Post-traumatic tension pneumocephalus and cystic angiomatosis of the skull: case report

Background Tension pneumocephalus is an increasing air trapped intracranially. Either spontaneous, post-traumatic or iatrogenic in origin. Cystic angiomatosis is a benign vascular hamartoma of the skeleton, when acquired it is either due to trauma or infection. This is the second report in English literature of post-traumatic delayed tension pneumocephalus with the development of cystic angiomatosis of the skull bone. Case presentation A 55-year-old gentlemen, presented with scalp swelling of 6-month duration with history of head trauma 2 years back. The swelling was increasing and associated with progressive walking difficulties and left hearing loss. CT scan and MRI revealed extradural pneumocephalus, parietal and occipital pneumatocele, and multiple lytic bony lesions, left mastoid hyperpneumatization with inner table defect communicating with the extradural space. Diagnosis of delayed extradural tension pneumocephalus was made. Surgical exploration revealed multiple bony defects of parietal, temporal and squamous part of left temporal bones, confirmed extradural pneumocephalus with intact dura. Repair of mastoid defect of (0.5 × 0.5 cm), excision of pneumatocele and removal of lytic bones were performed. Defective bone “cribriform-like” was identified at occipital and parietal regions centrally with a defect of nearly 7 × 7 cm. Future cranioplasty was considered after 6 months. Histology of bony chips and surrounding soft tissues is recognized as cystic angiomatosis. Conclusions The present case developed two very rare complications, following trivial head trauma; the first complication was delayed extradural tension pneumocephalus with pneumatocele which presented 2 years after trauma, the origin of air was from a defect of the inner table of the mastoid, the second complication was cystic angiomatosis of the skull bones. Both complications were managed surgically in one operative session as a combined neurosurgery and otolaryngology teams approach.

Two rare cases of cystic angiomatosis and a literature review

Cystic angiomatosis is a rare disease characterized by disseminated multifocal hemangiomatous and/or lymphangiomatous cystic lesions of the skeleton with possible visceral organ involvement. Only a few dozens of such patients worldwide have been described in the literature. This article presents two case reports of the patients admitted to the D. Rogachev NRMCPHOI with suspected Langerhans cell histiocytosis. The patient’s parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications. During the investigation, multiple cysts of the skull bones, spine, pelvic bones and limbs, as well as of the spleen were found in both patients. A biopsy of the bone cysts of the skull revealed no data in favor of histiocytosis or other neoplasms. Cystic angiomatosis was diagnosed in both cases. This is a rare disease that should be kept in mind in the differential diagnosis in patients with cystic lesions of the bones and visceral organs.

Cystic angiomatosis of the bone, liver, and spleen

Introduction/Objective Cystic angiomatosis is a very rare entity characterized by multifocal cystic angiomatous lesions of the skeletal system, occasionally involving visceral organ systems. Clinical presentation and progression are extremely variable. Cases with liver involvement are even rarer, and have been described as invariably fatal. The pathogenesis of this disorder is poorly understood, and no treatments are currently available. Methods/Case Report A 24 year old man with history of polysubstance abuse and untreated hepatitis C presented with acute abdominal pain. Imaging revealed hepatosplenomegaly and multiple liver, spleen, and skeletal lesions. Differential diagnoses included metastatic disease, lymphoma, and infection. Apart from known untreated chronic hepatitis C, all infectious, autoimmune and hypercoagulability work-ups were negative. Biopsies of the liver and iliac bone lesions showed areas of benign-appearing vascular proliferation in a background of fibrosis. The vascular endothelial cells were positive for CD34 and CD31 but negative for D2-40. On follow-up approximately eight months later, the patient was asymptomatic. Results (if a Case Study enter NA) NA Conclusion Liver involvement by cystic angiomatosis is exceedingly rare and has been associated with increased mortality. This case provides an example of a patient with incidentally discovered cystic angiomatosis involving liver, spleen, and bone who remains asymptomatic at follow-up. Additional cases are required to understand the pathophysiology and disease course in this group of patients, and to investigate possible therapeutic targets.

Cystic angiomatosis skull lesion obliteration with neuroendovascular sclerotherapy as a unique treatment: case report

Cystic angiomatosis is a rare bone condition with complex presentation and difficult treatment. Current management strategies have poorly tolerated side effects and a low likelihood of disease eradication. The control of calvarial lesions that are symptomatic usually involves surgical excision and subsequent cranioplasty. This paradigm can present with a risk of morbidity and mortality depending on the anatomy of the lesion. Here, the authors present a novel approach to a difficult-to-treat occipital calvarial lesion directly overlying the transverse sinus, performing a small, partial-thickness craniectomy and alcohol sclerotherapy in a combined neurosurgery-neuroendovascular approach. At 3 years after treatment, the authors noted a complete, encouraging radiographic and clinical outcome.

Systemic Cystic Angiomatosis Mimicking Metastatic Cancer: A Case Report and Review of the Literature

Systemic cystic angiomatosis is a rare benign disorder due to the maldeveloped vascular and lymphatic system with less than 50 cases reported in literature so far. We report here a case of systemic cystic angiomatosis (SCA) with multisystem involvement affecting the neck, thyroid, thoracic cavity, and skeletal system. The patient initially presented in her 4th decade of life with isolated lymphangioma in the neck requiring surgery. However, she experienced full-blown manifestations of SCA in her 6th decade which closely mimicked metastatic cancer. The diagnosis of SCA could only be established after multiple biopsies. The radiological and histological features of SCA with its course over 31 years in this patient have been described.