancement in brain parenchyma, as signs of reduced glymphatic clearance in idiopathic normal hydrocephalus, and hypothesize that reduced glymphatic function is instrumental for dementia in this disease. The study shows promise for glymphatic magnetic resonance imaging as a method to assess human brain metabolic function and renders a potential for contrast enhanced brain extravascular space imaging.

from #ORL-Sfakianakis via xlomafota13 on Inoreader http://ift.tt/2vaupPN

from OtoRhinoLaryngology - Alexandros G. Sfakianakis via Alexandros G.Sfakianakis on Inoreader http://ift.tt/2uREB4j