COLONIC OBSTRUCTION IN AN ADULT PATIENT
DR.ANTOINE NASTA
GENERAL SURGERY
CASE PRESENTATION
20Y.O. MALE SAUDI
ABSOLUTE CONSTIPATIN+VOMITING+ABDOMINAL DISTESION FOR AT LEAST 4 DAYS.
ADMITTED ON 20/4/1424
PAST HISTORY
CHRONIC CONSTIPATION FOR MANY YEARS.
NO PAST HISTORY FOR ABDOMINAL SURGERY,CHRONIC MEDICATIONS OR SYSTEMIC ILLNESS.
THE BROTHER (25 Y.O.) HAD COLONIC SURGERY IN CHILDHOOD
CLINICAL EXAMINATION
PATIENT WAS EMATIATED,FEBRILE(38.2) WITH MILD DEHYDRATION.
THE ABDOMEN WAS HUGELY DISTENDED,RESONANT,NON TENDER.
THERE WAS NO FREE FLUID,THE B.S. WERE SLUGGISH,AND
THE HERNIAL ORIFICES WERE FREE
INVETIGATIONS
W.B.C.=15.900/mm3
Hb.=12.5g%
K+=3mm/L
X-RAY ABDOMEN:HUGELY DISTENDED COLON.NO FREE GAZ
TREATMENT AND PROGRESS
PATIENT WAS DIAGNOSED AS A CASE OF COMPLETE COLONIC OBSTRUCTION(VOLVULUS??),
HE WAS PREPARED FOR SURGERY UNDER G.A.
ON OPERATION:THE COLON WAS HUGELY DISTENDED FROM CAECUM TILL
THE DESCENDING COLON ,
BUT WITHOUT SEVERE TENSION OR SEROSAL LACERATIONS OR ISCHEMIC SIGNS.THE SIGMOID
WAS COLLAPSED,AND THE RECTUM WAS SO FINE THAT IT COULD NOT BE INTUBATED BY A FLATUS TUBE.
THE SMALL BOWEL WAS NOT DISTENDED
PROCEDURE
HIRSCHSPRUNG DISEASE WAS SUSPECTED
APPENDECTOMY WAS PERFORMED TO ASPIRATE THE COLON THROUGH THE STUMP
LATERAL COLOSTOMY WAS PERFORMED ON THE DESCENDING COLON
RECTAL BIOPSY WAS TAKEN AFTER CLOSURE OF THE ABDOMEN
HISTO-PATHOLOGY
APPENDIX AND COLON:GANGLION CELLS ARE PRESENT
RECTAL BIOPSY:ABSENCE OF GANGLION CELLS,HYPERTROPHY OF NERVE FIBERS
POST-OP COURSE
PATIENT WAS PUT ON N.G.T. AND I.V.F
THE COLOSTOMY FUNCTIONNED WITH THE HELP OF A CATH. INTRODUCED IN
AFTER REMOVAL OF THE N.G.T.,THE ABD. BECAME VERY DISTENDED AGAIN(6TH DAY POST OP.)
N.G.T. WAS INSERTED AGAIN,AND BROUGHT MORE THAN 5 L.OF FLUIDS
PLEIN CT-SCAN OF THE ABD.DONE,IT SHOWED DISTENSION OF THE WHOLE G.I.T.
WE DECIDED TO RE-EXPLORE THE ABDOMEN AGAIN.
2d LAPAROTOMY
SEVERE GASTRIC DISTENSION WITH THICK WALL.
THE DUODENUM MEASURED >10 cm.
ALL INTESTINAL LOOPS WERE SEVERELY DISTENDED BUT WITHOUT TENSION OR SIGNS OF ISCHEMIA
HUGE DISTENSION OF THE COLON(CAECUM MEASURED >15cm.)
TILL THE SIGMOID
ONLY RECTUM WAS STILL VERY NARROW (MICRORECTIA)
PROCEDURE
CAECOSTOMY ON TUBE
LEFT COLOSTOY TERMINALISED
POST-OP. COURSE
PATIENT WAS PUT ON N.G.T. ,T.P.N.AND TRIPLE ANTIBIOTICS
THE NGT PRODUCED HUGE AMOUNT OF FLUID DAILY(5-6L)
THE COLOSTOMY NEVER FUNCTIONED
HE WAS SHIFTED TO THE I.C.U.
FOR PROPER FLUID MANAGEMENT WHEN B.U.N STARTED TO INCREASE
HIS V.S. REMAINED STABLE,BUT 10 DAYS AFTER STARTING TPN
HE DEVELOPPED SEVERE METABOLIC ACIDOSIS(Ph=7,HCO3=6.6)
FEW HOURS LATER HE C/O SEVERE HEADACHE
THEN DEVELOPPED CONVULSIONS WITH DETERIORATION OF HIS L.O.C,HYPOTENSION AND DESATURATION…
FRESH BLOOD STARTED COMING FROM THE NGT. IN BIG AMOUNTS
THE COAG. PROFILE SHOWED PTT>100,AND INCREASED D-DIMER
HE WAS TREATED BY MASSIVE TRANSFUSION OF
PRBC+FFP+PLATELETS,BESIDE BIG AMOUNT OF BICAR.
THE CONDITIONS CONTINUED TO DETERIORATE ,AND DIED ON 16/5 /1424
DIAGNOSIS??
HIRSCHSPRUNG DISEASE(ADULT FORM)??
OR….
IDIOPATHIC INTESTINAL PSEUDO-OBSTRUCTION???
IF HIRSCHSPRUNG…
WHAT IS IN FAVOR OF HIRSCHSPRUNG:1-THE COLONIC DISTENSION 2-THE MICRO RECTUM 3-THE HISTOPATH.
WHAT IS AGAINST:THE POST OP. COURSE WITH THE HUGE AND ATONIC DISTENSION OF THE PAN G.I.T.
INTESTINAL PSEDO-OBSTRUCTION
HYPOGANGLIOSIS
CHRONIC ADYNAMIC ILEUS
PSEUDO-HIRSCHSPRUNG DISEASE
VISCERAL NEUROPATHY
VISCERAL MYOPATHY
DIGNOSIS OF PSEUDO-OBSTRUCTION
SUSPECTED WHEN A GROUP OF CLINICAL,PARA-CLINICAL AND EVOLUTIVE FINDINGS ARE PRESENT…
POSITIVE CLINICAL ARGUMENTS
YOUNG ADULT(26)
ALTERNATION SUB-OBSTRUCTION/DIARRHEA
L.O.W.
FAMILIAL PREDISPOSITION
POSITIVE EXPLORATION FINDINGS
X-RAY:DISTENSION OF BOWEL AND DELAYED PROGRESSION OF BARIUM
DIGESTIVE MOTRICITY:PROLONGED TRANSIT TIME,ANOMALY IN
THE INTESTINAL MOTRICITY,AND ANOMALY IN THE ESOPHAGEAL MOTRICITY
NEGATIVE ARGUMENTS
ABSENCE OF ORGANIC OBSTACLE
ABSENCE OF ELECTROLYTIC DISORDERS
ABSENCE OF CERTAIN DRUGS:OPIATES,ANTI-DEPRESSORS,GANGLIOPLEGIC…
THE I.I.P. IS A RARE DISEASE,HAVING SEVERE PROGNOSIS
THE DIAGNOSIS IS MADE BY EXCLUSION
THE PRESENCE OF ANOMALY OF MOTRICITY OR HISTOLOGY(INTESTINAL PLEXUS
OR MUSCLES)WILL BE OF GOOD HELP.
histopathology
The usual techniques(HES) are unsufficient,the use of
immunohisto –chemistry and electronic microscope can show some muscular or nervous anomalies in the majority of cases.
Familial forms
Multiple spontaneous mutations affecting the nervous system or the muscular metabolism
The age of beginning of symptoms ,and the extent of the intestinal anomalies are variable.
management
No specific treatment.only prevention and palliation
The attacks of the disease are precipitated by:infection,electrolytic disorders,G.A..,surgery…
NGT,T.P.N ,antibiotics
Surgery is unavoidable to establish the diagnosis. Stoma and bypass operations are not the solution because it is a diffuse disease.
No efficient medication.
Why we failed to label our case?
Not enough diagnostic tools
No manometry
Weakness in the histopathology:no comment on the muscular layers,no specific colorations,no immunohistochemistry.
litterature
1-submucosal hypogangliosis:the patient with I.I.P..
of neurogenic type had a defect in the sub-mucosal plexus,
whereas visceral neuropathies are usually caracterised by defects of the myenteric plexus.
(Von Boyen et al.Germany-Am.J.G.E)
2-INTESTINAL NEURONAL DYSPLASIA (BOSMAL ET AL.-ITALY, KAPUR-USA)
3-IDIOPATHIC MYENTERIC GANGLIONITIS(INFLAMMATORY NEUROPATHY).
THE IDIOPATHIC FORM IS RARE,SOME OTHER ETIOLOGIES COULD BE FOUND:
PARANEOPLASIC,INFECTIOUS OR NEUROLOGIC DISORDERS….
(DE GIORGIO ET AL.-ITALY)
Connective tissue disorders (desmosis coli)