Rofo 2013; 185(9): 877-878
DOI: 10.1055/s-0033-1335918
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© Georg Thieme Verlag KG Stuttgart · New York

A Difficult Nasogastric Tube in a Patient with Nephrogenic Systemic Fibrosis

B. Vollnberg
,
F. Knebel
,
M. Böhm
,
S. Brockmöller
,
M. Dewey
Further Information

Publication History

03 May 2013

02 June 2013

Publication Date:
15 July 2013 (online)

A 71-year-old woman with advanced renal dysfunction (stage V) was hospitalized via our emergency department in a soporous condition with fever (40 °C), coughing, hypotonia (90/40 mm Hg), and tachycardia (140 bpm). Until then she had lived in a nursing home for the elderly. On auscultation she showed coarse crackles over the left lower area which was in good correlation with infiltrates in this region on plain radiograph ([Fig. 1a]). Blood work revealed elevated CRP (39 mg/dl) and PCT (105 µg/l), and the blood culture grew E. coli and S. aureus. She also had painful and diffusely hardened skin with ulcerations/indurations which was pronounced at the calves.

Zoom Image
Fig. 1 Pneumothorax caused by a nasogastric tube and histologic findings. a Initial chest radiographs before the nasogastric tube showed a normal right lung that reached the chest wall (arrowheads) and infiltrates into the left lower lung with left-sided diaphragmatic elevation. b Pneumothorax on a chest radiograph after nasogastric tube placement (arrow) with the outer lung borders (arrowheads) not reaching the chest wall and the deep sulcus sign (asterisk) which is typical for pneumothorax on a portable plain film of the chest. Based on the plain film b, we initially suggested that the nasogastric tube had been malpositioned in the right main bronchus and had perforated there. Beyond the right main bronchus, the nasogastric tube made a 360° loop and ended in the posteromedial pleural sinus. Computed tomography was performed showing a perforation of the esophagus by the nasogastric tube (arrow in c) whose tip was located in the posterior sinus (arrow in d). At the time of computed tomography, a thoracostomy tube had been placed (asterisks in d) to treat the pneumothorax. Low-power image of hematoxylin and eosin stained biopsy of the left calf e. It shows dermal fibrosis with a greater amount of spindle cells (arrows) than normally encountered in the dermis e. Increased number of factor XIIIa positive dentritic cells, beside fibrocytes f.

Abb. 1 Pneumothorax hervorgerufen durch eine Magensonde und histologische Befunde. a Das initiale Röntgen des Thorax vor Anlage der Magensonde zeigte eine normale rechte Lunge ohne Anzeichen für einen Pneumothorax (Pfeilköpfe) sowie pneumonische Infiltrate im linken Lungen unterlappen. b Pneumothorax (Pfeilköpfe zeigen die Lungengrenzen, Stern weist auf den tiefen Sulcus hin) nach Anlage der Magensonde (Pfeil). Basierend auf dieser bettseitigen Aufnahme wurde eine Fehllage der Magensonde in den rechten Hauptbronchus mit daraus resultierende Perforation der Lunge vermutet. Die Computertomographie zeigte jedoch eine Perforation des Ösophagus durch die Magensonde (Pfeil in c), deren Spitze im posterioren Sinus lokalisiert war (Pfeil in d). Nachfolgend wurde eine Thoraxdrainage rechtsseitig zur Therapie des Pneumothorax eingebracht (Sterne in d). Niedrig aufgelöste Hämatoxylin-Eosin-Färbung der Hautbiopsie am linken Unterschenkel e mit einer dermalen Fibrose und erhöhten Anzahl von Spindelzellen (Pfeile in e) sowie einer erhöhten Anzahl von Faktor XIIIa positiven dentritischen Zellen neben Fibrozyten f.

She was sent to intensive care and an aortic valve endocarditis with a 13*3 mm floating structure was identified on echocardiography as the most likely cause of the sepsis. As a routine procedure, a nasogastric tube was inserted on her second day in the ward. Radiograph showed a new pneumothorax on the right ([Fig. 1b]). Based on the portable chest radiograph, the most likely mechanism was perforation via the right mainstem bronchus, which is also the most common location of nasogastric tube misplacement. To further elucidate this, computed tomography was performed and showed dorsal perforation of the esophagus ([Fig. 1c, d]). The first case of a pneumothorax induced by a nasogastric tube was reported in 1981 (Hollimon P W, McFee AS, Arch Surg 1981; 116: 970.) and no esophageal perforation by a nasogastric tube in adults resulting in pneumothorax has been reported. A thoracostomy tube was placed to treat the pneumothorax. Unfortunately, the patient’s condition worsened further and she passed away 4 weeks after aortic valve replacement.

Which etiology caused a nasogastric tube to be able to perforate the esophagus and led to the pneumothorax? Autopsy showed septic-toxic cardiovascular failure due to bacterial endocarditis of the aortic valve to be the most likely cause of death. At the same time, histopathology was able to demonstrate dermal fibrosis and focal skin indurations. Thus, the clinical and histological data ([Fig. 1e, f]) suggested nephrogenic systemic fibrosis (Cowper SE, Rabach M, Girardi M. Eur J Radiol 2008; 66: 191 – 199) as the basis for perforation.

Nephrogenic systemic fibrosis was first described as “scleromyxedema-like cutaneous diseases” in renal-dialysis patients by Cowper et al. in 2000 (Cowper SE, et al., Lancet 2000; 356: 1000 – 1001). It was later proven by the same group and other investigators that nephrogenic fibrosis is a rare systemic disease with about 335 cases reported so far (www.icnsfr.org) that also affects the liver, lungs, heart, esophagus, and musculature. The development is triggered by a release of Gd³+, starts with the accumulation of Gd³+ in the skin, probably ameliorated by circulating fibrocytes. The details of its pathogenesis are not entirely clear and recent experimental data suggest that certain Gd-based contrast agents interfere with the signals that inhibit the differentiation of monocytes from fibrocytes (Vakil V et al., J Magn Reson Imaging 2009; 30: 1284 – 1288).

Typically, clinical signs such as indurations of the skin and histological proof of nephrogenic systemic fibrosis occur years after the intravenous administration of high-doses of Gd-based magnetic resonance contrast agents. The other major risk factor is severe renal impairment with a glomerular filtration rate of below 30 ml/min/1.73 m². Our patient was on chronic renal-dialysis with autosomal polycystic kidney disease, had a GFR of 10 ml/min/1.73 m², and received 5 examinations before kidney transplantation with Gd-based contrast agents in the year 2005, before the FDA and EMEA advised against its use in patients on dialysis in 2007. Further magnetic resonance examinations were done because of post-transplantation complications (perfusion loss of 10 % of the parenchyma at the lower pole due to non-anastomosed caudal renal artery, suspected thrombosis due to  large lymphocele, and acute rejection [Banff IIB]) and led to an accumulated dose of 133 ml Gd-DTPA® that equaled 9 single doses of the Gd-based contrast agent (0.1 mmol/kg/BW). Sadowski et al. reported that one of their 13 NSF patients died of esophageal rupture (without indicating if a gastric tube had been placed) (Sadowski EA et al., Radiology 2007; 243: 148 – 157). It is likely that only a few of the 335 patients with NSF reported so far have received nasogastric tubes and esophageal perforation resulting in pneumothorax has not been reported in adults in the literature. Therefore, it is difficult to assess the extent to which the risk of esophageal perforation is increased with a gastric tube in NSF patients. Nevertheless, it appears cautious and careful to control the intraluminal positioning of nasogastric tubes especially in NSF patients before their use. In summary, since the new strict guidelines on Gd-based contrast agent use in patients with renal dysfunction (Heinrich M, Uder M, Fortschr Röntgenstr 2007; 179: 613 – 617) have resulted in broad acceptance and adherence by institutions and a drop in new cases, we are hoping that such unusual complications as in our patient will have gone the way of the dodo.