A Rare Presentation of Crohn’s disease

                A Rare Presentation of Crohn’s disease

Case Capsule

21 Year Old Male presented to the Emergency Department with Generalized Abdominal Pain , Fever and  Vomiting of 5 Days.   No urinary complaints , No constipation/ Diarrhoea .No significant past illnesses.

On Examination, patient was Conscious, with Pulse Rate 80/minute  and

Blood Pressure 120/80 mm of Hg, He was Afebrile. Not icteric.

Per Abdomen  showed generalized voluntary guarding and with tenderness mainly over the Left Iliac Fossa -Bowel sounds were present.

Per Rectal Examination- NAD               Other Systems-NAD

Plain X ray Abdomen in Erect Posture was taken from the Casualty- Showed a few nonspecific minimally dilated loops

USG Abdomen was showing-  Diffuse Jejunal Wall Thickening with Sluggish Peristalsis.

Admitted in the Surgical ward for the evaluation of Abdominal pain with Fever & Vomiting

Possibility of  Inflammatory pathology was made and the patient was kept on NPO/ Ryle's Tube Aspiration, Broad spectrum Antibiotics, Closed Bladder Drainage, Supportive Therapy.

Blood  Hb 13.2g%  TC 5000/cmm  P55% L 43%   Eo 2% ESR 43mm   

Blood Group O +ve

 Platelet Count 2.4 Lakhs            RBS 135mg%

 Blood Urea-19mg%                     S Creatinine 0.7mg%

 Serum Na+ 137 meq /L               Serum  K+ 4.1meq/L

Serum Amylase 33U/L                Serum Lipase 105U/L

Serum Bilirubin 0.9mg%            Serum ALP 31U/L 

Serum calcium 7.5mg%              Serum LDH 416U/L

SGOT/SGPT    55 & 33                  PT/ INR 1.57  

Total Protein 6g/L                        S Albumin 3.1g/L                            

HIV, HBsAg ,Anti HCV, Mantoux  were Negative

Widal & Dengue IgG & IgM were  Negative

Peripheral Smear showed a Blood picture within normal limits except for Reactive Neutrophilia with Shift to Left and Toxic Granules.

Ryles Tube Aspirate – Mild to moderate amount~ 150-600 ml- Bilious

Repeat USG on 3rd Post Admission Day showed Fluid filled small bowel loops noted in left lumbar region with minimally thickened bowel loops. Adjacent mesentery appear hyperechoic- ?Diverticulitis

However, considering his age we couldn’t welcome the diagnosis of Diverticulitis- Hence went ahead with next higher investigation –

CT Scan Abdomen which showed Jejunal Dilatation with suspicious narrow segment of  proximal ileum, mesenteric lymphadenopathy & stranding. Possibility of jejunal obstruction due to ? Inflammatory adhesions to be considered.

Patient Developed Bilious Vomiting on 5^th Day & Repeat X ray Abdomen revealed  Multiple Air Fluid Levels.

Built up the patient with FFP and with other pre operative preparations he was posted for Exploratory Laparotomy

The per operative findings were- Jejunal loops found thickened & hyperemic at the mesenteric border for a length of 45 cm beginning at 15 cm from the DJ Flexure, Multiple firm lymph nodes along the surrounding mesentery, Minimal sero sanguinous free fluid, Jejunal loops found mildly dilated

No definite mass felt in the Ileum and Jejunum. No evidence of saponification /bowel perforation. Rest of small bowel, Appendix, Large bowel, Sigmoid, solid organs and Stomach were Normal. No Meckel’s  diverticulum found .

Hence a Peritoneal Lavage was given, Mesenteric Lymph node Biopsy taken

Hemostasis achieved and the wound closed in layers with No drain insitu .

 No per op Blood Transfusion.Specimen sent for Histo pathological Examination and the patient was shifted to General Post Op ward.

Considering the Per op finding ~ Medical Gastro as well as Surgical Gastro Consultations were Taken

 The Medical Gastro Enterologist retrospectively elicited the history of Self induced vomiting after binge eating about 3 days/week for the last 6 months

The possibilities considered were 1. Crohn’s Disease, 2. Intestinal Tuberculosis  3. GI Lymphoma

2^nd, 3^rd ,4^th & 5^th Post op days were uneventful

Patient passed stools on 6^th Post Op day

Started on Oral Fluids  from Post Op day 7

On 8^th Post Op day patient complained of Abdominal Pain. His Pulse rate was 84/min and found to have tenderness over the LIF

Abdominal pain continued for one more day ie POD 9

On POD 10, Patient developed Vomiting of altered blood on Post Op day 10 ( 3 Episodes ) during the day time. In the night he had 2 more episodes of Hemetemesis. Hence Ryles tube was re introduced.

On 11^th POD, 750 ml more bloody aspirate was drained through the Ryles tube on Early morning of Post Op day 11

His Pulse Rate was 100/min and Blood Pressure was 106/90 mm of Hg . Patient developed sweating and cold clammy peripheries. Presumptive Diagnosis made at that time was  Compensated Hypovolemic Shock  due Upper GI Bleed

Immediately 2 units of Packed Cell Transfusion were given. And he was shifted to the Surgical ICU

At the time of arrival at the ICU the patient was conscious, PR 90/min, Resp Rate 26/min BP100/60mm Hg and SPO2 was 99

Per abdomen  soft. Expansile pulsation over the Epigastrium. Tenderness over LIF and Left Hypochondrium was present.

Hb 9.8g%  PCV 28.8

The possibilities considered were ? Aneurismal bleed ? Major Arterial  bleed  ?Mesenteric Vascular Ischemia

Emergency Medical Gastro Consultation was taken. OGD Scopy on the same day morning showed Pooling of blood in D1 & D2. Blood coming from D2àStomach

Suggested Emergency Angiography and Embolization .On the very same day afternoon patient was taken for Angiography .

Angiogram showed abnormal nodularity with areas of narrowing involving Common hepatic artery and Pancreatico duodenal artery. SMA showed diffuse narrowing of branches and abnormal Capillary Blush in the region of jejunal loops in Lt lower abdominal quadrant.  Punctuate contrast filled opacities  in Rt & Middle Colic branches of SMA (?Dysplastic Changes).IMA showed no significant abnormality.Possibility of underlying Vasculitis needs exclusion.

Medical gastro enterologist reviwed the patient  and suggested steroids in view of the vasculitis. Hence the patient was started on with Inj Methyl Prednisolone 1 g I/v Od x 3 days.

Complete work up for Vasculitis including ANCA, pANCA, RF etc done – All Negative.

TB Ig G & Test for Leptospirosis were  Negative. Repeat CRP value was 284.4

Histopathology Report   of lymph node came asà Reactive Changes &  Peri adenitis. No Granuloma

Medicine consultation taken for the need of any Immunoglobin therapy for Vasculitis

The opinion of the Physician was to continue steroid in a higher dose.

On POD 13, Ryles tube aspirate was ~1300 ml, Abdominal pain increased in severity. Hence a repeat CT Abdomen was taken which showed Loops jejunum dilated and filled with blood .No evidence of Gangrene of the bowel.

Hence the decision to continue the conservative management was taken.

On POD 16,patient Complained of severe Abdominal pain and distress in the early morning  (2.10 Am) . On examination Abdomen was tensed and rigid.

Developed melena and hemetemesis at 2.55 AM His Pulse Rate 152/min  BP  160/100 mm of Hg . Patient posted for Emergency Laparotomy.

Findings were- Old collected blood( Hemoperitoneum) with clots ~ 2.5 Litres

Necrosed  small bowel with walls sloughed off and lumen exposed due to multiple perforations. Necrosed areas were adherant to Transverse colon Unhealthy Small bowel  -  Major lesions were near the  DJ Flexure. Liver was pale  - Sub diaphragmatic collections present.

Procedure Done:- Adhesions released, Entire unhealthy areas bearing small bowel segment resected ( from DJ Flexure  to ~ 40 cm proximal to Ileocaecal valve) , Both ends exteriorized, proximal with a Tube Duodenostomy and distal end as a cutaneous fistula .

On table surgical gastro opinion was sought and concurrence for the procedure obtained.Wash given,  Drain placed

Wound converted in to Laparostomy with a Sterile urobag anchored to the wound edges.

Patient was cared in Mechanical Ventilator ( CMV ) with CVP Monitoring, Total Parenteral Nutrition started and other supportive measures continued.

Pathological findings were very Typical of Crohn’s Disease. With  a short aggressive course of the Illness we made the Final Diagnosis of- Fulminant Crohn’s Disease.

Vienna Classification of  Crohn’s Disease

ž Fibro Stenotic Disease ( Sticturing )

ž Fistulizing Disease  ( Penetrating )

ž Aggressive Inflammatory/ Fulminant Disease

Peroperative or diagnostic Laparoscopic finding in Crohn’s disease is                            ‘ creeping fat ’:- that is encroachment of mesenteric fat to bowel serosa. Another feature is strictures. Any resection anastamosis of such strictures can result in non healing Gastro Intestinal Fistula!