12 y old Saudi girl not known to have any medical illness before presented to the ER with Hx of abdominal pain for 3 days .

The pain started in upper abdomen ,colicky in nature radiated to the back and associated repeated attack of vomiting . The last attack of vomiting was bilious

There was Hx of absolute constipation for the 3 days.

No previous Hx of bloody diarrhea ,fever , abdominal distention.

The past surgical HX was –ve.

No previous Xx for allergy or B.T.

The systemic review was unremarkable.

She was conscious, oriented and in sever pain can not lie down from it.

The vital sign :

HR : 140 bpm

Temp : 37.7 c

BP : 130/80 mmHg

RR: 25 bpm

She was slightly pale but not jaundice .

Abdominal examination showed guarding all over it with tenderness .

There was palpable mass occupying the upper part of the abdomen which was irregular in shape, measure 10x12x8cm, hard, tender smooth surface.

Sluggish bowel sound .

The investigation:

CBC:

WBC : 24.2

Hb :10.5

Platelet : 260

Na: 130

K : 4.4

Cl : 93 -low

Urea: 5.4

Creatinin :61

Amylase : 29

Glucose: 7.7

PH :7.37

Pco2 : 5.58

Po2 : 5.22

Abdominal x-ray:

No distended bowel, no air-fluid level and no air under diaphragm

A large segment of intussusceptions is seen extending from the R.L.Q of the abdomen passing middle abdomen to the Lt side

There was edema in the walls of the bowel loop.

Dilated, fluid-filled proximal small bowel is also seen.

No free peritoneal collection.

Conclusion:

Large segment of small bowel intussusceptions.

During the examination incidentally we found pigmentation in the inner aspect of the lower lip.

The Pt admitted in 14/1/09 as case of petuz –jegher syndrome with

intussusception for exploratory laprotomy with possible resection.

the incision done transverse in the Rt side lower abdomen.

There was huge c-shape mass, sausage-like occupying the central part of the abdomen which was difficult to deliver out side .

Trial of reduction was attempted ,which was easy at the beginning but mid way it was quite impossible.

Several serosal tears occurred .

Atrial of widening in the neck of intussusception mass was done but failed as well to complete the reduction.

Entrotomy was done for intussuscipiens loop which facilitated the reduction.

After the reduction it was noted that around 40 cm of jejunal loop were doubtful, so hot saline packing done for 10 minutes.

A5 cm proximally turned out to be completely unhealthy almost necrotic with a leading point , which constituted of large polyp.

this 5cm was excised from the proximal doubtful area of the affected bowel.

Distally, there was another area with sever serosal tear of 5 cm was excised as well.

Tow anastomoses were done.

Irrigation done profusely for abdominal cavity.

The decision was taken to close the abdomen over this with still doubtful area for possible second look.

Apenrose drain was applied .

she kept NPO ,vital q 4hrs,abdominal girth q8hrs

NGT on free drain &triple antibiotic(ampi-genta&flagyl)

2nd day post op she had multiple spike of fever (28.5 c).

The abdominal girth was static 62 cm for the last 2 days.

She received PRBCS due to low Hb 8.1 post op

day abdominal girth increased 3cm.

She passed small amount of bloody stool twice (melena).

Large amount of the NGT drainage.

Bowel sound was sluggish.

Fever still on/off.

The abdominal ex ,soft but sever tenderness.

she stared on clear fluid but didn’t tolerate it and she vomited ,so she kept NPO again .

There was plan to remove the drain because there was nothing drained since we inserted before 5 day.

In the after noon the Penrose drained 100cc of greenish secretion .

Distended colonic segment with fecal material and air.

No air under diaphragm

There are some prominent small bowel loop

No air fluid level.

There was large collection seen in the mid abdomen anteriorly containing large air pockets fluid and hypodense material with the tip of the Penrose seen inside this collection.

Significantly dilated jejunal loops and distal bowel loops.

The contrast didn’t reach the distal small bowel

Large fluid collection seen in the mid abdomen with air pocket, there is high

possibility of disruption of one of anastomatic sites.

The finding:

Necrotic jujenal loop around 40 cm.

Three perforation out side the segment in other wise healthy bowel.

Feculent peritonitis.

necrotic segment excised.

Suturing of the three perforation in tow layers.

Peritoneal irrigation.

Excoriorization of both end through the wound.

She shifted to ICU for 24 hrs for observation.

We she shifted to the word kept NPO on TPN

On the 7th day post op she start the oral

feeding for liquid diet.

In 1/2/09 jujenostomy closure done.

she discharged home in 9/2/09 in a good condition with OPD appt with gastroenterologist.

She admitted for upper &lower GI endoscopy .

EGD:small polyp in the antrum and biopsy was taken from it which showed:

(Hyper plastic gastric polyp with focal regenerative changes)

The colonoscopy was normal.

She did barium follow through which was normal.

She discharged home in the same day in good condition.

(PJS) is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with mucocutaneous melanocytic macules.

It is appears to be a germline mutation of the (serine/threonine kinase 11) tumor suppressor gene in most cases (66-94%).

Although the intestinal lesions are hamartomas, patients with Peutz-Jegherssyndrome (PJS) have a 15-fold increased risk of developing intestinal cancer compared with that of the general population.

the frequency from 1 case per 60,000 people to 1 case per 300,000 people.

The principal causes of morbidity in Peutz-Jeghers syndrome (PJS) stem from the

intestinal location of the polyps (ie, small intestine, colon, stomach).

Morbidity includes small intestinal obstruction and intussusception (43%), abdominal pain (23%), hematochezia (14%), and prolapse of a colonic polyp (7%), and these typically occur in the second and third decades of life.

Intestinal obstruction can occur in about 50% of patients, and it is usually localized in the small bowel.

Almost 50% of patients with Peutz-Jegherssyndrome (PJS) develop and die from cancer by age 57 years.

Due to the increased risk of pancreatic adenocarcinoma in Peutz-Jeghers syndrome (PJS), screening with endoscopic ultrasound has emerged as a relatively new tool for early diagnosis

The average age at Peutz-Jeghers syndrome (PJS) diagnosis is 23 years in men and 26 years in women.

Pigmented lesions are present in the first years of life and may fade at puberty, except for lesions on the buccalmucosa, making the diagnosis possible in pediatric patients with a high level of suspicion

characterized by the combination of pigmented lesions in the buccal mucosa and gastrointestinal polyps.

Family history of Peutz-Jeghers syndrome

Repeated attack of abdominal pain in patients younger than 25 years

Prolaps of tissue from the rectum .

Menstrual irregularities in females due to hyperestrogenism from sex cord tumors.

Gynecomastia in males due to the production of estrogens from Sertoli cell testicular tumors.

Precocious puberty.

Gastrointestinal intussusception with bowel obstruction.

Cutaneous pigmentation

A rectal mass (rectal polyp) may be found during a rectal examination. In rare cases (7% of cases), the polyp can prolapse outside the anus if it reaches a significant size.

Gynecomastia and growth acceleration (due to Sertoli cell tumor) .

Testicular mass.

CBC- iron deficiency anemia.

The (CEA) test has been used by some physicians for screening and monitoring of cancer degeneration.

Hemoccult, a type of fecal occult blood test.

Enteroclysis study (preferred) and dedicated small bowel follow-through x-rays are used to determine the presence and the location of small intestinal polyps.

Esophagogastroduodenoscopy (EGD).

Colonscopy.

Capsule enteroscopy.

Push enteroscopy, intraoperative enteroscopy, and double-balloon enteroscopy (diagnostic and therapeutic options).

These imaging studies may include ultrasonography as well as computed tomography (CT) scanning with pancreatic details or magnetic resonance cholangiopancreatography (MRCP).

Medical Care:

Annual physical examination that includes evaluation of the breasts, abdomen, pelvis, and testes.

Annual complete blood cell (CBC) count

Removal of hemorrhagic or large polyps (>5 mm) by endoscopic polypectomy .

Some suggestions for surveillance for cancer

Push enteroscopy and interoperativeenteroscopy with polypectomy to remove larger polyps.

Laparotomy and resection, as indicated, for small intestinal intussusception, obstruction, or persistent intestinal bleeding may be necessary.

Surgical treatment of extraintestinal cancers detected by surveillance and diagnosis is required.

Forty-eight percent of patients with(PJS) develop and die from cancer by age 57 years. Others may potentially have a normal life span.

The patient with Peutz-Jeghers syndrome should be educated on the potential symptoms of intestinal obstruction and instructed on the

need for cancer surveillance.

THANK YOU

Dr.Amani ALhaddad