A Case of Peutz-Jegher Syndrome

A CASE OF PEUTZ-JEGHER SYNDROME
MajSMEHROTRA *,LtCoIPSENGUPTA +,
Maj CG MURLIDHARAN #
MJAFI 1999; 55 : 253-254

KEY WORDS: Intestinal obstruction; Peutz-jegher syndrome.
Introduction Case Report
A
An 18-year-old was admitted with 3 day history of abdomen
n autosomal dominant disorder, Peutz-Jegher pain, vomiting, distension and constipation. He was operated in
syndrome is characterized by hamartomatous 1985 for ileocecal intussusception resulting from a polyp and was
polyposis of the gastrointestinal tract. It is asymptomatic thereafter. Clinical examination revealed a thin boy
distinguished from other polyposis syndromes by the with distended abdomen having visible peristalsis. He also had
melanotic pigmentation of lips and palms. (Fig 1,2) Plain X-ray of
classical mucocutaneous pigmentation [1]. We report the abdomen revealed multiple air fluid levels. Biochemical and
here an eighteen year old boy who developed intesti- haematological parameters were normal. Conservative treatment
nal obstruction twice over a period of 13 years and did not succeed and he was subjected to laparotomy after five
required multiple laparotomies. days.
Surgery revealed a densely contracted mesentry with interloop
adhesions. A dense stricture causing obstruction at the ileocaecal
junction was resected and end to end anastomosis performed. Mul-
tiple large polyps were palpable in the proximal jejunum and re-
moved via enterotomies. The patient had an uneventful postopera-
tive recovery. Histopathological examination of the polyps re-
vealed hamartomatous features. There were hypertrophic bands of
muscularis mucosae enclosing islands of normal intestinal glands
containing goblet cells. (Fig 3). HPE of stricture showed gross
fibrotic thickening.
Postoperative barium meal follow through and enema of the
patient did not reveal any remaining polyp. His two siblings nei-
ther had any melanotic pigmentation nor any polyp on radiological
investigation.
Discussion
Gastorintestinal polyposis syndromes are rare he-
reditary disorders. They commonly present with
bleeding or intermittent small bowel obstruction [2].
Fig. 1: Melanotic pigmentation of lips Fig. 2: Melanotic pigmentation of palms
* Surgeon, + Pathologist, # Radiologist, Military Hospital, Yol.

254 Mehrotra, Sengupta and Murlidharan
pancreas, breast and lung. Ovarian and testicular tu-

mours may also develop with greater frequency in
these patients and need appropriate treatment [7]. Our
patient presented with intussuception in 1985 when
the syndrome was not evident. The nature of the polyp
was reported as being angiomatous. During the pre-
sent exploration multiple proximal jejunal polyps were
found apart from the fibrotic narrowing at the site of
previous surgery. It needs to be stressed that radical
removal of the polyps must be the aim of surgery.
Patients need to be closely followed by examination,
endoscopy and radiological investigations to avoid the
complications of regrowth and detect malignant de-
Fig. 3: Hamartomatous polyposis of gastrointestinal tract
generation.
Many familial polyposis are transmitted as autosomal REFERENCES
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of Peutz-Jegher syndrome immediately distinguishes it In: Sabiston DC. Ed.Text book of surgery. Philadelphia:
from other forms of polyposis. Seven cases of Peutz- WB saunders 1991:925.
Jegher syndrome were found over a study period of 3 2. Leaper DJ. Tumours of the colon. In: Schwartz SI, Ellis H,
Eds. Maingot's abdominal operations. LondOfl: Prentice Hall
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3. Ko FY, Wu Te, Hwan B. Intestinal polyps in children and
the third decade with no racial predisposition. Our pa- a40lescents-A review of 103 cases. Acta Paediatr Sin
tient did not have pigmentation earlier during initial 1~5;3~3): 197-202.
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years old. The tendency to have recurrent obs.truction Jegll$' syndro~. Br J SUrg 1995;822(10):1311.-4
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less appreciated than the disorder [4]. In addition, op- Cases at the Mainheim clinic over 25 years. LaDgenbecks
erations frequently involve complications and need to Arch C1)ir 1995;380(1):43-52.
be repeated [5]. Radical removal of all polyps must be 6. Miyahara M. Saito T. Etoeh Ie et al. Appendiceal intus-
the aim of treatment. sU$ception due to an appendiceal malignant pol~an associa-
tiori in a patient with Peutz-Jegher syndromc:report of a case.
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premalignant, associated adenomatous polyps with 7. Wilson DM, Pitts WC, Mintz RL, Rosenfield RO. Testicular
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increased risk of developing carcinomas involving
MJAFI. VOL 55. NO.3. 1999

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A CASE OF PEUTZ-JEGHER SYNDROME

MJAFI 1999; 55 : 253-254

Introduction Case Report

Fig. 1: Melanotic pigmentation of lips Fig. 2: Melanotic pigmentation of palms

* Surgeon, + Pathologist, # Radiologist, Military Hospital, Yol.

pancreas, breast and lung. Ovarian and testicular tu-

MJAFI. VOL 55. NO.3. 1999

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