Splenic rupture or infarction associated with Epstein-Barr virus infectious mononucleosis: a systematic literature review.

BACKGROUND: Epstein-Barr virus (EBV), also known as human herpesvirus 4, is one of the most common pathogenic viruses in humans. EBV mononucleosis always involves the spleen and as such it predisposes to splenic rupture, often without a trauma, and splenic infarction. Nowadays the goal of management is to preserve the spleen, thereby eliminating the risk of post-splenectomy infections. METHODS: To characterise these complications and their management, we performed a systematic review (PROSPERO CRD42022370268) following PRISMA guidelines in three databases: Excerpta Medica, the United States National Library of Medicine, and Web of Science. Articles listed in Google Scholar were also considered. Eligible articles were those describing splenic rupture or infarction in subjects with Epstein-Barr virus mononucleosis. RESULTS: In the literature, we found 171 articles published since 1970, documenting 186 cases with splenic rupture and 29 with infarction. Both conditions predominantly occurred in males, 60% and 70% respectively. Splenic rupture was preceded by a trauma in 17 (9.1%) cases. Approximately 80% (n = 139) of cases occurred within three weeks of the onset of mononucleosis symptoms. A correlation was found between the World Society of Emergency Surgery splenic rupture score, which was retrospectively calculated, and surgical management: splenectomy in 84% (n = 44) of cases with a severe score and in 58% (n = 70) of cases with a moderate or minor score (p = 0.001). The mortality rate of splenic rupture was 4.8% (n = 9). In splenic infarction, an underlying haematological condition was observed in 21% (n = 6) of cases. The treatment of splenic infarction was always conservative without any fatal outcomes. CONCLUSIONS: Similarly to traumatic splenic rupture, splenic preservation is increasingly common in the management of mononucleosis-associated cases as well. This complication is still occasionally fatal. Splenic infarction often occurs in subjects with a pre-existing haematological condition.

Epstein-Barr virus latent gene EBNA-1 genetic diversity among transplant patients compared with patients with infectious mononucleosis.

INTRODUCTION: As a step toward evaluating the association between Epstein-Barr virus genetic diversity and post-transplant lymphoproliferative disorder (PTLD), we conducted a preliminary study to compare the genetic diversity of the EBNA-1 gene among transplant patients and patients with infectious mononucleosis (IM). METHODS: We sequenced the EBNA-1 gene in blood samples from study subjects using Sanger methodology. The sequences were aligned with a reference strain and compared with publicly available sequences. RESULTS: We analyzed 33 study samples and 25 publicly available sequences along with the reference strain B95-8. The evaluable samples were from sixteen patients with IM (median age 14.0 years, range 2-24) and 17 transplant patients. There were six children without PTLD (median age 1.93 years, range 0.79-7.46) and 11 who developed PTLD (median age 5.67 years, range 0.96-17.45). A predominant EBNA-1 variant (P-thr) was identified across the study groups. Differences were observed between the samples from the IM patients compared with the transplant samples. CONCLUSION: The predominant EBNA-1 strain is in contrast to reports of the predominant strain in North America. The results suggest differences between the EBNA-1 strains among the study groups. Further studies will examine the relationship between EBNA-1 strains and PTLD occurrence and outcomes.

Clinical practice guideline: tonsillitis II. Surgical management.

In 2013, a total of 84,332 patients had undergone extracapsular tonsillectomies (TE) and 11,493 a tonsillotomy (TT) procedure in Germany. While the latter is increasingly performed, the number of the former is continually decreasing. However, a constant number of approximately 12,000 surgical procedures in terms of abscess-tonsillectomies or incision and drainage are annually performed in Germany to treat patients with a peritonsillar abscess. The purpose of this part of the clinical guideline is to provide clinicians in any setting with a clinically focused multi-disciplinary guidance through the surgical treatment options to reduce inappropriate variation in clinical care, improve clinical outcome and reduce harm. Surgical treatment options encompass intracapsular as well as extracapsular tonsil surgery and are related to three distinct entities: recurrent episodes of (1) acute tonsillitis, (2) peritonsillar abscess and (3) infectious mononucleosis. Conservative management of these entities is subject of part I of this guideline. (1) The quality of evidence for TE to resolve recurrent episodes of tonsillitis is moderate for children and low for adults. Conclusions concerning the efficacy of TE on the number of sore throat episodes per year are limited to 12 postoperative months in children and 5-6 months in adults. The impact of TE on the number of sore throat episodes per year in children is modest. Due to the heterogeneity of data, no firm conclusions on the effectiveness of TE in adults can be drawn. There is still an urgent need for further research to reliably estimate the value of TE compared to non-surgical therapy of tonsillitis/tonsillo-pharyngitis. The impact of TE on quality of life is considered as being positive, but further research is mandatory to establish appropriate inventories and standardized evaluation procedures, especially in children. In contrast to TE, TT or comparable procedures are characterized by a substantially lower postoperative morbidity in terms of pain and bleeding. Although tonsillar tissue remains along the capsule, the outcome appears not to differ from TE, at least in the pediatric population and young adults. Age and a history of tonsillitis are not a contraindication, abscess formation in the tonsillar remnants is an extremely rare finding. The volume of the tonsils should be graded according to Brodsky and a grade >1 is considered to be eligible for TT. The number of episodes during 12 months prior to presentation is crucial to indicate either TE or TT. While surgery is not indicated in patients with less than three episodes, a wait-and-see policy for 6 months is justified to include the potential of a spontaneous healing before surgery is considered. Six or more episodes appear to justify tonsil surgery. (2) Needle aspiration, incision and drainage, and abscess tonsillectomy are effective methods to treat patients with peritonsillar abscess. Compliance and ability of the patient to cooperate must be taken into account when choosing the surgical method. Simultaneous antibiotic therapy is recommended but still subject of scientific research. Abscess tonsillectomy should be preferred, if complications have occurred or if alternative therapeutic procedures had failed. Simultaneous TE of the contralateral side should only be performed when criteria for elective TE are matched or in cases of bilateral peritonsillar abscess. Needle aspiration or incision and drainage should be preferred if co-morbidities exist or an increased surgical risk or coagulation disorders are present. Recurrences of peritonsillar abscesses after needle aspiration or incision and drainage are rare. Interval TE should not be performed, the approach is not supported by contemporary clinical studies. (3) In patients with infectious mononucleosis TE should not be performed as a routine procedure for symptom control. TE is indicated in cases with clinically significant upper airway obstruction resulting from inflammatory tonsillar hyperplasia. If signs of a concomitant bacterial infection are not present, antibiotics should not be applied. Steroids may be administered for symptom relief.

Splenic rupture in infectious mononucleosis: A systematic review of published case reports.

INTRODUCTION: Infectious mononucleosis (IM) is a common viral illness that predominantly causes sore throat, fever and cervical lymphadenopathy in adolescents and young adults. Although usually a benign, self-limiting disease, it is associated with a small risk of splenic rupture, which can be life-threatening. It is common practice therefore to advise avoiding vigorous physical activity for at least 4-6 weeks, however this is not based on controlled trials or national guidelines. We reviewed published case reports of splenic rupture occurring in the context of IM in an attempt to ascertain common factors that may predict who is at risk. METHOD: A search of MEDLINE and EMBASE databases was performed for case reports or series published between 1984 and 2014. In total, 52 articles or abstracts reported 85 cases. Data was extracted and compiled into a Microsoft Excel(®) spreadsheet. RESULTS: The average patient age was 22 years, the majority (70%) being male. The average time between onset of IM symptoms and splenic rupture was 14 days, with a range up to 8 weeks. There was a preceding history of trauma reported in only 14%. Abdominal pain was the commonest presenting complaint of splenic rupture, being present in 88%. 32% were successfully managed non-operatively, whereas 67% underwent splenectomy. Overall mortality was 9%. CONCLUSIONS AND RECOMMENDATIONS: From our data, it appears that men under 30 within 4 weeks of symptom onset are at highest risk of splenic rupture, therefore particular vigilance in this group is required. As cases have occurred up to 8 weeks after the onset of illness, we would recommend avoidance of sports, heavy lifting and vigorous activity for 8 weeks. Should the patient wish to return to high risk activities prior to this, an USS should be performed to ensure resolution of splenomegaly. The majority of cases reviewed had no preceding trauma, although previous studies have suggested this may be so minor as to go unnoticed by the patient. It is therefore prudent to warn patients about the symptoms of splenic rupture to ensure prompt presentation and minimise treatment delay rather than focusing purely on activity limitation.

Case report: a diagnostically challenging conjunctival mass caused by the Epstein-Barr virus.

We present a paediatric case of infectious mononucleosis in a 13-year old, manifesting with follicular conjunctivitis and a conjunctival mass in one eye with no evidence of leucocytosis on the blood count. The diagnosis was confirmed following surgical excision and biopsy. The case represented a diagnostic challenge due to its atypism and given the steady increase in the prevalence of EBV-related ocular diseases in the last years, this report can serve as an example to prompt earlier serological tests to identify the aetiology in similar cases. This is important because EBV can be treated with acyclovir early in the active viral phase.

Severe genital ulceration in an acute epstein-barr virus infection.

Genital ulceration is a rare manifestation of infectious mononucleosis caused by Epstein-Barr virus (EBV). We report a girl with severe genital ulceration and tissue necrosis during primary EBV infection that required surgical debridement. The excised genital tissue was EBV polymerase chain reaction-positive whereas in situ hybridization was negative. This suggests that the ulceration was likely because of the inflammatory response to the virus and not because of the intense viral infection of the genitalia.

[Occult rupture of the spleen in a patient with infectious mononucleosis]. / Rottura splenica "occulta" in paziente con mononucleosi infettiva.

INTRODUCTION: Rupture of the spleen can be secondary to abdominal traumas (usually closed trauma) or spontaneous, can interest an organ normal or with morphological alterations secondary to various pathologies. Among the diseases responsible of occult rupture, infectious diseases are the most frequent and, among these, infectious mononucleosis, that is complicated with splenic rupture in 0.5% of the cases, with 30% of mortality. CASE REPORT: P.M., 16 years old female, admitted with acute abdomen, progressive anaemia and incipient cardiovascular instability, associated with suggestive clinical diagnosis of infectious mononucleosis, confirmed by serological findings and histological examination. Because of the imaging of subcapsular splenic haematoma, probably ruptured and with peritoneal bleeding we opt for emergency laparotomy intraoperative findings allows to splenectomy. DISCUSSION: Splenic rupture in infectious mononucleosis often presents as left hypochondrial pain, rare in uncomplicated cases; its occurrence in a patient with a recent diagnosis of infectious mononucleosis or with clinical or laboratory features suggestive of acute EBV infection, should always be investigated with an urgent abdominal ultrasound scan or CT. This approach is mandatory when hypochondrial pain is associated with pain referred to the left shoulder (Kehr's sign), peritoneal irritation and haemodynamic instability. Patients with splenic rupture in infectious mononucleosis generally undergo emergency splenectomy.

Ocular manifestation of primary VZV infection in a splenectomized patient.

PURPOSE: To report a rare case of primary varicella zoster virus (VZV)-associated retinal vasculitis in a splenectomized patient. DESIGN: Case report. RESULTS: After manifestation of VZV-associated retinal vasculitis, a splenectomized patient experienced binocular loss of vision. CONCLUSIONS: For the development of VZV-associated uveitis, the presence of specific T cells are necessary. Here, the authors present a rare case of VZV-associated retinal vasculitis in a splenectomized patient.

Infectious mononucleosis manifested as a cecal mass.

An 11-year-old boy presented with symptoms of periappendiceal abscess. At laparotomy, a tumor-like mass involving the ileocecal mesentery and adjacent part of the cecum and covered by the omentum was found. A right hemicolectomy was performed, followed by primary ileocolic anastomosis. On histologic examination, the tumor-like mass proved to be a lymph node block pushing the adjacent wall of the cecum. The overall histologic and immunophenotypic findings were consistent with (a) abnormal immune lesion mimicking lymphomatous process because of infection by Epstein-Barr virus and (b) extensive acute inflammatory infiltration of the cecum and adjacent tissues.

[Evidence based indications for tonsillectomy]. / Evidenz basierte Indikationen zur Tonsillektomie.

Tonsillectomy (TE) is one of the most frequent as well as one of the most controversial surgical interventions. The objective of this paper was to assess the evidence of the most common indications for TE. For this purpose the literature of the past 25 years was analysed using the criteria of evidence based medicine. In children seventy-five percent of TEs are performed because of recurrent tonsillitis. Several randomised clinical trials (RCTs) have demonstrated the efficacy of TE in this disease. This indication is based on evidence grade I-II. No consensus has yet been reached, however, about the number of annual episodes that justify TE. The remaining paediatric TEs are performed to relieve symptoms of airway obstruction. TE has been shown to improve obstructive symptoms in up to 100% of patients. It is the accepted treatment of paediatric obstructive sleep apnoea, although the evidence is not based on major RCTs. In adults, too, the majority of TEs are performed for recurrent or chronic tonsillitis. There are no good RCTs, but the indication can be based on a series of well controlled studies (evidence grade III). Obviously, TE is indicated if there is suspicion of neoplasm. Peritonsillar abscess per se is no indication for TE, unless the abscess cannot be drained otherwise. TE plays no role in the standard management of OSAS and mononucleosis.

Florid infectious mononucleosis: clinicopathological correlation in acute tonsillectomy.

In patients with infectious mononucleosis (IM), acute tonsillectomy is advocated only in the minority who develop severe airway compromise. In such florid cases, the pathological diagnosis of IM, well known to morphologically simulate that of lymphoma, is particularly challenging. The present case describes a 15-year old male with a clinical course consistent with IM in whom acute tonsillectomy was performed due to progressive airway obstruction. The striking histological findings emphasize the need for close clinicopathological correlation. Recently available ancillary studies are described which both surgeon and pathologist should be familiar with in the differentiation of IM from other lymphoproliferative conditions.

[Infectious mononucleosis: when is tonsillectomy indicated?]. / Infektiöse Mononukleose: Wann ist eine Tonsillektomie indiziert?

INTRODUCTION: Infectious mononucleosis is usually a benign, self-limited disorder of the lymphopoietic system. Tonsillopharyngitis is a common presentation of the disease. Occasionally upper airway obstruction or a prolonged swallowing disorder require surgical intervention. METHODS: We conducted a retrospective analysis of all infectious mononucleosis cases seen at the ENT clinic in St. Gallen, Switzerland, between 1994 and 1998. RESULTS: 21 records were analysed, among which 19 patients needed hospital care. 11 cases required tonsillectomy. The average duration of fever of the patients undergoing surgery was markedly lower (9 days) than the conservatively managed cases (14.2). DISCUSSION: Tonsillectomy is well tolerated, eliminates airway obstruction and rapidly improves swallowing.

Infectious mononucleosis and bilateral peritonsillar abscesses resulting in airway obstruction.

Upper airway obstruction is an uncommon but recognized complication of infectious mononucleosis. The management depends upon the degree of airway compromise. In the case described, severe airway obstruction was treated by securing the airway with awake fibre-optic endoscopic intubation and then proceeding to tonsillectomy. Bilateral inferiorly loculated quinsies were encountered unexpectedly and drained. This is the first report of 'bilateral' quinsies, associated with infectious mononucleosis and severe airway obstruction. The association, pathogenesis and significance of this finding are also discussed.

Prolonged muscle weakness following emergency tonsillectomy in a patient with familial periodic paralysis and infectious mononucleosis.

A thirteen-year-old girl with normokalaemic familial periodic paralysis (FPP) suffered life threatening upper airway obstruction secondary to tonsillopharyngitis resulting from infectious mononucleosis (IM). Emergency tonsillectomy was performed, but her postoperative course was complicated by persistent muscle weakness requiring a very prolonged period of artificial ventilation.

Acute tonsillectomy in the management of infectious mononucleosis.

Life-threatening upper airway obstruction can be caused by tonsillopharyngitis secondary to infectious mononucleosis (IM). The administration of corticosteroids, emergency tracheostomy and acute tonsillectomy have been advocated as ways of managing this problem. In a series of 25 patients admitted over a five-year period with IM, 15 were judged to have symptoms severe enough to warrant the administration of corticosteroids. Six of these 15 patients had little improvement in their condition and thus underwent acute tonsillectomy. There were no significant complications of this surgery. A further three patients who received corticosteroids required tonsillectomy for recurrent tonsillitis later in the study period. By contrast, only one of the ten patients who did not receive corticosteroids subsequently required tonsillectomy. Acute tonsillectomy is of value in selected cases of IM tonsillopharyngitis. It may decrease the morbidity of recurrent tonsillitis after IM, in addition to averting the immediate risk of respiratory obstruction.

Spontaneous splenic rupture in infectious mononucleosis.

Spontaneous splenic rupture occurs in association with infectious mononucleosis. Three cases of serologically proven infectious mononucleosis complicated by spontaneous splenic rupture are described. In two, the diagnosis was confirmed preoperatively by abdominal computerized tomographic (CT) scan. Treatment by splenectomy was curative in all three patients. Infectious mononucleosis patients who have acute abdominal pain must be considered for potential splenic hemorrhage.

[Infectious mononucleosis and splenic pseudocyst: presentation of a case]. / Mononucléose infectieuse et pseudokyste splénique: présentation d'un cas.

The authors report the clinical case of an 18-year-old patient who presented with a symptomatic mass in the left upper quadrant 6 months after having infectious mononucleosis. The preoperative investigation consisted principally of echography and computerized axial tomography of the abdomen, which demonstrated a cystic mass of the lower pole of the spleen. Splenectomy was carried out. Histologic examination showed a pseudocyst of the spleen with a fibrous capsule without an epithelial lining. The treatment and pathogenesis of pseudocysts of the spleen secondary to infectious mononucleosis are discussed.

[Antibody formation against specific Epstein-Barr virus antigens in infectious mononucleosis with tonsillectomy in the acute phase of the disease]. / Antikörpertiterverlauf gegen Epstein-Barr-Virus-spezifische Antigene bei infektiöser Mononukleose mit Tonsillektomie in der akuten Krankheitsphase.

In the present study, the clinical course and the specific humoral immunological response to Epstein-Barr virus antigens have been investigated in patients affected with infectious mononucleosis after tonsillectomy in the acute phase of the disease, compared against conservatively treated patients. Clinical results confirm that tonsillectomy in the acute phase of infectious mononucleosis has a favourable effect on the course of the disease, and reduces the duration of the disease by about half of the usual time. Statistically significant differences - indicating a confined, delayed humoral immunological response or unresponsiveness to some extent - regarding the examined antigens after tonsillectomy in the acute phase of infectious mononucleosis, could be observed between the two groups of patients in respect of the production of antibodies against viral capsid and nuclear antigens of the Epstein-Barr virus and heterophilic antibodies. For this reason, tonsillectomy should be suggested only as therapy of infectious mononucleosis in anginous courses of the disease which appear life-threatening.