CAS E REP O R T Open Access
Cronkhite-Canada syndrome associated with rib
fractures: a case report
Bosi Yuan, Xinxin Jin, Renmin Zhu, Xiaohua Zhang, Jio ng Liu, Haijun Wan, Heng Lu, Yunzhu Shen, Fangyu Wang
*
Abstract
Background: Cronkhite-Canada syndrome (CCS) is a rare multiple gastrointestinal polyposis. Up till now, many
complications of CCS have been reported in the literature, but rib fracture is not included.
Case Presentation: We report a case of a 58-year-old man who was admitted to our hospital with a 6-month
history of frequent diarrhea, intermittent hematochezia and a weight loss of 13 kg. On admission, physical
examination revealed alopecia of the scalp, hyperpigmentation of the hands and soles, and dystrophy of the
fingernails. Laboratory data revealed hypocalcaemia and hypoproteinemia. Esophago gastroduodenoscopy, video
capsule endoscopy and colonoscopy revealed various sizes of generalized gastrointestinal polyps. Histological
examination of the biopsy specimens obtained from the stomach and the colon showed adenomatous polyp and
inflammatory polyp respectively. Thus, a diagnosis of CCS was made. After treatment with corticosteroids for
24 days and nutritional sup port for two months, his clinical condition improved. Two months later, he was
admitted to our hospital for the second time with frequent diarrhea and weight loss. The chest radiography
revealed fractures of the left sixth and seventh ribs. Examinations, including emission computed tomogra phy, bone
densitometry test, and other serum parameters, were performed, but could not identify the definite etiology of the
rib fractures. One month later, the patient suffered from aggravating multiple rib fractures due to the ineffective
treatment, persistent hypocalcaemia and malnutrition.
Conclusions: This is the first case of a CCS patient with multiple rib fractures. Although the association between
CCS and multiple rib fractures in this case remains uncertain, we presume that persistent hypocalcaemia and
malnutrition contribute to this situation, or at least aggravate this rare complication. Besides, since prolonged
corticosteroid therap y will result in an increased risk of osteoporotic fracture, CCS patients who accept
corticosteroid therap y could be potential victims of rib fracture.
Background
Cronkhite-Canada syndrome (CCS) is a rare acquired
polyposis syndrome characterized by multiple gastroin-
testinal polyps with alopecia, nail dystrophy, and hyper-
pigmentation. The syndrome was first reported in 1955
by Cronkhite and Canada [1]. Since then, more than 400
cases of Cronkhite-Canada syndrome had been reported
worldwide, with 75% of them coming from Japan [2].
The mean age of onset is reported to be 59 years, and the
male to female ratio is 3:2 [3]. So far, no definite etiology
of CCS has been determined, though mental stress or
physical fatigue is thought to be involved [4], and no
strong evidence to suggest a f amilial predisposition.
Moreover, various kinds of concomitant diseases of CCS
havebeenreported.HerewedescribeacaseofCCSina
patient with multiple rib fractures.
Case Presentation
A 58-year-old man was admitted to our hospital with a
6-month history of frequent watery diarrhea (10-20 times
per day), intermittent hematochezia, and a weight loss of
13 kg. Two months after onset of symptoms, he noticed
pigmentation in the palms and hair loss. He had a nega-
tive family history of gastrointestinal disease and conge-
nital disease. On physical examination, the patient was
found t o have marked alopecia, brownish macular pig-
mentation over the palms and soles, and o nychodystro-
phy of the fingernails. The remainder of the physical
examination was unremarkable.
* Correspondence: wangfy65@gmail.com
Department of Gastroenterology, Jinling Hospital, Jiangsu province, China
Yuan et al. BMC Gastroenterology 2010, 10:121
http://www.biomedcentral.com/1471-230X/10/121
© 2010 Bosi et al; licensee BioMed Central Ltd. This i s an Open Access article distributed under the terms o f the Creative Commons
Attribution L icense (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, pro vided the origin al work is properly cited.
Initial laboratory data showed that his albumin level
was 31.2 g/L (normal range 35-55 g/L), serum potas-
sium 3.1 mmol/L (normal range 3.5-5.5 mmol/L) and
serum calcium 1.7 mmol/L (normal range 2.1-2.6
mmol/L). Other blood parameters, including thyroid
hormones, parathyroid hormone and immunoglobulins,
were within the normal range. The chest radiograph was
negative. Esophagogastroduodenoscopy, video capsule
endoscopy and colonoscopy were performed for further
evaluation of the gastrointestinal tract and they identi-
fied various s izes of generalized gastrointestinal polyps
(Figures 1, 2 and 3). Histological examination of the
biopsy specimens obtained from the stomach and the
colon showed adenomatous polyp and inflammatory
polyp respectively. Thus, a diagnosis of CCS was made.
We starte d corticosteroid therapy for him with oral pre-
dnisone (40 mg per day for 2 weeks and then reduced
thedosageto30mgperdayandlasteditfor10days),
but then we discontinued it because the clinical situa-
tion of the pa tient became better . At the same time, the
patient was treated with nutritional supplementation by
parenteral and enteral nutrition. His situation improved
gradually after two months of treatment . The frequenc y
of diarrhea decr eased to 2 times per day, the we ight
increased by 5 kg, and the hair and fingernails regrew,
but the levels of serum calcium (1.9 mmol/L) and albu-
min (30.6 g/L) were still lower than the normal range.
He returne d home for home nutritional support by ent-
eral nutrition.
Two months later, he was admitted to our hospital for
the second time wit h frequent diarrhea (7-8 times per
day) and a weight loss of 7 kg. Laboratory data showed
that his serum albumin level was 28.2 g/L and serum
calcium 1.7 mmol/L. His chest radiograph showed frac-
tures of the left sixth and seventh ribs (Figure 4). Since
the patient had not suffered from any load or trauma in
the chest, this concomitant complicatio n initially led us
to presume that there was a possibility of rib metastasis
of a malignant tumor. Emission Computed Tomography
(ECT) was performed and it showed no increased tracer
uptake in the skeleta l system. Bone densitometry tests
on vertebrae lumbales and caput femoris were normal.
Further examination for checking bone metastasis was
not per formed because of the patient ’s financial situa-
tion, and his examinations and clinical features indicated
no definite malignant tumor. Since his nutritional status
was poor and he had no complain of pain i n chest,
orthopedic surgeons and che st surgeons advis ed us to
supply calcium and nutrition for him and to restrict his
chest wall movement. After one-month treatmen t,
which was similar t o our previous treatment except for
the corticosteroid therapy, his clinical condition mark-
edly improved again. His serum albumin level increased
to 35.2 g/L and calcium t o 1.9 mmol/L. However, the
rib fractures persisted.
After one-month of home nutritional support, he was
admitted to our hospital for the third time to improve
his nutritional status. His chest radiograph rev ealed
aggravating multiple ri b fractures (Figure 5). S ince he
did not complain of chest pain and respiratory distress
when walking or resting, we consulted the orthopedic
surgeons and chest surgeons again and received the
same treatment recommendations. After one-month
combination therapy, based on nutritional support, his
weight increased by 4.5 kg, but hypocalcaemia (calcium
level, 1.9 m mol/L), hypoalbuminemia (albumin level,
30.9 g/L) and multiple rib fractures still persisted.
Discussion
CCS has been reported over half a century [1]. The symp-
toms of CCS are characterized by the presence of ectoder-
mal abnormalities, including alopecia, onchodystrophy
and cutaneous hyperpigmentation, and they also a sso-
ciated with prominent clinical features, including diarrhea,
Figure 1 Endoscopic views. Esophagogastroduodenoscopy showed diffuse polypoid lesions extending from cardia to the first part o f the
duodenum. A, Cardia; B, Stomach; C, Duodenum.
Yuan et al. BMC Gastroenterology 2010, 10:121
http://www.biomedcentral.com/1471-230X/10/121
Page 2 of 5
weight loss, hypogeusia, anorexia and intestinal malab-
sorption [1,3]. In the present case as well as other reported
cases, endoscopic char acteristics of CCS were impressive
(Figures 1, 2 and 3) and histological examination of polyps
showed diversity in appearance: inflammatory, hyperplastic
and adenomatous types. Although CCS is considered a
benign condition, the occurrence of cancer in the stomach
and colorectum of CCS patients is about 13% (50/387),
and there is a possibility of serrated adenoma-carcinoma
sequence in colorectal cancer [4].
Figure 2 Endoscopic views. Video Capsule Endoscopy showed multiple herpes-like and strawberry-like polyps studded in most of the jejunum
and ileum. A and B, Jejunum; C and D, Ileum.
Figure 3 Endoscopic views. Colonoscopy identified numerous, hyperemic, sessile and pedunculated polyps in the colorectum. A, Descending
Colon; B, Transverse Colon.
Yuan et al. BMC Gastroenterology 2010, 10:121
http://www.biomedcentral.com/1471-230X/10/121
Page 3 of 5
Many complications such as fatal gastrointestinal
bleeding, intussusception, electrolyte abnormalities, pro-
tein-losing enteropathy, and severe acute pancreatitis
may occur with CCS, possibly contributing to poor out-
comes [3,5-8]. Inte restingly, the present case was compli-
cated by r ib fractures, which, to our kno wledg e, has not
been reported in the published literature. Since our
patient had no medical h istory of trauma, persistent
cough, chest pain and congenital disease, and our exami-
nations d id not establish the definite eti ology of the rib
fractures. In addition, low dosage prednisone for 24 days,
in our opinion, could not have resulted in the multiple
rib fract ures. Therefore, we presume that persistent
hypocalcaemia and malnutrition contribute to this situa-
tion, or at least aggravate this rare complication.
Although it is believed that no specific treatment h as
consistently resulted in definite improvement, current lit-
erature favors a combination therapy, which could induce
remission as seen in our patient, based on nutritional
support and corticosteroids [9]. Since prolonged corticos-
teroid therapy will result in an increased risk of osteo-
porotic fracture, CCS patients who accept corticosteroid
treatment should be monit ored for the potential ha rmful
complications such as rib fracture.
Although the overall mortality has been reported as
high as 55% a ccording to an early study [10], the prog-
nosis is now thought to be better than earlier case
reports, with improvement in medical treatment and
increased understanding of the syndrome.
Conclusions
This is the first case of a CCS patient with multiple rib
fractures. Although the association between CCS and
multiple rib fractu res in this case remains uncertain, we
presume that persistent hy pocalcaemia and malnutrition
contribute to this situation, or at least aggravate this
rare complication. Besides, s ince prolonged corticoster-
oid therapy will result in an increased risk of osteoporo-
tic fracture, CCS patients who acce pt corticosteroid for
treatment could be potential victims of rib fracture.
Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompany-
ing images. A copy o f the written consent is available
for review by the Editor-in-Chief of this journal.
Abbreviations
CCS: Cronkhite-Canada syndrome.
Acknowledgements
Written consent was obtained from the patient for publication of this paper.
The authors thank Atsushi Irisawa and Klaus Mönkemüller for their highly
constructive suggesti ons. No financial relationships with a commercial entity
producing health-care related products and/or services relevant to this
article.
Authors’ contributions
YB reviewed the literature and drafted the manuscript; JX, LH, SY and ZR
created figures, collected clinical data and analyzed data; LJ, JX, WH and LH
were involved in the care of the patient, WF performed the endoscopy; SY,
ZX and WF revised the manuscript. All authors read and approved the final
manuscript.
Figure 4 X-ray image. A chest radiograph showed fractures of the
left sixth and seventh ribs (arrow).
Figure 5 X-ray image. A chest radiograph showed multiple
fractures of the left fourth to seventh ribs (arrow) and right fifth to
ninth ribs (arrow).
Yuan et al. BMC Gastroenterology 2010, 10:121
http://www.biomedcentral.com/1471-230X/10/121
Page 4 of 5
Competing interests
The authors declare that they have no competing interests.
Received: 20 February 2010 Accepted: 18 October 2010
Published: 18 October 2010
References
1. Cronkhite LW Jr, Canada WJ: Generalized gastrointestinal polyposis; an
unusual syndrome of polyposis, pigmentation, alopecia and
onychotrophia. N Engl J Med 1955, 252:1011-1015.
2. Riegert-Johnson DL, Osborn N, Smyrk T, Boardman LA: Cronkhite-Canada
syndrome hamartomatous polyps are infiltrated with IgG4 plasma cells.
Digestion 2007, 75:96-97.
3. Ward EM, Wolfsen HC: Review article: the non-inherited gastrointestinal
polyposis syndromes. Aliment Pharmacol Ther 2002, 16:333-342.
4. Yashiro M, Kobayashi H, Kubo N, Nishiguchi Y, Wakasa K, Hirakawa K:
Cronkhite-Canada syndrome containing colon cancer and serrated
adenoma lesions. Digestion 2004, 69:57-62.
5. Yasuda T, Ueda T, Matsumoto I, Shirasaka D, Nakajima T, Sawa H,
Shinzeki M, Kim Y, Fujino Y, Kuroda Y: Cronkhite-Canada syndrome
presenting as recurrent severe acute pancreatitis. Gastrointest Endosc
2008, 67:570-572.
6. Isomoto H, Urata M, Nakagoe T, Sawai T, Nomoto T, Oda H, Nomura N,
Takeshima F, Mizuta Y, Murase K, Shimada S, Murata I, Kohno S: Proximal
extension of cap polyposis confirmed by colonoscopy. Gastrointest
Endosc 2001, 54:388-391.
7. Mizuno M, Matsumoto T, Iida M, Shimizu M: Magnifying colonoscopic
features in non-neoplastic polyps of the colorectum. Gastrointest Endosc
1997, 46:537-541.
8. Ward EM, Wolfsen HC: Pharmacological management of Cronkhite-
Canada syndrome. Expert Opin Pharmacother 2003, 4:385-389.
9. Chadalavada R, Brown DK, Walker AN, Sedghi S: Cronkhite-Canada
syndrome: sustained remission after corticosteroid treatment. Am J
Gastroenterol 2003, 98:1444-1446.
10. Daniel ES, Ludwig SL, Lewin KJ, Ruprecht RM, Rajacich GM, Schwabe AD:
The Cronkhite-Canada syndrome. An analysis of clinical and pathologic
features and therapy in 55 patients. Medicine 1982, 61:293-309.
Pre-publication history
The pre-publication history for this paper can be accessed here:
http://www.biomedcentral.com/1471-230X/10/121/prepub
doi:10.1186/1471-230X-10-121
Cite this article as: Yuan et al.: Cronkhite-Canada syndrome associated
with rib fractures: a case report. BMC Gastroenterology 2010 10:121 .
Submit your next manuscript to BioMed Central
and take full advantage of:
• Convenient online submission
• Thorough peer review
• No space constraints or color figure charges
• Immediate publication on acceptance
• Inclusion in PubMed, CAS, Scopus and Google Scholar
• Research which is freely available for redistribution
Submit your manuscript at
www.biomedcentral.com/submit
Yuan et al. BMC Gastroenterology 2010, 10:121
http://www.biomedcentral.com/1471-230X/10/121
Page 5 of 5
