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      Hamman’s syndrome in diabetic ketoacidosis

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          Abstract

          Hamman’s syndrome (spontaneous subcutaneous emphysema and pneumomediastinum) is a rare complication of diabetic ketoacidosis (DKA), with a multifactorial etiology. Awareness of this syndrome is important: it is likely underdiagnosed as the main symptom of shortness of breath is often attributed to Kussmaul’s breathing and the findings on chest radiograph can be subtle and easily missed. It is also important to be aware of and consider Boerhaave’s syndrome as a differential diagnosis, a more serious condition with a 40% mortality rate when diagnosis is delayed. We present a case of pneumomediastinum, pneumopericardium, epidural emphysema and subcutaneous emphysema complicating DKA in an eighteen-year-old patient. We hope that increasing awareness of Hamman’s syndrome, and how to distinguish it from Boerhaave’s syndrome, will lead to better recognition and management of these syndromes in patients with diabetic ketoacidosis.

          Learning points:
          • Hamman’s syndrome (spontaneous subcutaneous emphysema and pneumomediastinum) is a rare complication of DKA.

          • Presentation may be with chest or neck pain and shortness of breath, and signs are subcutaneous emphysema and Hamman’s sign – a precordial crunching or popping sound during systole.

          • Boerhaave’s syndrome should be considered as a differential diagnosis, especially in cases with severe vomiting.

          • The diagnosis of pneumomediastinum is made on chest radiograph, but a CT thorax with water-soluble oral contrast looking for contrast leak may be required if there is high clinical suspicion of Boerrhave’s syndrome.

          • Hamman’s syndrome has an excellent prognosis, self-resolving with the correction of the ketoacidosis in all published cases in the literature.

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          Most cited references6

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          Spontaneous mediastinal emphysema

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            Spontaneous pneumomediastinum: clinical and natural history.

            To evaluate the clinical characteristics and natural history of patients presenting with spontaneous pneumomediastinum. A retrospective case series was conducted to identify patients diagnosed with spontaneous pneumomediastinum. ICD-9 discharge codes were used for 1984 to 1990 at two institutions, and emergency department records of a third hospital were reviewed for 1981 to 1986. Clinical features, interventions, complications, setting, etiology, symptoms, and length of hospital stay were recorded. Three university tertiary care hospitals. All ED patients more than 12 years old with a diagnosis of spontaneous pneumomediastinum. None. Seventeen cases were identified. Age range was 15 to 41 years (mean, 25 years). Presenting symptoms were chest pain in eight (47%), dyspnea in three (18%), both symptoms in three (18%), and neither in three (18%). Three patients complained only of throat discomfort. Nine (52%) had a Hamman's crunch, 11 (65%) had subcutaneous emphysema, and two (11%) had a small pneumothorax. Five (29%) were smokers, and five (29%) had normal esophograms. Thirteen of 17 (76%) cases were associated with illicit inhalation drug use. Twelve cases (70%) had history of a "Valsalva-type" maneuver. All but three were admitted to a hospital, with a mean stay of 2.5 days (range, one to six). No patient suffered complications or required interventions for spontaneous pneumomediastinum. Specifically, no patient developed a subsequent pneumothorax or airway compromise. The three patients not admitted were followed up by telephone contact. All did well with rapid resolution of their symptoms. Most spontaneous pneumomediastinum cases occur in the setting of inhalational drug use. One hundred percent of patients will have a symptom directly related to the spontaneous pneumomediastinum, with 82% presenting with either dyspnea or chest pain. Most (88%) will present with either subcutaneous emphysema or a Hamman's crunch on examination. Simple spontaneous pneumomediastinum has a very benign course and does not require hospitalization. Serial radiographs, likewise, did not change the medical management of spontaneous pneumomediastinum.
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              The influence of the 'golden 24-h rule' on the prognosis of oesophageal perforation in the modern era.

              Rupture of the oesophagus is a surgical emergency with significant morbidity and mortality. We present our experience in managing such patients in a tertiary care cardiothoracic unit. We conducted a retrospective clinical review of patients who were admitted following rupture of the oesophagus over a period of 6 years (2002-2008). In our unit, there were 27 admissions following isolated rupture of the oesophagus, of which 18 were males and nine were females. The median age was 65 years (range 22-87). Twenty-four (89%) presented with spontaneous perforations (Boerhaave's syndrome) and three (11%) were iatrogenic. Primary surgical repair was done in 21 (77%) patients, a two-stage repair in 8% and conservative management in 16.6%. Mean hospital stay was 31 days (range 13-80 days). Overall, in-hospital mortality was five out of 27 patients (18.5%). Time from onset of symptoms to diagnosis of oesophageal perforation was early ( 24 h) in the remaining 10 (37%) patients. In four out of the five non-survivors, there was a >24-h delay in diagnosis. The mortality rate among patients with a delayed diagnosis was 40% compared to 6.2% among those who were diagnosed in <24 h (p=0.047). Our review confirms that an early diagnosis and management ('golden 24 h') are crucial for successful outcome in patients with rupture of the oesophagus. We reiterate the importance of critical care support, particularly in the early stages of management. For early detection, the primary and secondary care sectors need to be better educated. Copyright 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                30 November 2017
                2017
                : 2017
                : 17-0135
                Affiliations
                [1]Charing Cross Hospital , Imperial College Healthcare Trust, London, UK
                Author notes
                Correspondence should be addressed to A Benjamin; Email: Andrea.benjamin2@ 123456nhs.net
                Article
                EDM170135
                10.1530/EDM-17-0135
                5712834
                29218226
                01fd740f-d860-4155-b1f7-d7a626379565
                © 2017 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 06 October 2017
                : 08 November 2017
                Categories
                Unique/Unexpected Symptoms or Presentations of a Disease

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