info@biomedres.us
+1 (502) 904-2126
One Westbrook Corporate Center, Suite 300, Westchester, IL 60154, USA
Site Map
ISSN: 2574 -1241
ISSN: 2574 -1241
Received: July 06, 2022; Published: July 15, 2022
*Corresponding author: María José Coronado Assad, Epidemiologist, University CES, Colombia
DOI: 10.26717/BJSTR.2022.45.007164
Background: Developmental venous anomalies are the most frequently encountered common form of vascular malformations, with a reported incidence of up to 2.6% in a series of 4,069 brain autopsies. Parenchymal veins are divided into superficial and deep draining veins. Cerebral cavernous malformations, also known as cavernomas or cavernous hemangiomas, are clusters of abnormal hyalinized capillaries with no intervening brain tissue. The medusa head sign is seen in a developmental venous anomaly, where multiple radially arranged tributaries drain into a larger vein.
Methodology: A narrative review was carried out through various databases from January 2007 to February 2022; the search and selection of articles was carried out in journals indexed in English. The following keywords were used: developmental venous anomalies, cavernomas, medusa head, cerebral venous angioma.
Results: Developmental venous anomaly (DVA), also known as cerebral venous angioma, is a congenital malformation of the veins that drain the normal brain. The etiology of developmental venous anomalies remains uncertain but may be related to developmental arrest of venous structures. Isolated developmental venous anomalies do not require treatment. The jellyfish head sign is seen in a developmental venous anomaly (DVA), where multiple radially arranged tributaries drain into a larger vein.
Conclusion: This review offers precise information on the location of developmental venous anomalies, the studies available for their diagnosis, and the therapeutic approach offered, as well as other differential diagnoses.
Keywords: Developmental Venous Anomalies; Cavernomas; Medusa Head; Cerebral Venous Angioma
The developmental venous abnormality is the most frequent configuration of vascular malformations, it has an incidence of 2.6% in a series of 4069 brain autopsies. In 1967, Wolf et al. reported on the first patient who died from intracranial hemorrhage due to a lesion that was diagnosed as a venous angioma [1]. Since then, various terminologies have been used, such as venous malformation, venous angioma, and medullary venous malformation, implying that they were considered rare lesions, involving a high risk of bleeding [2]. Parenchymal veins are divided into superficial and deep draining veins. Superficial draining veins include pial veins, intracortical veins, subcortical veins, and superficial medullary veins. The deep draining veins are deep and form the four venous convergence zones on their way to the subependymal veins [3]. Cerebral cavernous malformations, which are also known as cavernomas or cavernous hemangiomas, are a class of capillaries that present hyaline alteration in the absence of intervening brain tissue. Consequently, to recurrent microhemorrhages and thrombosis, they are surrounded by deposits of hemosiderin and gliosis [2,3]. The jellyfish head sign is very common in developmental venous anomalies, where many radial inflows drain into a larger vein. Although this sign by itself does not have any clinical importance, since it does not represent any compromise for the patient’s health, just as it is a rare sign [4,5]. The importance of the jellyfish head sign lies in the association that it could have with other vascular malformations that, if they can compromise the patient’s health, so this sign, if present, is very suggestive that it may be associated with other pathologies [6,7]. For this reason, it was decided to carry out this work, in order to provide precise information on the location of developmental venous anomalies, the studies available for their diagnosis, and what is the therapeutic approach that is offered, as well as other diagnoses. differentials.
A systematic review was carried out, in which a search was carried out in various databases, which we highlight PubMed, Scielo and ScienceDirect. The compilation and selection of articles was carried out in journals indexed in English from the years 2007 to 2022. Keywords, the terms were carried out according to the DeCS and MeSH methodology: Developmental venous anomalies; cavernomas; jellyfish head; cerebral venous angioma. 58 original and review publications associated with the theme were identified, only 28 articles met the established inclusion requirements, such as articles that were in a range not less than the year 2007, that were full text articles and that reported on the developmental venous anomalies and the medusa head sign. As exclusion criteria, we found articles that did not have sufficient information and that did not present the full text at the time of review.
The mean age of patients presented was 35.8 years ranging from 21 -52 years. Males were 5 while 4 were females. Tuberculosis of soft tissue, nerves was found in one patient. Infected tendons were found in two patients. One patient had extensor tendons repaired (zone VI) previously. It was re-explored, debrided and ATT started afterwards (Figures 1 & 2). Another patient had swelling and decreased function of her right ring finger which she developed in her pregnancy. It was suspected to be tendinitis upon ultrasound and radiographs. Exploration and debridement of the finger was performed and ATT started after fluid cytology and histopathology reports were discussed with infectious disease department Two females were found to have compound ganglion with tuberculosis. One of those presented with swelling and decreased range of motion in her left wrist, palm and index finger. Compound ganglion was suggested on MRI which was confirmed by tissue biopsy. Antituberculous regime was started but when no improvement was observed, it was explored. ‘Rice bodies’ were found per operatively (Figure 3). The region was debulked and ATT continued for 1 year (Table 1).
Figure 1:
A. Inflamed ulnar nerve in distal arm, elbow and proximal forearm region.
B. Distal and proximal Ulnar nerve ends after excision showing caseation.
C. Excised ulnar nerve showing inflammation and caseous material at ends.
Figure 2: Previously repaired extensor tendons showing caseation.
Figure 3:
A. ‘Rice bodies’ in wrist and palm.
B. Rice bodies.
Table 1: Summary of cases of upper extremity tuberculosis.
Tuberculosis of bones were found in four patients. Lesions were found in carpal bones in one case and in metacarpals in another. Distal radius was found to be involved in one patient, and was a patient with lesion of mid shaft humerus. One patient had previously taken anti-tuberculous therapy for pulmonary TB and later developed the lesion in distal radius. Another patient was later found to have TB in spine on bone scan who later developed lesion in metacarpal bones. None of the other 7 patients showed any systemic disease. In 6 out of 9 cases, the disease involved the dominant upper limb showing a preponderance for the dominant side.
Infection of mycobacterium tuberculosis occurs via inhalation into the respiratory tract. If the mycobacterium escapes expulsion function of mucocilliary system, or the subject is immunocompromised acquired, the mycobacteria reach the alveoli where they are phagocytosed by macrophages. Equilibrium between the inoculum and the quality of the host’s immune response results in formation of granuloma. Granuloma is an organized structure of different inflammatory cells, which encapsulates the bacteria in a hypoxic and highly acidic environment. Ultimately, the granuloma undergoes caseous necrosis. This caseous necrotic mass then undergoes gradual calcification. When granuloma formation is incomplete, liquefaction occurs, allowing the mycobacterium to spread contiguously to surrounding tissues. Sometimes the liquefying granuloma erodes into a blood vessel resulting in blood borne spread of the bacillus, with seeding in sites outside the lungs. This is the mode of spread by which extra-pulmonary TB occurs [11].
In relevance to upper limb, tubercular tenosynovitis most commonly involves the flexor tendons of the dominant hand. Hand involvement is seen in 10% of patients with musculoskeletal disease, and dominant hand of the male population is affected common. Local findings at the time of exploration includes graunolomas, areas of fibrosis and classical ‘Rice Bodies’. The pathophysiology of ‘rice bodies’ associated with TB synovitis is interesting. Pimm and Waugh revised the history of rice bodies initially thought to be detachments of the synovial membrane or collection of fibrin exudates. In 1927, Rogers proved they are composed of tubercular material. The bodies are formed from synovial tubercle and are attached to the wall of the tubercular sac. Later, they seperate from the wall and appear similar to polished white rice. They contain an inner vague core of acidophilic material and an outer layer composed of collagen and fibrin. Rice bodies can be seen in other synovial disorders such as rheumatoid arthritis, and fungal infections, seronegative inflammatory arthritis and synovial chondromatosis [12]. Radiograph of local lesion is more or less normal, unless in an advanced stage when the chronic mass effect causes bony erosion. Ultrasound is affordable, easy and early non-invasive diagnostic modality which shows an increased volume of synovial tendon sheath and a fluid collection. MRI is gold standard investigation because of excellent soft tissue demarcation. T2-weighted images gives an outline of fluid content inside synovium with a mixed solid cystic appearance. Gadoliniumenhanced imaging can also be done. Histopathology of synovial tissue shows granulomatous lesions with caseation surrounded by multiple giant and epithelioid cells [13].
Management of such cases involves multi-disciplinary approach. It involves input from infectious disease specialists, radiologists and reconstructive surgeons. Surgical treatment is done where it is thought that alone medical treatment would improve the condition of the patient. Additionally, it is done in cases of neuropathies [14]. Additionally, surgery avoids complications such as spontaneous tendon rupture and possible functional limitations. Synovectomy with removal of all rice bodies was the treatment of choice in the cases reported by us, followed a three-month interval for definitive reconstruction. A study suggested surgical debridement with excision of involved synovium and irrigation along with anti TB medication regimen for 12 months [15]. In case when there is joint involvement, tuberculous arthritis is generally a monoarticular disease that typically involves the spine or large and medium-sized joints. Moreover it must be differentiated with rheumatoid arthritis On the other side, rheumatoid arthritis (RA) is usually a symmetric polyarticular disease with commonly involves peripheral joints [16].
Along with conventional tests for detection of mycobacterium tuberculosis rapid molecular tests are also available. These are GeneXpert MTB/ RIF (Cepheid, Sunnyvale, CA, USA) and the Reverse Hybridization Test on strips Geno Type MTBDR plus (HAIN Life Sciences, Nehren, Germany). GeneXpert MTB/RIF is the only rapid molecular test recommended by WHO for the rapid diagnosis of tuberculosis. It detects presence of the Mycobacterium tuberculosis complex gene in specimens and the genomic sequences of the main mutations causing Rifampicin resistance. The reporting time is 2h. Extra pulmonary samples reveal the high sensitivity and the specificity of GeneXpert MTB/RIF for the diagnosis of extrapulmonary tuberculosis as compared to those found for pulmonary specimens. The sensitivity of the GeneXpert MTB/RIF vary in a series between 64.3% in pus samples and 100% in osteoarticular samples. The specificity was 64% in ganglion samples and 100% in osteoarticular samples. The sensitivity is low for soft tissue samples, they best show up on histological analysis [17,18]. Besides advances in the treatment of TB, the emergence of multidrug-resistant tuberculosis (MDR-TB) is a global concern. Pakistan is ranked 5th position in terms of a high burden of MDRTB in the world [19,20]. With this literature review we stress upon managing this entity in a multi-disciplinary setting.
Isolated tuberculosis is a significant entity. It is considered when diagnosing unexplained presentations in limbs. A prompt diagnosis of exclusion and treatment makes patient disease free.
Authors have no financial and personal relationships with any organization that could influence this work.
Abstract
PDF
