Clin J Gastroenterol (2009) 2:22–26 DOI 10.1007/s12328-008-0038-8
CASE REPORT
A lethal case of pneumatosis intestinalis complicated by small bowel volvulus Siong-Seng Liau Æ Charlotte Cope Æ Malcolm MacFarlane Æ Neil Keeling
Received: 10 June 2008 / Accepted: 28 August 2008 / Published online: 19 November 2008 Ó Springer 2008
Abstract Pneumatosis intestinalis (PI) is a condition characterised by gas-filled cystic malformations on the intestinal wall. It is often secondary to an underlying disease process. We describe the case of a 76-year-old gentleman who presented with intermittent abdominal pain, altered bowel habits and weight loss. Barium enema was unremarkable, apart from sigmoid diverticulosis. CT scan found evidence of pneumatosis on small bowel walls with benign pneumoperitoneum. As there was no sign of intraabdominal crisis, he was initially treated conservatively. Unfortunately, he required re-admission 6 weeks later with symptoms of bowel obstruction and clinical signs of perotinitis. At laparotomy, he was found to have extensive small bowel infarction due to volvulus from ‘twisting’ around the axis of superior mesenteric vessels. Evidently, this occurred secondary to a congenitally long small bowel mesentery that predisposed him to volvulus. Extensive small bowel resection was performed. The postoperative course was complicated by persistent hypotension, which proved fatal. This case report draws attention to the rare association between PI and small bowel volvulus predisposed by a congenitally long mesentery, which can present initially with a benign picture (intermittent, reversible volvulus), but subsequently be complicated by lethal bowel infarction (irreversible volvulus). This case raises several issues about the management of this rare condition. Clinicians must realise that PI, though often benign, can S.-S. Liau (&) C. Cope N. Keeling Department of Surgery, West Suffolk Hospital, Bury St Edmunds IP33 2QZ, UK e-mail: [email protected] M. MacFarlane Department of Radiology, West Suffolk Hospital, Bury St Edmunds IP33 2QZ, UK
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present with lethal complications, and early recognition of such complications can be life-saving. Keywords Pneumatosis intestinalis Small bowel volvulus Intestinal ischaemia Surgery Background Pneumatosis intestinalis (PI) is a rare condition characterised by multiple gas-filled cysts in the subserosal or submucosal wall of small or large bowels. Although the aetiology of cystic malformation is largely unknown, a number of factors are thought to contribute to the pathogenesis of PI, including mucosal injury, increased intraluminal pressure and entrance of gas-producing bacteria into the bowel wall [1]. This may occur spontaneously (primary or idiopathic PI, approximately 15%), but in the majority of cases, it is a manifestation of underlying disease (i.e., secondary PI). Primary PI is usually termed pneumatosis cystoides intestinalis. Secondary PI has been associated with systemic conditions, such as infection, drugs (e.g., chemotherapy and immunosuppressants), trauma and connective tissue diseases, but more commonly, it results from gastrointestinal conditions, such as bowel ischaemia, obstruction or inflammatory bowel disease [2]. Given the rarity of this condition, there is no consensus on the management strategy for this condition. Here, we present a case of pneumatosis intestinalis associated with a congenitally long small bowel mesentery that was complicated by small bowel volvulus, which proved fatal. Case presentation A 76-year-old man was referred to the colorectal clinic with a 1-year history of altered bowel habit, abdominal
Clin J Gastroenterol (2009) 2:22–26
distension, low abdominal pain and weight loss. He complained of increased bowel frequency with loose stools and a mucus discharge, but no rectal bleeding. He was known to be hypertensive and had a past history of prostatic carcinoma treated with radiotherapy. Clinical examination revealed a slightly distended abdomen that was soft and minimally tender in the lower abdomen on deep palpation. Rigid sigmoidoscopy was unremarkable. Barium enema was normal apart from mild sigmoid diverticulosis. He subsequently underwent a computed tomographic (CT) scan of the abdomen and pelvis. Interestingly, this revealed the presence of pneumoperitoneum and identified gas-filled cysts on the wall of small bowel loops, suggestive of pneumatosis intestinalis (Fig. 1). No obvious cause for these abnormalities was detected on the scan. Given these unusual findings, the patient was admitted directly to a surgical ward following his CT scan. He reported no new symptoms, and careful examination revealed a soft abdomen with no perotinism. His laboratory results were unremarkable. In the absence of any indication of intraabdominal crisis, he was discharged with an outpatient follow-up arrangement. Six weeks later, just prior to the outpatient follow-up, he presented to the Emergency Department with an 8-h history of sudden onset, central abdominal pain, distension and vomiting. Clinically, he had signs of generalised perotinitis. Blood tests revealed a markedly raised white cell count of 21.5 9 109/l and C-reactive protein of 3 mg/l. His other blood results were: haemoglobin 15.4 g/dl, platelets 332 9 109/l, urea 7.9 lmol/l, creatinine 111 lmol/l, liver function tests of bilirubin 7 lmol/l, alkaline phosphatase 204 U/l, alanine transaminase 20 U/l and normal amylase of 30 U/l. Arterial blood gas analysis revealed significant metabolic acidosis (pH 7.23, bicarbonate 15.7 mmol/l and base excess of -11.3 mmol/l), with normal pO2 at 11.9 kPa and pCO2 at 5.10 kPa. Chest and abdominal plain radiography revealed massive subdiaphragmatic air and multiple dilated loops of small bowel with well-demarcated wall indicating a positive Wriggler’s sign (Fig. 2). Given that the patient was clinically unwell with perotinitis, the impression was of an intra-abdominal crisis, either due to perforation or acute obstruction. Urgent exploratory laparotomy was therefore performed. Intraoperatively, there was extensive small bowel infarction due to volvulus of most of the small bowels, involving a twoand-a-half clockwise turn around a long, pendulous small bowel mesentery, which twisted and occluded the superior mesenteric vessels (Fig. 3). There was some evidence of blood-stained ascites. In addition, multiple gas-filled subserosal cysts on the wall of ileum were encountered. Despite ‘untwisting’ of the volvulus, the bowel ischaemia was clearly irreversible. The untwisted small bowel mesentery was fan-shaped with the longest segment measuring at
23
Fig. 1 CT scans of the patient during the initial presentation. a Coronal CT scan of the abdomen showing gaseous cysts in the subserosal wall of small intestine. b Axial CT scan of the abdomen showing pneumatosis intestinalis and pneumoperitoneum using the lung window. Gaseous subserosal cysts are indicated by arrows
least 30 cm from its root, though the mesenteric attachment to the retroperitoneum was not particularly narrow. Hence, extensive small bowel resection was performed with a jejuno-ileal anastomosis (approximately 60 cm of remnant small bowel). The entire colon was normal. The patient was transferred to the intensive care unit postoperatively where he was persistently hypotensive and suffered two cardiac arrests from which he was successfully resuscitated. Unfortunately his condition continued to deteriorate.
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24
Fig. 2 Plain radiography performed during the subsequent acute presentation. a Chest radiograph showing massive pneumoperitoneum under both diaphragms (indicated by arrows). b Abdominal radiograph demonstrating distended loops of small bowels, with positive Wriggler’s sign confirming free intraperitoneal gas (indicated by arrows)
Following discussion with his family, it was agreed that active treatment should be withdrawn, and he died shortly afterwards. In retrospect, this adverse outcome was most probably due to the ischaemic bowel necrosis, which led to significant fluid imbalance, electrolyte derangement and overwhelming sepsis.
Discussion Pneumatosis intestinalis was first described in 1783 by DuVernoi [3] during cadaveric examination. From the
123
Clin J Gastroenterol (2009) 2:22–26
surgical standpoint, PI is a radiological finding with its significance very much dependent on the underlying pathologic process. In particular, PI is known to be associated or complicated by volvulus of the various regions of the gastrointestinal tract, including the midgut [4], caecal [5], sigmoid [6] and gastric volvulus [7]. These conditions are important to recognise as they may require operative treatment. To the authors’ knowledge, there has been only a single published report of pneumatosis intestinalis associated with small bowel volvulus [8]. This differed from midgut volvulus as it occurred in the absence of malrotation and involved only the small bowels with no caecal or large bowel involvement. In the current report, we described an interesting case of PI in which the patient was initially found to have benign pneumoperitoneum, but subsequently re-presented with small bowel volvulus (around the axis of superior mesenteric vessels) secondary to a congenitally long mesentery. The initial recognition of benign pneumoperitoneum in the current case led to the non-operative management. The initial CT scan did not reveal any evidence of a cause for the development of PI in the small bowels. In the subsequent acute presentation, clinical perotinitis in a case of previously known pneumatosis intestinalis warranted an urgent laparotomy. We speculate that the intermittent ‘twisting’ and ‘untwisting’ of the long small bowel mesentery (reversible volvulus) led to the patient’s intermittent symptoms and relative lack of clinical signs during the initial presentation. Given that ischaemia is an aetiological factor for development of PI [1], the intermittent, reversible ischaemia from reversible volvulus probably led to development of PI. The development of bulbous pneumatosis cysts on the small bowel wall together with the congenitally long and redundant mesentery provided the combination of mechanical factors that led to the lethal volvulus (irreversible volvulus). This torsion process could be likened to the ‘twisting of a bunch of grapes (pneumatosis cysts on the wall of small bowels) on a long stem (long mesentery)’. The torsion of pendulous mesentery finally led to irreversible volvulus, occlusion of the superior mesenteric vessels, infarction and perotinitis, which accounted for the final presentation. In the current case, although we have provided a plausible mechanism for the disease process linking PI, long mesentery and volvulus, it remains unknown which of the processes come first. The association of a long bowel mesentery and volvulus has been well described. Classically, a long mesocolon is a pre-condition for colonic volvulus, especially in the sigmoid colon [9, 10]. However, the association between a long mesentery and small bowel volvulus is less well described, but not without precedent. Coder et al. [11] have described a case of small bowel volvulus resulting from a congenitally long mesentery.
Clin J Gastroenterol (2009) 2:22–26
25
Fig. 3 Diagram illustrating the intraoperative findings of small bowel volvulus with extensive infarction secondary to a twist of mesentery along the axis of the superior mesenteric vessels. The predisposing factor to volvulus was thought to be a congenitally long small bowel mesentery, possibly in combination with the gaseous cysts on the bowel wall. Of note, the large bowel wall was normal with no evidence of pneumatosis coli
Furthermore, a long small bowel mesentery is a predisposing factor for an ileosigmoid knot, a variant of mid-gut volvulus [12]. Our speculation that the presence of pneumatosis cysts serves as a further mechanical factor that exacerbates volvulus is supported by previously published reports. Moote et al. found that in patients with sigmoid pneumatosis, there is a high incidence of a long sigmoid colon and mesentery. It is argued that pneumatosis cysts and a long, redundant sigmoid colon or mesentery contribute to the development of volvulus. In addition, Adomnicai et al. [8] has reported a case in which PI was thought to be the cause of small bowel volvulus. A recent article by Brill et al. [13] has described a case of PI managed successfully by non-operative measures as the patient did not have any sign of perotinism. Importantly, benign pneumoperitoneum from PI (presumably due to rupture of gaseous cysts without communication with GI tract lumen) in the absence of perotinitis can be treated conservatively. We agree with Brill and colleagues that all patients with pneumatosis intestinalis presenting with abdominal pain should be investigated by an urgent CT scan unless the patient is perotinitic, in which case a laparotomy is indicated. A CT scan can aid in the early detection of complications of PI, such as small bowel volvulus, which can be lethal if detected late. Characteristic CT findings of small bowel volvulus include the ‘whirl’ sign of the rotated mesentery and ‘peacock’s tail’ sign due to torsion of the bowel around the mesenteric axis [14]. In addition, the clinician should have a low index of suspicion as early detection of intra-abdominal mischief in cases of PI can avert an unfavourable outcome. If the current patient had presented earlier during the second episode, a simple
manoeuvre of ‘untwisting’ of the mesentery at laparotomy could have been life-saving and potentially curative, at least in the short term. While there are no agreed-upon guidelines for the investigation and management of patients with an incidental radiological finding of pneumatosis intestinalis, it is prudent to assume that PI is often secondary to an underlying disease. Hence, it is important to identify any potential causes, with CT scan being the best imaging modality as it provides the surgeon with an excellent survey for an underlying intra-abdominal cause or other associated pathological conditions. Lastly, short-term follow-up with CT scan may be appropriate for patients with incidental findings of pneumatosis intestinalis.
Conclusion This case report highlights the importance of clinicians recognising this rare association between pneumatosis intestinalis and small bowel volvulus secondary to a long, redundant mesentery. The importance of early recognition is that surgical intervention can be performed before irreversible bowel ischaemia sets in.
References 1. Pear BL. Pneumatosis intestinalis: a review. Radiology. 1998;207(1):13–9. 2. St Peter SD, Abbas MA, Kelly KA. The spectrum of pneumatosis intestinalis. Arch Surg. 2003;138(1):68–75.
123
26 3. DuVernoi J. Anatomische Beobachtungen der unter der aeusseren und inneren Haut der Gedaerme eingeschlossenen Luft. Phys Med Abhandl Acad Wissensch Petersburg. 1783;2:182. 4. Yang CJ, Lu CH, Murakami J, Franken EA Jr. General case of the day. Midgut volvulus with necrotic small bowel. Radiographics. 1987;7(3):605–7. 5. Lawrentschuk N, Nguyen H. Pneumatosis coli in a case of caecal volvulus. Hosp Med. 2002;63(7):436–7. 6. Gillon J, Holt S, Sircus W. Pneumatosis coli and sigmoid volvulus: a report of four cases. Br J Surg. 1979;66(11):802–5. 7. Elidan J, Gimmon Z, Schwartz A. Pneumoperitoneum induced by pneumatosis cystoides intestinalis associated with volvulus of the stomach. Am J Gastroenterol. 1980;74(2):189–95. 8. Adomnicai G, Mihalache S, Ioan M, Mihalache G, Boureanu C. A case of volvulus of the small intestine caused by cystic pneumatosis. Rev Med Chir Soc Med Nat Iasi. 1967;71(4):1017–20.
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Clin J Gastroenterol (2009) 2:22–26 9. Pannell M, Byard RW. Sigmoid volvulus and unexpected death in the elderly. J Clin Forensic Med. 2001;8(4):228–30. 10. Grodsinsky C, Ponka JL. Volvulus of the colon. Dis Colon Rectum. 1977;20(4):314–24. 11. Coder DM, Schewitz LJ, Falls HC. Midgut volvulus following cesarean section. Obstet Gynecol. 1976;47(2):231–3. 12. VerSteeg KR, Whitehead WA. Ileosigmoid knot. Arch Surg. 1980;115(6):761–3. 13. Brill SE, Skipworth J, Stoker DL. Conservative management of pneumatosis intestinalis and massive pneumoperitoneum in the acute abdomen: a case report. Ann R Coll Surg Engl. 2008;90(2): W11–3. 14. de Korte N, Grutters CT, Snellen JP. Small bowel volvulus diagnosed by the CT ‘‘whirl sign’’. J Gastrointest Surg. 2008;12: 1469–70.
CASE REPORT
A lethal case of pneumatosis intestinalis complicated by small bowel volvulus Siong-Seng Liau Æ Charlotte Cope Æ Malcolm MacFarlane Æ Neil Keeling
Received: 10 June 2008 / Accepted: 28 August 2008 / Published online: 19 November 2008 Ó Springer 2008
Abstract Pneumatosis intestinalis (PI) is a condition characterised by gas-filled cystic malformations on the intestinal wall. It is often secondary to an underlying disease process. We describe the case of a 76-year-old gentleman who presented with intermittent abdominal pain, altered bowel habits and weight loss. Barium enema was unremarkable, apart from sigmoid diverticulosis. CT scan found evidence of pneumatosis on small bowel walls with benign pneumoperitoneum. As there was no sign of intraabdominal crisis, he was initially treated conservatively. Unfortunately, he required re-admission 6 weeks later with symptoms of bowel obstruction and clinical signs of perotinitis. At laparotomy, he was found to have extensive small bowel infarction due to volvulus from ‘twisting’ around the axis of superior mesenteric vessels. Evidently, this occurred secondary to a congenitally long small bowel mesentery that predisposed him to volvulus. Extensive small bowel resection was performed. The postoperative course was complicated by persistent hypotension, which proved fatal. This case report draws attention to the rare association between PI and small bowel volvulus predisposed by a congenitally long mesentery, which can present initially with a benign picture (intermittent, reversible volvulus), but subsequently be complicated by lethal bowel infarction (irreversible volvulus). This case raises several issues about the management of this rare condition. Clinicians must realise that PI, though often benign, can S.-S. Liau (&) C. Cope N. Keeling Department of Surgery, West Suffolk Hospital, Bury St Edmunds IP33 2QZ, UK e-mail: [email protected] M. MacFarlane Department of Radiology, West Suffolk Hospital, Bury St Edmunds IP33 2QZ, UK
123
present with lethal complications, and early recognition of such complications can be life-saving. Keywords Pneumatosis intestinalis Small bowel volvulus Intestinal ischaemia Surgery Background Pneumatosis intestinalis (PI) is a rare condition characterised by multiple gas-filled cysts in the subserosal or submucosal wall of small or large bowels. Although the aetiology of cystic malformation is largely unknown, a number of factors are thought to contribute to the pathogenesis of PI, including mucosal injury, increased intraluminal pressure and entrance of gas-producing bacteria into the bowel wall [1]. This may occur spontaneously (primary or idiopathic PI, approximately 15%), but in the majority of cases, it is a manifestation of underlying disease (i.e., secondary PI). Primary PI is usually termed pneumatosis cystoides intestinalis. Secondary PI has been associated with systemic conditions, such as infection, drugs (e.g., chemotherapy and immunosuppressants), trauma and connective tissue diseases, but more commonly, it results from gastrointestinal conditions, such as bowel ischaemia, obstruction or inflammatory bowel disease [2]. Given the rarity of this condition, there is no consensus on the management strategy for this condition. Here, we present a case of pneumatosis intestinalis associated with a congenitally long small bowel mesentery that was complicated by small bowel volvulus, which proved fatal. Case presentation A 76-year-old man was referred to the colorectal clinic with a 1-year history of altered bowel habit, abdominal
Clin J Gastroenterol (2009) 2:22–26
distension, low abdominal pain and weight loss. He complained of increased bowel frequency with loose stools and a mucus discharge, but no rectal bleeding. He was known to be hypertensive and had a past history of prostatic carcinoma treated with radiotherapy. Clinical examination revealed a slightly distended abdomen that was soft and minimally tender in the lower abdomen on deep palpation. Rigid sigmoidoscopy was unremarkable. Barium enema was normal apart from mild sigmoid diverticulosis. He subsequently underwent a computed tomographic (CT) scan of the abdomen and pelvis. Interestingly, this revealed the presence of pneumoperitoneum and identified gas-filled cysts on the wall of small bowel loops, suggestive of pneumatosis intestinalis (Fig. 1). No obvious cause for these abnormalities was detected on the scan. Given these unusual findings, the patient was admitted directly to a surgical ward following his CT scan. He reported no new symptoms, and careful examination revealed a soft abdomen with no perotinism. His laboratory results were unremarkable. In the absence of any indication of intraabdominal crisis, he was discharged with an outpatient follow-up arrangement. Six weeks later, just prior to the outpatient follow-up, he presented to the Emergency Department with an 8-h history of sudden onset, central abdominal pain, distension and vomiting. Clinically, he had signs of generalised perotinitis. Blood tests revealed a markedly raised white cell count of 21.5 9 109/l and C-reactive protein of 3 mg/l. His other blood results were: haemoglobin 15.4 g/dl, platelets 332 9 109/l, urea 7.9 lmol/l, creatinine 111 lmol/l, liver function tests of bilirubin 7 lmol/l, alkaline phosphatase 204 U/l, alanine transaminase 20 U/l and normal amylase of 30 U/l. Arterial blood gas analysis revealed significant metabolic acidosis (pH 7.23, bicarbonate 15.7 mmol/l and base excess of -11.3 mmol/l), with normal pO2 at 11.9 kPa and pCO2 at 5.10 kPa. Chest and abdominal plain radiography revealed massive subdiaphragmatic air and multiple dilated loops of small bowel with well-demarcated wall indicating a positive Wriggler’s sign (Fig. 2). Given that the patient was clinically unwell with perotinitis, the impression was of an intra-abdominal crisis, either due to perforation or acute obstruction. Urgent exploratory laparotomy was therefore performed. Intraoperatively, there was extensive small bowel infarction due to volvulus of most of the small bowels, involving a twoand-a-half clockwise turn around a long, pendulous small bowel mesentery, which twisted and occluded the superior mesenteric vessels (Fig. 3). There was some evidence of blood-stained ascites. In addition, multiple gas-filled subserosal cysts on the wall of ileum were encountered. Despite ‘untwisting’ of the volvulus, the bowel ischaemia was clearly irreversible. The untwisted small bowel mesentery was fan-shaped with the longest segment measuring at
23
Fig. 1 CT scans of the patient during the initial presentation. a Coronal CT scan of the abdomen showing gaseous cysts in the subserosal wall of small intestine. b Axial CT scan of the abdomen showing pneumatosis intestinalis and pneumoperitoneum using the lung window. Gaseous subserosal cysts are indicated by arrows
least 30 cm from its root, though the mesenteric attachment to the retroperitoneum was not particularly narrow. Hence, extensive small bowel resection was performed with a jejuno-ileal anastomosis (approximately 60 cm of remnant small bowel). The entire colon was normal. The patient was transferred to the intensive care unit postoperatively where he was persistently hypotensive and suffered two cardiac arrests from which he was successfully resuscitated. Unfortunately his condition continued to deteriorate.
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24
Fig. 2 Plain radiography performed during the subsequent acute presentation. a Chest radiograph showing massive pneumoperitoneum under both diaphragms (indicated by arrows). b Abdominal radiograph demonstrating distended loops of small bowels, with positive Wriggler’s sign confirming free intraperitoneal gas (indicated by arrows)
Following discussion with his family, it was agreed that active treatment should be withdrawn, and he died shortly afterwards. In retrospect, this adverse outcome was most probably due to the ischaemic bowel necrosis, which led to significant fluid imbalance, electrolyte derangement and overwhelming sepsis.
Discussion Pneumatosis intestinalis was first described in 1783 by DuVernoi [3] during cadaveric examination. From the
123
Clin J Gastroenterol (2009) 2:22–26
surgical standpoint, PI is a radiological finding with its significance very much dependent on the underlying pathologic process. In particular, PI is known to be associated or complicated by volvulus of the various regions of the gastrointestinal tract, including the midgut [4], caecal [5], sigmoid [6] and gastric volvulus [7]. These conditions are important to recognise as they may require operative treatment. To the authors’ knowledge, there has been only a single published report of pneumatosis intestinalis associated with small bowel volvulus [8]. This differed from midgut volvulus as it occurred in the absence of malrotation and involved only the small bowels with no caecal or large bowel involvement. In the current report, we described an interesting case of PI in which the patient was initially found to have benign pneumoperitoneum, but subsequently re-presented with small bowel volvulus (around the axis of superior mesenteric vessels) secondary to a congenitally long mesentery. The initial recognition of benign pneumoperitoneum in the current case led to the non-operative management. The initial CT scan did not reveal any evidence of a cause for the development of PI in the small bowels. In the subsequent acute presentation, clinical perotinitis in a case of previously known pneumatosis intestinalis warranted an urgent laparotomy. We speculate that the intermittent ‘twisting’ and ‘untwisting’ of the long small bowel mesentery (reversible volvulus) led to the patient’s intermittent symptoms and relative lack of clinical signs during the initial presentation. Given that ischaemia is an aetiological factor for development of PI [1], the intermittent, reversible ischaemia from reversible volvulus probably led to development of PI. The development of bulbous pneumatosis cysts on the small bowel wall together with the congenitally long and redundant mesentery provided the combination of mechanical factors that led to the lethal volvulus (irreversible volvulus). This torsion process could be likened to the ‘twisting of a bunch of grapes (pneumatosis cysts on the wall of small bowels) on a long stem (long mesentery)’. The torsion of pendulous mesentery finally led to irreversible volvulus, occlusion of the superior mesenteric vessels, infarction and perotinitis, which accounted for the final presentation. In the current case, although we have provided a plausible mechanism for the disease process linking PI, long mesentery and volvulus, it remains unknown which of the processes come first. The association of a long bowel mesentery and volvulus has been well described. Classically, a long mesocolon is a pre-condition for colonic volvulus, especially in the sigmoid colon [9, 10]. However, the association between a long mesentery and small bowel volvulus is less well described, but not without precedent. Coder et al. [11] have described a case of small bowel volvulus resulting from a congenitally long mesentery.
Clin J Gastroenterol (2009) 2:22–26
25
Fig. 3 Diagram illustrating the intraoperative findings of small bowel volvulus with extensive infarction secondary to a twist of mesentery along the axis of the superior mesenteric vessels. The predisposing factor to volvulus was thought to be a congenitally long small bowel mesentery, possibly in combination with the gaseous cysts on the bowel wall. Of note, the large bowel wall was normal with no evidence of pneumatosis coli
Furthermore, a long small bowel mesentery is a predisposing factor for an ileosigmoid knot, a variant of mid-gut volvulus [12]. Our speculation that the presence of pneumatosis cysts serves as a further mechanical factor that exacerbates volvulus is supported by previously published reports. Moote et al. found that in patients with sigmoid pneumatosis, there is a high incidence of a long sigmoid colon and mesentery. It is argued that pneumatosis cysts and a long, redundant sigmoid colon or mesentery contribute to the development of volvulus. In addition, Adomnicai et al. [8] has reported a case in which PI was thought to be the cause of small bowel volvulus. A recent article by Brill et al. [13] has described a case of PI managed successfully by non-operative measures as the patient did not have any sign of perotinism. Importantly, benign pneumoperitoneum from PI (presumably due to rupture of gaseous cysts without communication with GI tract lumen) in the absence of perotinitis can be treated conservatively. We agree with Brill and colleagues that all patients with pneumatosis intestinalis presenting with abdominal pain should be investigated by an urgent CT scan unless the patient is perotinitic, in which case a laparotomy is indicated. A CT scan can aid in the early detection of complications of PI, such as small bowel volvulus, which can be lethal if detected late. Characteristic CT findings of small bowel volvulus include the ‘whirl’ sign of the rotated mesentery and ‘peacock’s tail’ sign due to torsion of the bowel around the mesenteric axis [14]. In addition, the clinician should have a low index of suspicion as early detection of intra-abdominal mischief in cases of PI can avert an unfavourable outcome. If the current patient had presented earlier during the second episode, a simple
manoeuvre of ‘untwisting’ of the mesentery at laparotomy could have been life-saving and potentially curative, at least in the short term. While there are no agreed-upon guidelines for the investigation and management of patients with an incidental radiological finding of pneumatosis intestinalis, it is prudent to assume that PI is often secondary to an underlying disease. Hence, it is important to identify any potential causes, with CT scan being the best imaging modality as it provides the surgeon with an excellent survey for an underlying intra-abdominal cause or other associated pathological conditions. Lastly, short-term follow-up with CT scan may be appropriate for patients with incidental findings of pneumatosis intestinalis.
Conclusion This case report highlights the importance of clinicians recognising this rare association between pneumatosis intestinalis and small bowel volvulus secondary to a long, redundant mesentery. The importance of early recognition is that surgical intervention can be performed before irreversible bowel ischaemia sets in.
References 1. Pear BL. Pneumatosis intestinalis: a review. Radiology. 1998;207(1):13–9. 2. St Peter SD, Abbas MA, Kelly KA. The spectrum of pneumatosis intestinalis. Arch Surg. 2003;138(1):68–75.
123
26 3. DuVernoi J. Anatomische Beobachtungen der unter der aeusseren und inneren Haut der Gedaerme eingeschlossenen Luft. Phys Med Abhandl Acad Wissensch Petersburg. 1783;2:182. 4. Yang CJ, Lu CH, Murakami J, Franken EA Jr. General case of the day. Midgut volvulus with necrotic small bowel. Radiographics. 1987;7(3):605–7. 5. Lawrentschuk N, Nguyen H. Pneumatosis coli in a case of caecal volvulus. Hosp Med. 2002;63(7):436–7. 6. Gillon J, Holt S, Sircus W. Pneumatosis coli and sigmoid volvulus: a report of four cases. Br J Surg. 1979;66(11):802–5. 7. Elidan J, Gimmon Z, Schwartz A. Pneumoperitoneum induced by pneumatosis cystoides intestinalis associated with volvulus of the stomach. Am J Gastroenterol. 1980;74(2):189–95. 8. Adomnicai G, Mihalache S, Ioan M, Mihalache G, Boureanu C. A case of volvulus of the small intestine caused by cystic pneumatosis. Rev Med Chir Soc Med Nat Iasi. 1967;71(4):1017–20.
123
Clin J Gastroenterol (2009) 2:22–26 9. Pannell M, Byard RW. Sigmoid volvulus and unexpected death in the elderly. J Clin Forensic Med. 2001;8(4):228–30. 10. Grodsinsky C, Ponka JL. Volvulus of the colon. Dis Colon Rectum. 1977;20(4):314–24. 11. Coder DM, Schewitz LJ, Falls HC. Midgut volvulus following cesarean section. Obstet Gynecol. 1976;47(2):231–3. 12. VerSteeg KR, Whitehead WA. Ileosigmoid knot. Arch Surg. 1980;115(6):761–3. 13. Brill SE, Skipworth J, Stoker DL. Conservative management of pneumatosis intestinalis and massive pneumoperitoneum in the acute abdomen: a case report. Ann R Coll Surg Engl. 2008;90(2): W11–3. 14. de Korte N, Grutters CT, Snellen JP. Small bowel volvulus diagnosed by the CT ‘‘whirl sign’’. J Gastrointest Surg. 2008;12: 1469–70.
