Management of Concealed Type IV Endoleak and Aortic Sac Hygroma by Prone ContrASt EnHancement Computed Tomography Angiography.

BACKGROUND: Aortic sac hygroma and concealed endoleaks (EL) after endovascular aneurysm repair (EVAR) of abdominal aortic aneurysm needs particular attention with aggressive management as they are associated with rapid sac expansion and rupture risk. However, they can be erroneously reported as type IV or V EL with supine computed tomography (CT) scans, leading to delay in management. Therefore, we describe a novel diagnostic technique, 'Prone contrASt enHanced computed tomography Angiography' (PASHA), to document concealed EL METHODS: We present eight case descriptions with continuous sac expansion after primary EVAR. Management began with diagnosis using the PASHA imaging technique. PASHA is a multiphase CTA positional technique for increasing the accuracy of detecting EL after EVAR. Furthermore, the PASHA imaging technique also guides whether the open or endovascular intervention could be used effectively to manage the sac expansion. In synchrony with the PASHA technique, "EVAR GORE SalvAge FAbric Technique" (ARAFAT) was to salvage previous EVAR. RESULTS: The PASHA technique diagnosed all cases of type IIIb EL, as it enhanced the degree of contrast infiltration into the aortic sac when microleaks were present. ARAFAT was effectively used in five elderly patients. Another three had an open conversion; two with double breasting of the aortic sac and one EVAR explantation. CONCLUSIONS: The PASHA protocol helped classify and localize the concealed EL (type IV, V), which were not appropriately diagnosed by supine CT protocols. PASHA and ARAFAT were used as a fully functioning protocol to overcome apparent challenges in accurate diagnosis and subsequent concealed EL management in high-risk patients.

Perigraft hygroma - a rare cause of post-EVAR aneurysm growth.

Development of perigraft hygromas following repair of abdominal aortic aneurysms is extremely rare. A case is presented of a patient who was found to have a large hygroma despite two re-interventions on a previous EVAR.

Intracranial hypotension with a sixth cranial nerve palsy subsequent to massive thoracic CSF hygroma: a rare complication of thoracic disc excision.

BACKGROUND: Thoracic cerebrospinal fluid (CSF) hygroma is a rare and potentially devastating complication of the anterior thoracic approach to the spine. We present two cases in which this complication resulted in acute cranial nerve palsy and discuss the pathoanatomy and management options in this scenario. CASE REPORTS: Two male patients presented to our department with neurological deterioration due to a giant herniated thoracic disc. The extruded disc fragment was noted pre-operatively to be calcified in both patients. A durotomy was performed at primary disc prolapse resection in the first patient, whereas an incidental durotomy during the procedure caused complication in the second patient. These were repaired primarily or sealed with Tachosil(®). Both patients re-presented with acute diplopia. Imaging of both patients confirmed a massive thoracic cerebrospinal fluid hygroma and evidence of intracranial changes in keeping with intracranial hypotension, but no obvious brain stem shift. The hemithorax was re-explored and the dural repair was revised. The first patient made a full recovery within 3 months. The second patient was managed conservatively and took 5 months for improvement in his ophthalmic symptoms. CONCLUSIONS: The risk of CSF leakage post-dural repair into the thoracic cavity is raised due to local factors related to the chest cavity. Dural repairs can fail in the presence of an acute increase in CSF pressure, for example whilst sneezing. Intracranial hypotension can result in subsequent hygroma and possibly haematoma formation. The resultant cranial nerve palsy may be managed expectantly except in the setting of symptomatic subdural haematoma or compressive pneumocephaly.

External Hydrocephalus as a Cause of Infant Subdural Hematoma: Epidemiological and Radiological Investigations of Infants Suspected of Being Abused.

BACKGROUND: Acute subdural hematoma (ASDH) and chronic subdural hematoma (CSDH) in infants have been regarded as highly specific for abuse. Other causes of CSDH have not been investigated in a large population. PURPOSE: The purpose of this study was to investigate to what extent external hydrocephalus is present in infants with ASDH and CSDH undergoing evaluation for abuse. MATERIAL AND METHODS: Eighty-five infants suspected of being abused, with ASDH (n = 16) or CSDH (n = 69), were reviewed regarding age, risk factor profiles, craniocortical width (CCW), sinocortical width (SCW), frontal interhemispheric width (IHW), subarachnoid space width (SSW), and head circumference (HC). In infants with unilateral subdural hematoma (SDH), correlations between contralateral SSW and ipsilateral CCW and SDH width were investigated. RESULTS: Infants with CSDH had significantly lower mortality, were more often premature and male, and had significantly higher CCW, SCW, IHW, and SSW than infants with ASDH (P < 0.05). Ipsilateral CCW (R = 0.92, P < 0.001) and SDH width (R = 0.81, P < 0.01) correlated with contralateral SSW. Increased HC was more prevalent in infants with CSDH (71%) than in infants with ASDH (14%) (P < 0.01). Forty-two infants, all with CSDH, had at least one of CCW, SCW, or IHW ≥95th percentile. Twenty infants, all with CSDH, had CCW, SCW, and IHW >5 mm, in addition to increased HC. CONCLUSION: A substantial proportion of infants with CSDH who had been suspected of being abused had findings suggesting external hydrocephalus.

A natural history of aortic aneurysm hygroma.

A 56-year-old man with a family history of aortic aneurysm underwent routine repair in 2003. A postoperative computed tomography scan showed a 6-cm perigraft hygroma. Sudden onset of abdominal pain 12 months later revealed a larger hygroma, with an additional anterior fluid collection suggestive of contained rupture. The bilobed hygroma remained stable until 2010, when he presented with chills and severe abdominal pain. A computed tomography scan demonstrated free rupture of the sister hygroma, with air pockets observed within the sac. Conservative management was elected. Air pockets as well as the hygroma eventually resolved, and the patient remains well.

Anaphylaxis to ethylene oxide - a rare and overlooked phenomenon?

Spina bifida patients have been reported to be at increased risk of anaphylactic reactions during general anaesthesia. Following a reaction, latex is often incriminated as spina bifida patients are known to have an increased incidence of latex allergy. Ethylene oxide (EO) has recently been suggested to be an alternative cause, but in many cases reported in the literature, it seems that EO has not been considered as a cause. EO is a highly reactive gas widely used to sterilise heat-sensitive medical devices, and traces of EO can be found in many of the same products as latex. We present the case of a spina bifida patient with a known latex allergy, where EO was found to be the cause of an anaphylactic reaction during general anaesthesia. In addition, we describe measures taken during preparation of a subsequent general anaesthesia to minimise exposure to EO. Spina bifida patients seem to be at increased risk of sensitisation against EO due to repeated exposure, but only limited literature is available. To ensure that EO is considered as a cause in these cases, we recommend that testing for latex and EO go hand in hand following an anaphylactic reaction in this high-risk population.

Delayed posttraumatic unilateral occipital epidural hygroma in early childhood.

An extremely rare case of posttraumatic epidural hygroma in the left occipital supratentorial and infratentorial region is reported. A year and five months old child was admitted to the Clinic of Neurosurgery with sustained occipital head injury. She presented with drowsiness and vomiting due to intracranial hypertension. Initial computed tomography scan revealed left-sided fracture of the squamous part of the occipital bone without associated traumatic changes to the brain. A second spiral computed tomography scan was obtained two days later because of persisting symptoms of increased intracranial pressure. It demonstrated a newly formed left-sided epidural hygroma adjacent to the skull fracture in the left supratentorial and infratentorial occipital region. The case is discussed with emphasis on the mechanism of formation of epidural hygroma and an attempt has been made to outline the major predisposing factors leading to the development of this traumatic disease. Necessity for computed tomography follow-up is pointed out in order to diagnose delayed posttraumatic hygromas. The recommended surgical approach should include craniotomy centered at the site of the epidural hygroma and obligatory dural elevation by means of traction sutures to eliminate the posttraumatic epidural cavity.

Decompressive Craniectomy in Traumatic Brain Injury-Craniectomy-Related and Cranioplasty-Related Complications in a Single Center.

OBJECTIVE: Decompressive craniectomy (DC) relieves intracranial hypertension after severe traumatic brain injury (TBI), but it has been associated with poor clinical outcome in 2 recent randomized controlled trials. In this study, we investigated the incidence and explanatory variables for DC-related and cranioplasty (CP)-related complications after TBI. METHODS: In this retrospective study, we identified 61 patients with TBI who were treated with DC in the neurointensive care unit, Uppsala University Hospital, Sweden, between 2008 and 2018. Demography, admission status, radiology, and clinical outcome were analyzed. RESULTS: Eleven patients (18%) were reoperated because of postoperative hemorrhage after DC. Six (10%) developed postoperative infection during neurointensive care. Twenty-eight (46%) developed subdural hygromas and 10 (16%) received a permanent cerebrospinal fluid shunt. Sixteen patients (26%) died before CP. Median time to CP was 7 months (range, 2-19 months) and 32 (71%) were operated on with autologous bone and 13 (29%) with synthetic material primarily. In 9 patients with autologous bone (29%), the CP had to be replaced because of bone resorption/infection, whereas this did not occur after synthetic material (P = 0.04). However, all 4 postoperative hemorrhages after CP occurred when synthetic material was used (P = 0.005). CONCLUSIONS: DC and CP surgery have a high risk for complications, leading to additional neurosurgery in about one third of cases. Synthetic CP materials may decrease the risk of reoperation, but special care with hemostasis is required because of increased risk of postoperative hemorrhage. Future trials need to address these topics to further improve the outcome for these patients.

Compensatory parameters of intracranial space in giant hydrocephalus.

BACKGROUND AND PURPOSE: The main goal of the present study is to examine compensatory parameters of intracranial space in giant hydrocephalus. We also assess the early and late outcome and analyse complications in shunted cases. MATERIAL AND METHODS: Nine cases of giant hydrocephalus characterised by the value of Evans ratio > 0.5, ventricular index > 1.5, and the width of the third ventricle > 20 mm were considered. Using the lumbar infusion test and developed software we analysed the intracranial compensatory parameters typical for hydrocephalus. Based on the Marmarou model, the method depended on a repeated search for the best fitting curve corresponding to the progress of the test was used. Eight out of nine patients were therefore shunted. Patients were followed up for 9 months. RESULTS: Five out of eight shunted patients undoubtedly improved in a few days after surgery (62%). Complications (subdural hygromas/haematomas and intracerebral haematoma) developed in 5 (62%) cases in longer follow-up. A definite improvement was noted in 4 out of 8 operated cases (50%). CONCLUSIONS: To get the stable values of compensatory parameters, the duration of the infusion test must at least double the inflexion time of the test curve. All but one considered cases of giant hydrocephalus were characterized by lack of intracranial space reserve, significantly reduced rate of CSF secretion and by various degrees of elevated value of the resistance to outflow. Due to the significant number of complications and uncertain long-term improvement, great caution in decision making for shunting has to be taken.

[Abdominal cystic tumor revealing lymphangioleiomyomatosis]. / Tumeurs kystiques rétropéritonéales compliquées révélatrices d'une lymphangioléiomyomatose.

We report the case of a 39 year-old woman with many years of intermittent abdominal pain who was found to have cystic masses evocative of cystic lymphangioma involving the posterior mediastinal and retroperitoneum. Worsening abdominal pain led to a recommendation for laparoscopic unroofing and decompression of the cysts. During the postoperative period, hemorrhagic shock required reintervention with excision of the tumoral mass. Pathologic examination revealed lymphangioleiomyomatosis (LAM). On the 15th postoperative day, the patient developed a chylopneumothorax which required prolonged chest tube drainage. The presence of multiple polycystic lesions in the pulmonary parenchyma supported the diagnosis of diffuse LAM with primary extrapulmonary presentation. This diagnosis should be considered preoperatively since it modifies the treatment: a complete excision of the cystic lesions seems to be necessary in order to prevent bleeding and lymphatic extravasation.

[Hygroma following liposuction and -grafting in the gluteal area].

This case report is of a 24-year-old woman, who was referred to an orthopaedic department with sarcoma in her left thigh. Further investigations revealed, that she had previously undergone an autologous lipografting from her thighs to her gluteal area, and she had developed widespread, severe hygroma formation as a late complication to the procedure. The complication rate to liposuction and -grafting varies 7-10% with seroma being the most abundant. Knowledge of these complications is important in order to diagnose and refer the patients correctly.

Surgical treatment of cyst of the canal of Nuck and prevention of lymphatic complications: A single-center experience.

The canal of Nuck is a residue of the peritoneal evagination that runs along the round ligament through the inguinal canal in women. Its partial or total patency can lead to a cystic lymphangioma (CL). CL of the canal of Nuck in an adult female is a rare entity and its clinical diagnosis can be difficult or incorrect. Ultrasonography can be useful to identify the nature of groin masses. A potential CL of the canal of Nuck should always be considered in the differential diagnosis of inguinal swelling in adult females. Even if it is possible to consider conservative treatment, the optimal therapeutic option is surgical excision of the cystic mass and closure of the inguinal ring by an anterior approach. In this study, we report a case series of four women affected by a cyst of the canal of Nuck to underline the surgical treatment's therapeutic role of this pathological condition and the importance of preliminary identification of lymphatic vessels with BPV (Blue Patent Violet) in order to prevent lymphatic injuries such as lymphorrea and lymphocele in the groin after surgery due to the disruption of inguinal lymph nodes and lymphatics.

Novel clinical phenotype of generalised lymphatic dysplasia in a neonate: a missed diagnosis.

We report a preterm neonate who had a large cervical cystic hygroma and right chylothorax. She was operated on day-21 and a near-complete resection of cystic hygroma was done. She developed refractory hypoxemia and shock post surgery and died after 24 hours. During autopsy, the chest cavity was found to be filled with chyle. Histopathological examination showed dilated lymphatics in the pleura, hepatic capsule, serosa of stomach and intestines, peri-pancreatic regions, peri-renal capsule and peri-adrenal tissues suggestive of generalised lymphatic dysplasia. Clinical exome sequencing did not reveal any pathogenic mutation in the genes involved in primary lymphatic dysplasia, noonan syndrome or rasopathies.

Are Hygromas and Hydrocephalus After Decompressive Craniectomy Caused by Impaired Brain Pulsatility, Cerebrospinal Fluid Hydrodynamics, and Glymphatic Drainage? Literature Overview and Illustrative Cases.

BACKGROUND: Poorly understood cranial fluid accumulations are frequently observed after decompressive craniectomy and often termed "external hydrocephalus." These findings are difficult to explain using traditional models of hydrocephalus. METHODS: Representative cases, clinical management, and literature overview are presented. RESULTS: We present a hypothesis that abnormal cranial fluid accumulations develop after decompressive craniectomy in a vulnerable subset of patients as a result of 1) the large compliant cranial defect with durotomy causing reduced internal brain expansion, ventricular squeezing, and pulsatile cerebrospinal fluid (CSF) circulation; 2) impaired pulsatile CSF flow along major cerebral arteries and the adjoining perivascular spaces (Virchow-Robin spaces); 3) reduced clearance of interstitial fluid by the glymphatic system; and 4) redistribution of CSF from the subarachnoid space into the subdural and subgaleal compartments and the ventricles. CONCLUSION: Closure of the cranial defect with cranioplasty improves cerebral blood flow and CSF pulsatile circulation and is frequently sufficient to resolve the external hydrocephalus.

Successful endovascular treatment of aneurysm sac hygroma after open abdominal aortic aneurysm replacement: a report of two cases.

We report two patients with perigraft hygromas after open abdominal aortic aneurysm replacement treated with relining the existing graft by covered stents with subsequent decrease in the size of the hygroma and disappearance of symptoms on follow-up. To our knowledge, these two patients are the first reported cases of successfully treated perigraft hygromas after open repair of abdominal aortic aneurysm using endograft relining of the existing grafts. This endovascular treatment avoids the need for aortic graft excision and replacement, which may be a challenging undertaking.

Hygromas after endoscopic third ventriculostomy in the first year of life: incidence, management and outcome in a series of 34 patients.

BACKGROUND: Overdrainage in shunted patients is a known predisposing factor for the formation of hygromas, but little is known about risk factors in endoscopic third ventriculocisternostomy (ETV). MATERIALS AND METHODS: We retrospectively analysed data of 34 patients younger than 1 year with obstructive hydrocephalus, undergoing ETV, with respect to incidence, management, outcome and possible risk factors for the formation of hygromas. Hygromas were arbitrarily defined as a collection of cerebrospinal fluid of more than 10 mm in diameter over the paramedian hemispheric convexities, diagnosed by ultrasonography. RESULTS: They occurred in 9 of 34 (26%) patients 3 to 28 days after ETV. They were on the operated side in four and bi-lateral in five cases. There was no relevant age difference between patients with hygromas (median 127 days) and those without hygromas (median 166 days). Etiology of obstructive hydrocephalus had no impact on the frequency of hygromas. Hygromas occurred somewhat less frequently when a paediatric endoscope with an outer diameter of 3 mm was used for ETV instead of an endoscope with a diameter of 6 mm. Hygromas were asymptomatic and conservatively managed in five cases; 4 of 34 (12%) patients underwent surgery because of clinical symptoms of increasing intracranial pressure or increasing hygroma diameter. Two patients were treated with a temporary external drainage only and another two patients with an external drainage first and eventually a subduro-peritoneal shunt. There were no neurological long-term sequelae. CONCLUSION: Clear predisposing factors for the formation of hygromas could not be identified, but the outer diameter of the endoscope may play a role.

A resistant case of spontaneous intracranial hypotension.

The case of a 39-year-old with intractable spontaneous intracranial hypotension (SIH) is presented. He developed bilateral and symptomatic subdural hygromas that were drained in response to clinical deterioration, but proved ineffective. An initial MRI of the lumbar region suggested a lumbosacral CSF leak, but he failed to respond to local blood patching. Subsequent CT myelography revealed a thoracic dural leak and a second directed blood patch proved effective. The aetiology, pitfalls and management of SIH are summarized.