Bauhin’s Ileocecal Valve Syndrome—A Rare Cause for Small-Bowel Obstruction: Case

Case
Reports
Bauhin’s Ileocecal Valve Syndrome—A
Rare Cause for Small-Bowel Obstruction:
Report of a Case
Eviatar Nesher, M.D., Letizia Schreiber, M.D., Nahum Werbin, M.D.
Department of Surgery A, Tel-Aviv Souraski Medical Center, Sackler Faculty of Medicine,
Tel Aviv University, Tel Aviv, Israel
Benign ileocecal valve hypertrophy is a rare cause for intestinal obstruction. We describe a 51-year-old female with
clinical and imaging presentation of chronic small-bowel
obstruction. The patient was operated on, and a limited
thickening of the ileocecal bowel wall causing intestinal
obstruction was found. A right hemicolectomy was performed. On microscopic examination, severe fibrosis
with hypertrophied nerves was found without any additional findings of malignancy or inflammation. Review of
the relevant literature is presented. [Key words: Ileocecal
valve syndrome; Bauhin’s valve syndrome; Small-bowel
obstruction]
ined by her attending physician and underwent
complete blood laboratory examination, gynecologic
examination, colonoscopy, and abdominal ultrasound, all within the normal. When her complaints
aggravated, she was referred to our outpatient clinic.
On physical examination of the abdomen, distention and hyperperistaltic waves with obstructive peristaltic sounds were remarkable without any palpable
mass and a working diagnosis of chronic intestinal
obstruction was made, possibly the result of a smallbowel tumor. The patient was hospitalized and a
plain abdominal x-ray confirmed the clinical diagnosis of chronic, incomplete, small-bowel obstruction.
An abdominal CT examination (Fig. 1) added the
findings of a transitional zone at the terminal ileum
with a thickening of the bowel wall with extreme
small-bowel dilation and the presence of contrast
material and gas in the colon.
After this information, the patient was operated on.
During abdominal exploration very dilated smallbowel loops were seen with a focal, uniform thickening of the ileocecal wall of firm consistency, without
any macroscopic evidence of inflammation, vascular
engorgement, or neoplastic disease. A formal right
colectomy was performed.
Macroscopically the resected specimen showed a
marked annular thickening of the bowel wall in the
area of the ileocecal valve and the mucosa appeared
intact. The histologic findings in the thickened ileocecal valve revealed severe fibrosis of the submucosa, muscularis propria, and adjacent fatty tissue
C
hronic small-bowel, partial obstruction may be
caused by various reasons—benign or malignant. We present one of the rare etiologies that
causes obstruction: the ileocecal valve benign hypertrophy or Bauhin’s valve syndrome.
REPORT OF A CASE
A 51-year-old female suffered from intermittent
periumbilical colic pain, nausea and vomiting, anorexia, and weight loss of 5 to 6 Kg during six
months before her hospitalization. Except for a lumbar discectomy years ago, no other relevant medical
history was noted. During that period she was exam-
Correspondence to: Eviatar Nesher, M.D., Department of
Surgery ‘‘A’’ Ichilov Hospital, 6 Weizman Street, Tel Aviv, Israel,
e-mail: nwerb@hotmail.co.il
Dis Colon Rectum 2006; 49: 1–3
DOI: 10.1007/s10350-005-0308-1
* The American Society of Colon and Rectal Surgeons
Published online: 16 February 2006
1
NESHER ET AL
2
Figure 1. CT scan: note the very dilated small bowel and
the narrowing of the ileocecal area.
with hypertrophied nerves and the mucosa was focally eroded (Fig. 2). The ileum and the colon were
unremarkable.
After an uneventful postoperative course, the
patient was discharged on postoperative Day 7.
DISCUSSION
Gaspard (or Caspar) Bauhin (1560–1624), a botanist, anatomist, and physician, as well as Professor of
Greek in Basle, Switzerland, is presumed to be the
first to describe the ileocecal valve. His most remarkable contribution to anatomy was the reform in nomenclature, particularly of muscles, renaming them
according to: substance (semimembranous), origin
Dis Colon Rectum, April 2006
(arytenoideus), shape (deltoid, scalenus), origin and
insertion (cricothyroideus, styloglossus), number of
heads (triceps, biceps), size (vastus, gracilis), position (pectoralis), and use (pronator and supinator).
In botany he distinguished between genus and species and separated botany from materia medica.1
Bauhin’s valve syndrome was first described in
1953 by Debray et al.2 in a patient suffering from
recurrent diarrhea and weight loss with an enlargement of the ileocecal valve seen on x-rays. Lasser and
Rigler3 also reported on nine patients with Bauhin’s
valve enlargement on barium enema, and adding
nine more similar cases described the clinical and
radiologic presentation. The etiology that they proposed suggests increased fatty infiltration and not a
true lipoma; the only drawback for this radiologic
dissertation is that no surgical or pathology evidence
was produced to confirm the hypothetical diagnosis.
Since then, sporadic reports document the syndrome
as characterized by vague abdominal pain, cramps,
nausea, vomiting, pain in the right upper quadrant or
right lower quadrant of the abdomen, distention, constipation, diarrhea, active bleeding, or melena—all
presumably caused by a hypertrophic ileocecal valve.
Salem and McGee4 described the ileocecal syndrome as a result of lipomatosis of the ileocecal region, and Gazet5 in 1964 detailed the microscopic
appearance of a submucosal lipoma involving the
ileocecal junction, together with extensive hemorrhagic areas, macrophages containing hemosiderin,
and fibrous bands alternating with lipophagic reaction, appearing between the lipoma and the over-
Figure 2. A. Normal colonic wall. B. Mucosal and submucosal fibrosis.
Vol. 49, No. 4
ILEOCECAL (BAUHIN’S) VALVE SYNDROME
lying mucosa. We have not found in our study any
excessive lipomatous tissue, and noted only fibrosis
of the mucosa and submucosa.
The differential diagnosis of a prominent ileocecal
valve includes lipomatosis or fat deposition beneath
the mucosa, prolapse of the ileocecal valve into the
cecum, or a recently reduced intussusception. Lymphoma, adenocarcinoma—primary or metastatic6,7—
tuberculosis, or inflammatory bowel disease as well
as a solitary cecal ulcer also should be taken into
consideration. Outside pressure from a pericecal
mass can also cause similar complaints and signs
and has to be ruled out, preferably by CT.
The hypertropic valve can appear on plain abdominal x-rays as a translucent shadow with or without a central slit or stellate configuration.3,8 CT will
demonstrate thickened bowel wall, dilated small
bowel, and will rule out external pressure or malignancy in most cases.
Colonoscopy should be performed and can be
helpful in diagnosis showing changes in size and
shape of the ileocecal area, and biopsy specimens
can discard the possibility of malignant or inflammatory etiology.
The recommended treatment is a limited ileocecal
resection if malignancy is ruled out or a formal right
colectomy if malignancy is suspected. Recently, a
conservative surgical approach was described,9 performing dissection of the hypertrophic and swollen
part of the ileocecal sphincter through a cecotomy.
3
Because of the rarity of this syndrome and the
much more frequent occurrence of benign or malignant tumors, we recommend resection of the
involved bowel, especially in the acute patient with
bowel obstruction.
REFERENCES
1. Kyle RA, Shampo MA. Gaspard Bauhin. JAMA 1979;
242:1162.
2. Debray C, Rubens-Duval A, Pergola F, et al. Pseudotumoral inflammatory edema of the ileo-cecal valve.
Arch Mal Appar Dig Nutr 1953;42:163 – 73.
3. Lasser EC, Rigler LG. Ileocecal valve syndrome. Gastroenterol 1955;28:1 – 16.
4. Salem MH, McGee HH. Hypertrophy of ileocecal valve:
plastic repair. Arch Surg 1959;78:928 – 33.
5. Gazet JC. The ileocaecal valve syndrome. Br J Surg
1964;51:371 – 4.
6. Schnur MJ, Seaman WB. Prolapsing neoplasms of the
terminal ileum simulating enlarged ileocecal valves. AJR
Am J Roentgenol 1980;134:1133 – 6.
7. Rabau MY, Alon RJ, Werbin N, Yossipov Y. Colonic
metastases from lobular carcinoma of the breast: report
of a case. Dis Colon Rectum 1988;31:401 – 2.
8. Ng AC, Margolis AR. Roentgenographic finding of
primary carcinoma of the ileocecal valve. Surgery
1963;54:861 – 4.
9. Di Rienzo M, Mascitelli E, Angelucci D, et al. Surgical
conservative treatment for Bauhin’s syndrome. Ann Ital
Chir 2002;73:189 – 195.