Journal of the Royal Society of Medicine Volume 79 July 1986

406

Familial retrocaecal appendicitis

Y Shperber MD

A Halevy MD

J Oland MD

R Orda MD MS

Department of Surgery 'A', Assaf Harofeh Medical Center, Zerifin, Israel

Keywords: appendicitis, familial

Summary
Four members of the same family operated on for
acute retrocaecal appendicitis are reported. It is
suggested that they provide further support for the
hypothesis that an hereditary factor might be
involved in the pathogenesis of acute appendicitis.

Introduction
Acute appendicitis is the most common acute condition of the abdomen that requires surgical intervention. However, the occurrence of retrocaecal
appendicitis in members of the same family is a
rare event. The appearance of this phenomenon in
certain families may suggest the involvement of
some genetic factors rather than simple coincidence.
One such family is reported here.

Figure 1. Male (U) and female (0) family members affected

with retrocaecal acute appendicitis

Case reports (Figure 1)

Case 1: MB, the grandmother of the family, aged 62,
was admitted to hospital with a 24-hour history of
a right lower quadrant abdominal pain, fever and
mild leukocytosis. Upon physical examination, the
abdomen was found to be diffusely tender, and
guarding was most marked in the right lower quadrant. A tentative diagnosis of acute appendicitis was
made. Exploratory surgery revealed a retrocaecal
inflamed appendix, and an appendicectomy was
performed.
Case 2: LB, the granddaughter aged 13, was admitted
with a 10-hour history of a right lower quadrant

abdominal pain, fever and leukocytosis. Physical

examination revealed signs of peritoneal irritation
in the right lower quadrant. At operation a retrocaecal, acutely inflained appendix was found and
removed.
Case 3: EB, the grandson aged 4, was admitted with
abdominal pain of several hours duration, vomiting
and fever. On physical examination tenderness and
guarding were felt, especially in the right lower
quadrant. At operation a retrocaecal inflamed
appendix was found and removed.

Case 4: GB, another grandson, aged 10 and previously healthy, was admitted to hospital complaining of abdominal pain mostly in the right lower
quadrant and fever. Physical examination revealed
a tender abdomen, especially the right lower quadrant. Again, at operation, a retrocaecal inflamed
appendix was found and removed.

Discussion
Acute appendicitis is a very common surgical emergency. It occurs mainly in the second and third
decades of life and accounts for 1-2% of all surgical
operations'. Despite its frequent occurrence, the
precise cause of acute appendicitis still remains a
matter of controversy. Most authors agree that the
primary event is the obstruction of the lumen, followed by an inflammatory process2 s. However, it is
very difficult to establish the existence of any predisposing factor which promotes such a condition.
Baker6 described a family pedigree in which 50%
of its members were operated on for appendicitis.
Anderson et al.7 and also Arnbjornsson8 showed a
high incidence of appendicitis among immediate
family members (siblings, parents or both), a tendency not seen in a control group that underwent
abdominal operations other than appendicectomy.
The familial tendency to acute appendicitis may
perhaps be explained by environmental factors such
as a specific bacterial infection, certain food habits7,
or, as Baker6 suggested, a genetic difference in resistance to bacterial infection. Baker, however, did
not offer any scientific explanation to support this
statement.

Another point was raised by Downs9 in reviewing

a family of 22 individuals in whom appendicectomies
0141-0768/86/
had been performed: in 16 instances the appendix 070405-02/$02.00/0
was found to be kinked at its base by a fibrous band, o 1986
and Downs suggested that this peritoneal band The Royal
might be inherited. Although the presence of such a Society of
congenital malformation alone is not sufficient to Medicine

406

Journal of the Royal Society of Medicine Volume 79 July 1986

precipitate an attack of acute appendicitis, it could

well be considered as a predisposing factor in that it
represents the mechanism by which the lumen of the
appendix could be obstructed.
Some of the families reviewed by Perry and
Keeler"0 also showed various anomalies of the
appendix, such as a missing section of mesoappendix
or morphological and anatomical variations including a coiled, short, long or retrocaecal appendix.
They described a family pedigree containing 39
individuals who had suffered from appendicitis and
were operated upon; in 9 of them the appendix was
located retrocaecally.
Budd and Fonty" described a family pedigree in
which 15 individuals had an inflamed, retrocaecal
Eppendix, and concluded that the retrocaecal location is inherited as a simple dominant unitcharacter. Perry and Keller10 also suggested that
anatomical variations of the appendix might be
inherited as a simple dominant unit-character.
In the family reported here, the grandmother, two
grandsons and a granddaughter all suffered from
retrocaecal appendicitis. In the light of our, and
other previously reported studies, it would seem that
in certain cases some hereditary factors are involved
in the pathogenesis of acute appendicitis. Further
research is necessary to assess the exact value of
such genetic factors.

References
1 Condon RE. Appendicitis. In: Christopher D, Sabiston
DC, eds. Textbook of surgery, vol. 1. 12th ed. London:
WB Saunders 1981:1048:63
2 Wangesteen OH, Bowers WF. Significance of the obstructive factor in the genesis of acute appendicitis.
Arch Surg 1939;34:496-504
3 Wangesteen OH, Dennis C. Experimental proof of the
obstructive origin of appendicitis in man. Ann Surg
1939;110:629-33
4 Pieper R, Kager L, Tidefeldt U. Obstruction of appendix vermiformis causing acute appendicitis. Acta Chir
Scand 1982;148:63-72
5 Arnbjornsson E, Bengmark S. Role of obstruction in
the pathogenesis of acute appendicitis. Am J Surg
1984;147:390-2
6 Baker EGS. A family pedigree for appendicitis. J
Hered 1937;28:187-91
7 Andersson N, Griffiths H, Murphy J, et al. Is appendicitis familial? Br Med J 1979;ii:697-8
8 Arnbjornsson E. Acute appendicitis; a familial disease?
Curr Surg 1982;39:18-20
9 Downs TM. Congenital malformations of the appendix
- a familial disease. Ann Surg 1942;115:21-4
10 Perry T, Keeler CE. Thirty-nine cases of appendicitis
in a single family pedigree. Am JSurg 1939;46:259-65
11 Budd DC, Fonty WJ. Familial retrocaecal appendicitis. Am J Surg 1977;133:670-1

(Accepted 23 January 1986)