scholarly journals Grisel’s syndrome in Kawasaki disease

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Xiaoliang Liu ◽  
Kaiyu Zhou ◽  
Yimin Hua ◽  
Mei Wu ◽  
Lei Liu ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Conservative Therapy ◽  
Rare Complication ◽  
Delayed Diagnosis ◽  
Cervical Lymphadenopathy ◽  
Neurological Impairment ◽  
Erythrocyte Sedimentation ◽  
Grisel’S Syndrome ◽  
Female Children ◽  
Intravenous Immunoglobulin Resistance

Abstract Background Approximately 50–70% of patients with Kawasaki disease (KD) could present with cervical lymphadenopathy associated with deep neck inflammation, which may result in Grisel’s syndrome (GS). Given the possibility of neurological impairment owing to GS, it is important to understand the disease profile in KD. Therefore, we carried out this study to investigate this possible complication of KD, with the aim of improving pediatricians’ recognition and awareness. Methods Patients with KD complicated by GS in our hospital were retrospectively recruited for our study. The profiles of patients with GS (n = 10) were compared to those patients without GS (n = 1254). All the available literature describing these complications of KD was reviewed. Results The incidence of GS in KD was 0.6% in our population. Compared to patients without GS, KD patients with GS were older, presented with a significantly lower male:female ratio, and a higher incidence of cervical lymphadenopathy, a higher level of neutrophil count, and erythrocyte sedimentation rate. Ten articles reporting 14 KD patients with GS were reviewed. Of the total 24 patients, GS affected 7 males and 17 females, aged from 3.5 to 9 years old. Encouragingly, no delayed diagnosis and treatment of KD was found, and all patients received conservative therapy for GS, without intravenous immunoglobulin resistance, coronary artery lesions, and neurological impairment. Conclusions GS is a rare complication of KD with an incidence of 0.6%, predominantly affecting older, female children. The overall outcome of this disorder in KD was satisfactory with conservative therapy. Pediatricians, especially pediatric surgeons, should recognize and be aware of this possible complication of KD to avoid misdiagnosis and overtreatment.

scholarly journals A Case of Incomplete and Atypical Kawasaki Disease Presenting with Retropharyngeal Involvement

2019 ◽  
Vol 16 (18) ◽  
pp. 3262
Author(s):  
Chiara Isidori ◽  
Lisa Sebastiani ◽  
Susanna Esposito
Keyword(s):  
Kawasaki Disease ◽  
Delayed Diagnosis ◽  
Cervical Lymphadenopathy ◽  
Intravenous Immunoglobulins ◽  
Case Presentation ◽  
Clinical Criteria ◽  
Atypical Kawasaki Disease ◽  
Laboratory Examinations ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Background: Kawasaki disease (KD) is a childhood acute febrile vasculitis of unknown aetiology. The diagnosis is based on clinical criteria, including unilateral cervical lymphadenopathy, which is the only presenting symptom associated with fever in 12% of cases. A prompt differential diagnosis distinguishing KD from infective lymphadenitis is therefore necessary to avoid incorrect and delayed diagnosis and the risk of cardiovascular sequelae. Case presentation: We describe the case of a 4 years old boy presenting with febrile right cervical lymphadenopathy, in which the unresponsiveness to broad-spectrum antibiotics, the following onset of other characteristic clinical features and the evidence on the magnetic resonance imaging (MRI) of retropharyngeal inflammation led to the diagnosis of incomplete and atypical KD. On day 8 of hospitalisation (i.e., 13 days after the onset of symptoms), one dose of intravenous immunoglobulins (IVIG; 2 g/kg) was administered with rapid defervescence, and acetylsalicylic acid (4 mg/kg/day) was started and continued at home for a total of 8 weeks. Laboratory examinations revealed a reduction in the white blood cell count and the levels of inflammatory markers, thrombocytosis, and persistently negative echocardiography. Clinically, we observed a gradual reduction of the right-side neck swelling. Fifteen days after discharge, the MRI of the neck showed a regression of the laterocervical lymphadenopathy and a resolution of the infiltration of the parapharyngeal and retropharyngeal spaces. Conclusion: Head and neck manifestations can be early presentations of KD, which is frequently misdiagnosed as suppurative lymphadenitis or retropharyngeal infection. A growing awareness of the several possible presentations of KD is therefore necessary. Computed tomography (CT) or MRI can be utilised to facilitate the diagnosis.

La Maladie de Grisel: Spontaneous Atlantoaxial Subluxation

2001 ◽  
Vol 38 (3) ◽  
pp. 268-270 ◽  
Author(s):  
M. F. Meek ◽  
R. A. E. C. Hermens ◽  
P. H. Robinson
Keyword(s):  
Oral Cavity ◽  
Neck Pain ◽  
Rare Complication ◽  
Atlantoaxial Subluxation ◽  
Unusual Complication ◽  
Additional Investigation ◽  
Grisel’S Syndrome

Objective: “La maladie de Grisel” (Grisel's syndrome) is a spontaneously occurring atlantoaxial subluxation with torticollis. We present a case of atlantoaxial subluxation occurring in a 20-year period of pharyngoplasty surgery. The occurrence of a “spontaneous” atlantoaxial subluxation after oral cavity or pharynx operations is rare. Because some neck pain and stiffness are commonly seen after these kinds of operations, we would like to draw attention to this unusual complication. Symptoms associated with a torticollis after an operation in the oral cavity or pharynx requires additional investigation to exclude this rare complication. A review of the available literature concerning etiology and treatment of la maladie de Grisel is presented.

scholarly journals Pancreaticopleural Fistula: A Cause of Recurrent Pleural Effusions in Chronic Pancreatitis

2021 ◽  
Author(s):  
Ayah Megahed ◽  
Rahul Hegde ◽  
Pranav Sharma ◽  
Rahmat Ali ◽  
Anas Bamashmos
Keyword(s):  
Chronic Pancreatitis ◽  
Pancreatic Duct ◽  
Pleural Fluid ◽  
Rare Complication ◽  
Delayed Diagnosis ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Pleural Effusions ◽  
Diagnostic Challenge ◽  
Abdominal Symptoms ◽  
Pancreatic Duct Stenting

AbstractPancreaticopleural fistula is a rare complication of chronic pancreatitis caused by disruption of the pancreatic duct and fistulous communication with the pleural cavity. It usually presents with respiratory symptoms from recurrent large volume pleural effusions. Paucity of abdominal symptoms makes it a diagnostic challenge, leading often to delayed diagnosis. Marked elevation of pleural fluid amylase, which is not a commonly performed test, is a sensitive marker in its detection. Imaging with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography can help delineate the fistula. In this report, we present the clinical features, imaging, and management of a 59-year-old male patient with pancreaticopleural fistula, wherein the diagnosis was suspected only after repeated pleural fluid drainages were performed for re-accumulating pleural effusions and it was eventually successfully treated with pancreatic duct stenting. We review the literature with regards to the incidence, presentation, diagnosis, and management of this rare entity.

Therapy of acute Kawasaki disease

1991 ◽  
Vol 1 (3) ◽  
pp. 254-255
Author(s):  
Jane W. Newburger
Keyword(s):  
Myocardial Infarction ◽  
Early Childhood ◽  
Kawasaki Disease ◽  
Sudden Death ◽  
Cervical Lymphadenopathy ◽  
Unknown Etiology ◽  
Acute Kawasaki Disease ◽  
Arterial Insufficiency ◽  
Infancy And Early Childhood ◽  
Arterial Aneurysms

Kawasaki disease is an acute vasculitis of unknown etiology that occurs predominantly in infancy and early childhood. It is characterize by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy.1,2 Coronary arterial aneurysms, or ectasia, develop in approximately 15 to 25% of children with the disease, and may lead to myocardial infarction, sudden death, or chronic coronary arterial insufficiency.2–4

Abstract 132: Methylprednisolone Pulse Therapy for Kawasaki Disease with Symptomatic Myocarditis

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
yeo hyang kim ◽  
Chae Ok Shin ◽  
Myung Chul Hyun ◽  
Dong Seok Lee
Keyword(s):  
Kawasaki Disease ◽  
Systolic Function ◽  
Systemic Vasculitis ◽  
Cervical Lymphadenopathy ◽  
Febrile Illness ◽  
Pulse Therapy ◽  
Left Ventricular ◽  
Acute Stage ◽  
Z Score ◽  
Intravenous Methylprednisolone

Purpose: Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a systemic vasculitis, especially involving the coronary arteries. Although, sometimes, subclinical myocarditis is combined in KD, symptomatic myocarditis is extremely uncommon. We report a 7 year old boy who developed hypotension and decreased left ventricular systolic function (EF 40%) in the acute phase of KD. Case: A 7 year old boy (height 115 cm, body weight 20 kg) was admitted because of 2 days of persistent fever and left cervical lymphadenopathy (white blood cell count 17,870 /mm 3 , C reactive protein 23.6 mg/dL). Conjunctiva injection and lip redness developed on the 4th day of illness, and hypotension and tachycardia (SBP 59/DBP 29 mmHg, HR 153/bpm) were combined. The echocardiography revealed a decreased ejection fraction (EF) (40%) without chamber dilatation and normal coronary artery size (LM 1.9mm, z score=-1.3, RCA 2.3mm, z score=0.4). The level of N terminal pro BNP was 28,000 pg/mL. With a diagnosis of KD with myocarditis, he was initially treated with inotropics and intravenous immunoglobulin (2 g/kg). Without clinical improvement in spite of initial treatment, A change of coronary arterial size (LM 2.9mm, z score=1.2, RCA 3.1mm, z score=2.3) was developed and decreased LV systolic function (EF 45%) and fever were persisted. Then, he was given 3 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. He showed prompt clinical recovery after pulse therapy of intravenous methylprednisolone (SBP 95/DBP 49 mmHg, HR 98/bpm). Although EF was improved (59%), coronary arterial dilatation was progressed (LM 3.4mm, z score=2.4 RCA 5.5mm, z score=7.9). Conclusions: The present case serves to highlight the fact that methylprednisolone should be considered as the priority in children with KD who have symptomatic myocarditis during the acute stage.

Lymphadenitis as the Dominant Manifestation of Kawasaki Disease

PEDIATRICS ◽  
1994 ◽  
Vol 93 (3) ◽  
pp. 525-528
Author(s):  
Julie Kim Stamos ◽  
Kathleen Corydon ◽  
James Donaldson ◽  
Stanford T. Shulman
Keyword(s):  
Kawasaki Disease ◽  
Cervical Lymphadenopathy ◽  
Febrile Illness ◽  
The United States ◽  
Acute Febrile Illness ◽  
Conjunctival Injection ◽  
Coronary Abnormalities ◽  
Acquired Heart Disease ◽  
Mucosal Changes ◽  
Infants And Young Children

Kawasaki disease (KD) is an acute febrile illness primarily affecting infants and young children. Its importance relates to the fact that 20% to 25% of untreated patients develop coronary abnormalities that can lead to myocardial infarction or even to death.1 KD is a leading cause of acquired heart disease in children in many regions, including the United States.2 Because there are no specific diagnostic tests for KD, the diagnosis is established by the presence of fever and four of five criteria without other explanation for the illness: (1) nonexudative conjunctival injection; (2) oral mucosal changes; (3) changes of the peripheral extremities; (4) rash, primarily truncal; and (5) cervical lymphadenopathy.

Abstract 212: Acute Myocardial Infarction in the Acute Phase in Kawasaki Disease in Mexican Children.

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
William Sarmiento-Robles ◽  
Luis M Garrido-Garcia
Keyword(s):  
Myocardial Infarction ◽  
Kawasaki Disease ◽  
Acute Phase ◽  
Delayed Diagnosis ◽  
Clinical Manifestations ◽  
Unknown Origin ◽  
Z Score ◽  
Laboratory Parameters ◽  
Small Series ◽  
Coronary Aneurysms

Background: Kawasaki disease (KD) is an acute febrile vasculitis of unknown origin. Despite treatment with intravenous immunoglobulin during the acute phase of the disease, up to 5% of those affected will develop coronary aneurysms predisposing them to thrombotic complications that could result in myocardial infarction (AMI). In Mexico there are few reports of ischemic complications secondary to KD. Objective: To describe the clinical features, the laboratory parameters, treatment used and the outcome of children who presented with myocardial infarction during the acute phase of KD in a third level facility in Mexico City Methods: From our Institutional Database of KD we search for children who presented AMI in the acute phase of the disease from August 1995 to August 2014. We analyzed gender, age, clinical manifestations, time from the onset of the symptoms to diagnosis, laboratory parameters, treatment used, and outcome in the acute phase of the disease. Results: Eight infants were diagnosed with AMI during the study period. The median age at diagnosis was 8 months (range 2 to 53 months). Seven patients were male (87.5%). The median from the onset of the clinical manifestations to diagnosis of KD was 22 days (range 4 to 26 days). All patients developed giant coronary aneurysms (median Z-score 18.98, with a range of Z-score from 11.58 - 27.70). An abnormal EKG and abnormal perfusion tests demonstrated the myocardial infarction in all cases. Two patients died in the acute phase of cardiogenic shock, one more patient died of dilated cardiomyopathy 12 months after coronary bypass surgery with an overall mortality of 62.5% of this group. Conclusions: AMI is a fatal complication of KD. In our small series it was associated with a delayed diagnosis of the disease and therefore the development of giant coronary aneurysms. Treatment of AMI in children after KD is a medical challenge with a poor prognosis in children.

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