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Midgut volvulus as initial presentation of pneumatosis cystoides intestinalis Pneumatosis cystoides intestinalis (PCI) is a rare disease with unknown aetiology. It is characterized by the presence of gas-filled cysts within the submucosa or subserosa of the intestine; however, it can be located in any part of the intestinal tract.1 The pathogenesis, although unknown, seems to be related to the mucosal breakdown and bacterial fermentation. PCI is associated with a variety of diseases in which there is loss of bowel mucosal integrity and/or increased intraluminal pressure. PCI is usually incidentally discovered during diagnostic modalities being carried out for other reasons. In approximately 16% of cases, PCI presents as intestinal obstruction or perforation.2 Patients commonly present with non-specific gastrointestinal symptoms such as intermittent abdominal pain, nausea, and in cases with colonic involvement, diarrhoea, mucus discharge, rectal bleeding and constipation.3 Although previously PCI was thought to occur more frequently in the small intestine, more recent studies have shown that PCI appears more frequently in the colon (61.8%), followed by the small intestine (15.4%).4 A 72-year-old woman presented to the emergency department with a 3-week history of severe back pain with radiation to the abdomen and now recent onset of vomiting. On examination, the vital signs were normal and the abdomen was soft with only minimal tenderness without any peritoneal signs. Laboratory values were also within normal limits. An initial computed tomography (CT) scan without contrast was read as free intra-abdominal air under the right hemidiaphragm (Fig. 1). Because of the overall paucity of clinical
Fig. 1. An initial computed tomography scan of the abdomen and pelvis obtained without contrast revealed a few dots of free air and what appeared to be a larger pocket of gas in the right upper quadrant.
© 2015 Royal Australasian College of Surgeons
signs, a CT with contrast was obtained. The CT scan with oral contrast revealed a large duodenum diverticulum in the right upper quadrant and suggested a possible internal hernia; small pockets of free air were again visualized (Fig. 2). At this point, the patient was taken to the operating room for diagnostic laparoscopy. During laparoscopic exploration, at a distance of about 40 cm from the ligament of Treitz, the small bowel was twisting on itself going under the superior mesenteric artery. The internal hernia was reduced by gentle traction. Once this was accomplished, it became evident that a segment of bowel that was folding on itself contained multiple diverticula. There was no evidence of inflammatory changes, but there appeared to be air under the layer of visceral peritoneum. Since those diverticula had functioned as a lead point for the torsion, the segment of bowel was resected (35 cm) (Fig. 3) with a linear stapler. A standard side-to-side functional end-to-end anastomosis was performed. She was discharged home on postoperative day 4 tolerating regular diet. The pathology report revealed the following: small bowel segment with diverticular disease and serosal adhesions. Histological changes were consistent with arteriovascular malformation and severe atherosclerosis. Seven reactive lymph nodes were negative for neoplasm. In this unusual case report, the clinical presentation was of a volvulus around the superior mesenteric artery, likely secondary to the bulky PCI in the jejunum. To our knowledge, this is the first description of such a presentation of PCI. This underlies the importance of a complete clinical evaluation that does not only take into account the radiological findings. The initial CT scan in our case
Fig. 2. Computed tomography scan with contrast revealing a ‘swirl’ sign (arrow) at the site of the volvulus.
ANZ J Surg •• (2015) ••–••
2
Images for surgeons
bacterial overgrowth.9 Surgical treatment is reserved for patients with obstruction, ischaemia, perforation or associated tumour. As an uncommon pathology, this should be considered in cases of patients that present with free air without a clear aetiology. In conclusion, PCI remains a rare condition. Although mostly asymptomatic and incidentally discovered, it can cause a wide range of symptoms. A laparoscopic approach can be implemented with good results.
References
Fig. 3. Gross image of the resected small bowel segment causing the volvulus. Note the multiple subserosal gas-filled cysts typical of pneumatosis cystoides intestinalis.
reported a large pocket of free air and small bowel obstruction, which, during the operation, was ultimately determined to be a large cyst from the PCI. Our patient underwent an uneventful diagnostic laparoscopy, reduction of internal hernia and small bowel resection. Typically, the management of pneumoperitoneum is straightforward with immediate surgical exploration.5,6 However, in cases of PCI, this is not always the initial approach, and management should be tailored depending upon the patient’s accompanying symptoms and clinical condition. Alternative non-surgical therapies can also be considered.7,8 This includes antibiotics, octreotide, bowel rest, high flow of oxygen and hyperbaric oxygen therapy. The purpose of the antibiotics is to decrease the bacterial overgrowth, thought to be an essential component of the pathogenesis of PCI. Less defined is the role of prokinetic agents, thought to decrease stasis and additional
1. Korhonen K, Lovvorn HN, Koyama T et al. Incidence, risk factors, and outcome of pneumatosis intestinalis in pediatric stem cell transplant recipients. Pediatr. Blood Cancer 2012; 58: 616–20. 2. Wu LL, Yang Y, Dou Y, Liu QS. A systematic analysis of pneumatosis cystoids intestinalis. World J. Gastroenterol. 2013; 19: 4973–8. 3. Gagliardi G, Thompson IW, Hershman MJ, Forbes A, Hawley PR, Talbot IC. Pneumatosis coli: a proposed pathogenesis based on study of 25 cases and review of the literature. Int. J. Colorectal Dis. 1996; 11: 111–8. 4. Horiuchi A, Akamatsu T, Mukawa K, Ochi Y, Arakura N, Kiyosawa K. Review case report: pneumatosis cystoides intestinalis associated with post-surgical bowel anastomosis: a report of three cases and review of the Japanese literature. J. Gastroenterol. Hepatol. 1998; 13: 534–7. 5. Zhang H, Jun SL, Brennan TV. Pneumatosis intestinalis: not always a surgical indication. Case Rep. Surg. 2012; 2012: 719713. 6. Morris MS, Gee AC, Cho SD et al. Management and outcome of pneumatosis intestinalis. Am. J. Surg. 2008; 195: 679–82. 7. Voboril R. Pneumatosis cystoides intestinalis – a review. Acta Medica (Hradec Kralove) 2001; 44: 89–92. 8. Lim CXQ, Tan WJH, Goh BKP. Benign pneumatosis intestinales. Clin. Gastroenterol. Hepatol. 2014; 12: A25–6. 9. McCarville MB, Whittle SB, Goodin GS et al. Clinical and CT features of benign pneumatosis intestinalis in pediatric hematopoietic stem cell transplant and oncology patients. Pediatr. Radiol. 2008; 38: 1074–83.
Alex Ordonez, MD Fernando Dip, MD David Nguyen, MD Emanuele Lo Menzo, MD, PhD, FACS Szomstein Samuel, MD, FACS Raul Rosenthal, MD, FACS The Bariatric and Metabolic Institute, Section of Minimally Invasive Surgery, Department of General and Vascular Surgery, Cleveland Clinic Florida, Weston, Florida, USA doi: 10.1111/ans.13022
© 2015 Royal Australasian College of Surgeons
Midgut volvulus as initial presentation of pneumatosis cystoides intestinalis Pneumatosis cystoides intestinalis (PCI) is a rare disease with unknown aetiology. It is characterized by the presence of gas-filled cysts within the submucosa or subserosa of the intestine; however, it can be located in any part of the intestinal tract.1 The pathogenesis, although unknown, seems to be related to the mucosal breakdown and bacterial fermentation. PCI is associated with a variety of diseases in which there is loss of bowel mucosal integrity and/or increased intraluminal pressure. PCI is usually incidentally discovered during diagnostic modalities being carried out for other reasons. In approximately 16% of cases, PCI presents as intestinal obstruction or perforation.2 Patients commonly present with non-specific gastrointestinal symptoms such as intermittent abdominal pain, nausea, and in cases with colonic involvement, diarrhoea, mucus discharge, rectal bleeding and constipation.3 Although previously PCI was thought to occur more frequently in the small intestine, more recent studies have shown that PCI appears more frequently in the colon (61.8%), followed by the small intestine (15.4%).4 A 72-year-old woman presented to the emergency department with a 3-week history of severe back pain with radiation to the abdomen and now recent onset of vomiting. On examination, the vital signs were normal and the abdomen was soft with only minimal tenderness without any peritoneal signs. Laboratory values were also within normal limits. An initial computed tomography (CT) scan without contrast was read as free intra-abdominal air under the right hemidiaphragm (Fig. 1). Because of the overall paucity of clinical
Fig. 1. An initial computed tomography scan of the abdomen and pelvis obtained without contrast revealed a few dots of free air and what appeared to be a larger pocket of gas in the right upper quadrant.
© 2015 Royal Australasian College of Surgeons
signs, a CT with contrast was obtained. The CT scan with oral contrast revealed a large duodenum diverticulum in the right upper quadrant and suggested a possible internal hernia; small pockets of free air were again visualized (Fig. 2). At this point, the patient was taken to the operating room for diagnostic laparoscopy. During laparoscopic exploration, at a distance of about 40 cm from the ligament of Treitz, the small bowel was twisting on itself going under the superior mesenteric artery. The internal hernia was reduced by gentle traction. Once this was accomplished, it became evident that a segment of bowel that was folding on itself contained multiple diverticula. There was no evidence of inflammatory changes, but there appeared to be air under the layer of visceral peritoneum. Since those diverticula had functioned as a lead point for the torsion, the segment of bowel was resected (35 cm) (Fig. 3) with a linear stapler. A standard side-to-side functional end-to-end anastomosis was performed. She was discharged home on postoperative day 4 tolerating regular diet. The pathology report revealed the following: small bowel segment with diverticular disease and serosal adhesions. Histological changes were consistent with arteriovascular malformation and severe atherosclerosis. Seven reactive lymph nodes were negative for neoplasm. In this unusual case report, the clinical presentation was of a volvulus around the superior mesenteric artery, likely secondary to the bulky PCI in the jejunum. To our knowledge, this is the first description of such a presentation of PCI. This underlies the importance of a complete clinical evaluation that does not only take into account the radiological findings. The initial CT scan in our case
Fig. 2. Computed tomography scan with contrast revealing a ‘swirl’ sign (arrow) at the site of the volvulus.
ANZ J Surg •• (2015) ••–••
2
Images for surgeons
bacterial overgrowth.9 Surgical treatment is reserved for patients with obstruction, ischaemia, perforation or associated tumour. As an uncommon pathology, this should be considered in cases of patients that present with free air without a clear aetiology. In conclusion, PCI remains a rare condition. Although mostly asymptomatic and incidentally discovered, it can cause a wide range of symptoms. A laparoscopic approach can be implemented with good results.
References
Fig. 3. Gross image of the resected small bowel segment causing the volvulus. Note the multiple subserosal gas-filled cysts typical of pneumatosis cystoides intestinalis.
reported a large pocket of free air and small bowel obstruction, which, during the operation, was ultimately determined to be a large cyst from the PCI. Our patient underwent an uneventful diagnostic laparoscopy, reduction of internal hernia and small bowel resection. Typically, the management of pneumoperitoneum is straightforward with immediate surgical exploration.5,6 However, in cases of PCI, this is not always the initial approach, and management should be tailored depending upon the patient’s accompanying symptoms and clinical condition. Alternative non-surgical therapies can also be considered.7,8 This includes antibiotics, octreotide, bowel rest, high flow of oxygen and hyperbaric oxygen therapy. The purpose of the antibiotics is to decrease the bacterial overgrowth, thought to be an essential component of the pathogenesis of PCI. Less defined is the role of prokinetic agents, thought to decrease stasis and additional
1. Korhonen K, Lovvorn HN, Koyama T et al. Incidence, risk factors, and outcome of pneumatosis intestinalis in pediatric stem cell transplant recipients. Pediatr. Blood Cancer 2012; 58: 616–20. 2. Wu LL, Yang Y, Dou Y, Liu QS. A systematic analysis of pneumatosis cystoids intestinalis. World J. Gastroenterol. 2013; 19: 4973–8. 3. Gagliardi G, Thompson IW, Hershman MJ, Forbes A, Hawley PR, Talbot IC. Pneumatosis coli: a proposed pathogenesis based on study of 25 cases and review of the literature. Int. J. Colorectal Dis. 1996; 11: 111–8. 4. Horiuchi A, Akamatsu T, Mukawa K, Ochi Y, Arakura N, Kiyosawa K. Review case report: pneumatosis cystoides intestinalis associated with post-surgical bowel anastomosis: a report of three cases and review of the Japanese literature. J. Gastroenterol. Hepatol. 1998; 13: 534–7. 5. Zhang H, Jun SL, Brennan TV. Pneumatosis intestinalis: not always a surgical indication. Case Rep. Surg. 2012; 2012: 719713. 6. Morris MS, Gee AC, Cho SD et al. Management and outcome of pneumatosis intestinalis. Am. J. Surg. 2008; 195: 679–82. 7. Voboril R. Pneumatosis cystoides intestinalis – a review. Acta Medica (Hradec Kralove) 2001; 44: 89–92. 8. Lim CXQ, Tan WJH, Goh BKP. Benign pneumatosis intestinales. Clin. Gastroenterol. Hepatol. 2014; 12: A25–6. 9. McCarville MB, Whittle SB, Goodin GS et al. Clinical and CT features of benign pneumatosis intestinalis in pediatric hematopoietic stem cell transplant and oncology patients. Pediatr. Radiol. 2008; 38: 1074–83.
Alex Ordonez, MD Fernando Dip, MD David Nguyen, MD Emanuele Lo Menzo, MD, PhD, FACS Szomstein Samuel, MD, FACS Raul Rosenthal, MD, FACS The Bariatric and Metabolic Institute, Section of Minimally Invasive Surgery, Department of General and Vascular Surgery, Cleveland Clinic Florida, Weston, Florida, USA doi: 10.1111/ans.13022
© 2015 Royal Australasian College of Surgeons
