For theatre or not? An interesting GI case - PowerPoint Presentation

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For theatre or not? An interesting GI case

Dr J

Bretherton

, Radiology

SpR

Dr K

Planche

, consultant radiologist

Royal Free Hospital, London

Clinical Information

54-year-old female

Presenting complaint: abdominal pain and distension

Past medical history:

Vaginal hysterectomy for uterine prolapse

Raynaud’s

Chronic bloating and constipation

Laparotomy at another hospital (details unclear)

Examination findings:

Abdominal distension and generalised abdominal tenderness without guarding

PR examination: stool in rectum.

Stable observations

Investigations (X-rays)

Investigations (X-rays)

CXR:

subdiaphragmatic

free gas and dilated bowel loops.

AXR:

Rigler’s

sign

and

dilated bowel loops.

Investigations (contrast enhanced CT abdomen and pelvis)

Investigations (contrast enhanced CT abdomen and pelvis)

Large volume free fluid and

locule

of free gas (arrowed).

Investigations (CT- lung windows)

Investigations (CT- lung windows)

Dilated small bowel loops, large volume

pneumoperitoenum

and gas containing cystic structures in the bowel wall (arrowed) in keeping with

pneumatosis

No portal venous gas or bowel wall defect was demonstrated.

Investigations (CT- lung bases)

Investigations (CT- lung bases)

Patulous, fluid filled oesophagus (asterisk)

Mild dilatation of the lower lobe airways (arrow)

*

Final diagnosis

= Systemic sclerosis with benign spontaneous

pneumoperitoneum

How we confirmed the diagnosis

Our institution is a tertiary referral centre for systemic sclerosis and scleroderma, and the referring clinicians and reporting radiologist were familiar with this entity.

The dilated oesophagus

and early basal architectural distortion seen

on the CT

are characteristic features of systemic sclerosis. The clinical team were able to confirm that the patient had cutaneous manifestations of the disease (

calcinosis

and Raynaud’s phenomenon).

Notes from previous hospital admission elsewhere were obtained. The presentation was similar and the patient had a laparotomy for suspected perforation. No site of perforation was found, but the operating surgeon noticed small bowel

pneumatosis, and took an intra-operative biopsy to confirm.Patient improved with conservative management and was referred to Rheumatology.Repeat CT after six months (to investigate weight loss) showed resolution of the pneumoperitoneum but persistent small bowel dilatation and ascites.

Systemic Sclerosis

Systemic sclerosis is a multisystem autoimmune disease characterised by fibrosis of the skin and internal organs secondary to collagen accumulation, and

vasculopathy

[1].

GI manifestations are common, occurring in approximately 90% of patients, and the spectrum of GI involvement is wide (oesophagus and small bowel most commonly involved) [1].

Atrophy of muscularis propria

 replacement by collagen  decreased peristalsis  stasis and dilatation [1].

Pneumatosis in Systemic Sclerosis

Aka

pneumatosis

cystoides

intestinalisBenign condition characterised by air filled cysts in bowel wall and mesentery, most commonly occurring in the jejunum and ileum[2].May be associated with portal venous gas.Pathophysiology poorly understood but thought to involve dissection of gas into bowel wall either due to:

- Defect in bowel wall secondary to atrophy/

ischaemia

/bacterial overgrowth [3]

- Gut flora overgrowth and excessive hydrogen production because of bowel stasis[3].May be asymptomatic or associated with abdominal pain and distension[3]. Should not be associated with signs of peritonism/fever/raised inflammatory markers[3].May be exacerbated by immunosuppressive agents.

N.b. these can also mask signs of true perforation.

Benign spontaneous pneumoperitoneum

in Systemic Sclerosis

Almost always associated with

pneumatosis

.

Thought to occur due to cyst rupture [3].May also occur due to microperforation of jejunal diverticula [3].CT imaging is invaluable- reassuring findings are:

presence of

pneumatosis

absence of other signs such as fat stranding, fluid collection, discrete bowel wall defect

Can persist for years and does not require surgical treatment [3].

Key learning points

Radiologists and clinicians should be aware of this uncommon condition (benign spontaneous

pneumoperitoneum

in systemic sclerosis) to avoid unnecessary surgery in this high risk patient group.

These patients are also at risk of perforation due to

ischaemia and obstruction therefore clinical correlation is important (signs of peritonism = not benign pneumoperitoneum!). Clinical correlation can be challenging in context of immunosuppressant use.

CT is helpful to look for

pneumatosis

and absence of any signs suggesting other cause of

pneumoperitoneum.

Key learning points

Remember the classical imaging appearances of

pneumatosis

cystoides

intestinalis

References

[1] Schreiner et al.

Gastrointestinal Manifestations of Systemic

Sclerosis.

J Scleroderma Relat Disord. 2016; 1(3): 247–256.[2] Hassan & Devgun Pneumatosis Cystoides

Intestinalis

: A Rare Benign Cause of

Pneumoperitoneum. Case Rep Radiol. 2013; 2013: 353245.[3] Vischio et al. Benign Spontaneous Pneumoperitoneum in Systemic

Sclerosis. Journal of Clinical Rheumatology. 16(8):379-381, DEC 2010