Anorexia nervosa is a common eating disorder where patients have an affected body image and occurs predominantly in young adolescent females. The weight loss and starvation state of anorexia nervosa leads to widespread medical complications. We report a case of anorexia nervosa presenting with the association of pneumomediastinum, which was asymptomatic, and further development of ischemic liver injury.
Anorexia nervosa is a common eating disorder where patients have an affected body image and occurs predominantly in young adolescent females. Weight loss and starvation state of anorexia nervosa lead to widespread medical complications. We report a case of intended weight loss leading to primary pneumomediastinum) and grossly deranged liver function tests.
A 22-year old female presented to the gastroenterology outpatient clinic with a 7-month history of abdominal pain and weight loss.
Her symptoms came on halfway through a meal associated with a burning sensation in the back of her throat and early satiety. In 12 months her weight had dropped by 25 kg to 30.9 kg.
At that time, there were no evidence of issues concerning eating or excessive exercise, and no indication of self-induced vomiting. Her last period had been 5 months earlier.
Apart from having grommets, her past medical history was unremarkable. Her mother suffered from hypertension and hypothyroidism, but otherwise both her parents were in relatively good health.
She was an only child, living at home with her parents after completing a university degree; she worked part – time as a waitress. She did not smoke and consumed minimal alcohol.
On examination she was cachectic, with a blood pressure 80/50 mmHg and a regular heart rate 70 bpm. She had normal cardiovascular and respiratory examination. There was slight tenderness in the lower abdomen, with normal bowels sounds.
Her blood tests 7 months earlier had been unremarkable and those at the time of the clinic showed mild renal impairment, normocytic anaemia, leucopenia, thrombocytopenia but normal liver function, calcium, vitamin D and cortisol values. The level of follicle stimulating hormone and luteinizing hormones were suppressed. Her inflammatory markers and thyroid function was normal. She had no symptoms or biochemical features to suggest she was in a hypermetabolic state. Her autoimmune screen was negative.
Prior to being seen in clinic, she underwent a gastroduodenoscopy that revealed a small hiatus hernia and atrophic looking stomach. The duodenal (D2) biopsy result was normal consistent with negative tissue transglutaminase level.
The biopsy also did not suggest any features suggestive of parasitic, post-infectious infections or secondary deposition.
A computer tomography scan showed widespread surgical emphysema within the mediastinum, tracking down into the retroperitoneum of her upper abdomen, and moderate amount of free fluid in the pelvis.
Figure 1: Computer tomography (CT) scan in the transverse axis in lung windows demonstrating the presence of air, appearing black within the mediastinum (arrow) and in the subcutaneous tissue.
Figure 2: CT image in the coronal axis demonstrating the presence of surgical emphysema in the upper part of chest and air from the mediastinum extending into the retroperitoneal space.
She was admitted to hospital, where she underwent a water-soluble contrast swallow and repeat upper endoscopy. Both tests showed no evidence of esophageal perforation.
On the ward, she had episodes of hypoglycaemia, bradycardia, hypotension and hypothermia (33.5oC).
Her liver function tests became grossly deranged; alanine aminotransferase (ALD) rising to 1798,alkaline phosphatase of 649, bilirubin 22, with an INR 1.7. Viral hepatitis and autoantibody screen was negative. A review of the CT scan by the radiologists showed no evidence of portal vein thrombosis and the liver texture was unremarkable.
She was started on slow nasogastric feeding (25kcal/kg). She showed biochemical but no symptomatic features of re-feeding syndrome with hypophosphatemia and hypomagnesaemia, which were corrected through enteral routes. Initial intravenous treatment with thiamine, was followed by multivitamins tablets.
Her liver function tests started to improve towards baseline values, and it was thought her hepatic derangement was a combination of low blood pressure and ischaemic liver injury.
Clinically she improved, with no further hypoglycaemic or hypothermic episodes. She was seen by the psychiatrists, revealing underlying stress at university with studies, with a personality character consistent with social anxiety and perfectionism, with no features of depression or any other psychiatric illness.
She underwent further assessment at an specialised eating disorder hospital with the diagnosis of anorexia nervosa. Psychologically she was progressing well, with acceptance of her condition, and confiding that over the last year she undertook actions in order to avoid food for the purpose of weight loss and self-image. She would often tell her parents she had eaten at her friends or at work in order to avoid consumption of food at home.
She is currently making excellent progress nutritionally, with her weight increasing to 46 kg and resolution of her symptoms. Currently she is no longer receiving feeding and is maintaining her weight.
Pneumomediastinum is an air leak into the mediastinal tissues and is a recognised complication of anorexia nervosa. A literature search revealed 22 previous cases have been reported so far. Some of the cases have been preceded by a history of vomiting in an effort to lose weight ,, but the majority, like ours, occurred spontaneously , , .
Primary pneumomediastinum is a benign condition and has a self-limiting course, whereas secondary pneumomediastinum due to osephageal perforation is life-threatening with a mortality of 19.7% .
The differing outcome of primary as opposed to secondary pneumomediastinum, means the detection of free air in the mediastinum requires immediate admission and investigations for a possible oesophageal perforation.
The features of oesophageal rupture are pain especially on swallowing, dyspnoea, hypotension, tachycardia and cyanosis. They can be secondary to vomiting (Boerhaave’s syndrome), foreign bodies or iatrogenic .
We carried out both a gastromeira (water-soluble contrast) swallow and a gastroscopy to rule out an oesophageal perforation. The actual mechanisms that cause air to leak out into tissues in anorexia nervosa are not known. Animal studies have shown reduced alveolar elasticity during prolonged starvation, thus increasing the risk of alveolar rupture .
The reasons for the air leak have been discussed in a few published cases; air leaks out into the subcutaneous tissues because of weakness of the alveolar wall and loss of the connective tissues due to the starvation state.
Unlike in other cases, our patient did not complain of any chest pain, dyspnoea, difficulty swallowing or any significant palpable subcutaneous emphysema.
Hamman in 1939 described the signs and symptoms of pneumomediastinum. These include substernal chest pain that radiates to the neck and shoulders, palpable emphysema, air seen on the chest x-ray and the auscultatory finding of a crunching sound heard in synchrony with systole known as the Hamman’s sign. .
Spontaneous pneumomediastinum in anorexia nervosa will resolve spontaneously with weight gain and does not need any active intervention. In the majority of cases, the pneumomediastinum resolved within a few days of admission into hospital. Long term prognosis has only been reported in a few patients and is considered excellent.
Her liver function showed rapid deterioration with a marked increase in alanine aminotransferase, alkaline phosphatase and bilirubin values. Although deranged LFTs can occur in up to 40% of patients with anorexia nervosa , cases associated impaired synthetic function are rare. The marked changes did not mirror the drop in weight our patient had over a 7-month period. They were thought to be due to an acute hepatic ischemic injury from hypoperfusion, as a result of a combination of hypotension and bradycardia .
Another reason cited to cause moderately elevated LFTs in anorexia nervosa is hepatic steatosis, as seen in few other starvation states; there is an inverse relationship between level of adiposity and value of ALT and GGT ( r= -0.27 to -0.30 p Liver biopsy from patients with anorexia nervosa have shown changes of fatty liver, increased glycogen deposits, and periportal inflammation .
This patient’s LFTs improved with volume expansion and enteral feeding - hence this supports the theory that the more likely cause of her deranged LFT was hepatic hypoperfusion leading to ischaemic hepatitis. Rarely, there have been reports of acute liver failure secondary to anorexia nervosa often with fatal outcomes.
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