Iatrogenic ileal perforation: An accidental clamping of ahernia into the umbilical cord and a review of thepublished work

Koushi Asabe1,2, Yoichiro Oka1,2, Hiroki Kai1,2 and Takayuki Shirakusa1

1Department of Surgery, Division of Thoracic, Endocrine and Pediatric Surgery, Fukuoka University School of Medicine, and2Division of Pediatric Surgery, Maternity and Perinatal Care Center, Fukuoka University Hospital, Fukuoka, Japan

Abstract

This report describes the case of an ileal perforation secondary to clamping of the exomphalos minor in3-day-old girl. Eighteen accidental clamping or cutting cases were found in the published work. It is thoughtthat this defect resulted from either the clamping, ligation or cutting of the bowel in a small unrecognizedomphalocele. These complications are extremely rare, but should be held in mind when performing anumbilical ligation.Key words: complication, exomphalos minor, umbilical cord clamping.

Introduction

Omphalocele is an uncommon condition in whichthe abdominal viscera remain herniated through theumbilical portion of the abdominal wall into a mem-brane.1 The incidence is one in 6000–10 000 births2,3 andthe condition can be either major or minor, dependingon the size of defect.3 The anterior midline defect of theabdominal wall defect may vary in size. The diameterof the defect of a hernia into the umbilical cord isnearly always less than 4 cm, and the diameter of thesac of such a hernia contains loops of the small bowelonly. Jones subdivided exomphalos into defects with adiameter smaller than 2.5 cm, defects with a diameterof 2.5–5 cm and defects with a diameter larger than5 cm.4 A subdivision into small and large exomphalosbased on the absence or presence of liver in the herniasac has also been established.5 Sometimes the presenceof a small omphalocele may be missed, and in suchcircumstances a clamp, ligature or cutting appliedacross the omphalocele close to the abdominal wallmay transect a loop of intestine.6 Eighteen complicatedcases arising from the incorrect placement of clamps or

cutting incorporating exomphalos minor similar to thepresent case have been described (Table 1).1,3,6–14

Case Report

A 3-day old girl was referred with a 5–6-h history offluid discharge similar to intestinal juice from umbili-cal cord and a 1-day history of bilious vomiting thatwas associated with abdominal distention. The infanthad been born at 37 gestational weeks by spontaneousvaginal delivery and weighed 1995 g. The Apgar scoreswere 8 at both 1 and 5 min. The pregnancy had beenunremarkable with no history of polyhydramniosexcept for cleft lips. The karyotype was 46.XX. Theenlargement at the base of the umbilical cord wasligated using a silk ligature. An umbilical ligation ringwas also used at approximately 3 cm from the enlarge-ment base of the umbilical cord. The pinhole fromwhich a fluid similar to intestinal juice was dischargedwas noted among those ligations (Fig. 1). Fistulogra-phy demonstrated the fistula to be connected to thesmall intestine (Fig. 2). Preoperatively, the diagnosiswas a small omphalocele with division of an entrapped

Received: May 11 2007.Accepted: January 26 2008.Reprint request to: Dr Koushi Asabe, Division of Pediatric Surgery, Maternity and Perinatal Care Center, Fukuoka UniversityHospital, 7-45-1 Nanakuma, Jonan-ku, Fukuoka 814-0180, Japan. Email: asabe@fukuoka-u.ac.jp

doi:10.1111/j.1447-0756.2008.00799.x J. Obstet. Gynaecol. Res. Vol. 34, No. 4 Part II: 619–622, August 2008

© 2008 The Authors 619Journal compilation © 2008 Japan Society of Obstetrics and Gynecology

perforated small intestine. An emergency laparotomywas performed under general anesthesia. A semicircu-lar incision around the base of the umbilical cordrevealed a peritoneal sac containing an ileum measur-ing approximately 5 cm in length which was entrappedin the clamped small omphalocele. The diameter of the

Tab

le1

Acc

iden

talc

lam

ping

orcu

ttin

gof

hern

iain

toth

eum

bilic

alco

rd

Cas

eno

.A

utho

rYe

arSe

xG

esta

tion

alw

eeks

Age

(day

s)B

irth

wei

ght

(g)

Sym

ptom

sM

anag

emen

tof

cord

Out

com

e

1H

aulta

in19

32–

––

––

Cut

ting

Die

d2

––

––

–C

utti

ngD

ied

3H

olle

nber

gan

dR

ock7

1948

––

5–

Mor

ibun

dfr

omin

test

inal

obst

ruct

ion

–D

ied

4–

–0

––

Stum

pL

ived

5L

and

oret

al.8

1963

F–

6–

Abd

omin

ald

iste

ntio

nTa

peL

ived

6M

–4

–B

iliou

svo

mit

ing,

abd

omin

ald

iste

ntio

n–

Die

d7

Eck

stei

n919

63–

––

––

––

8–

––

––

––

9Va

ssy

and

Bol

es5

1975

M–

3–

Bili

ous

vom

itin

g,ab

dom

inal

dis

tent

ion

Stum

pL

ived

10F

–1

–A

bdom

inal

dis

tent

ion

Stum

pL

ived

11Ya

day

and

Men

gi10

1981

M–

224

00B

iliou

svo

mit

ing,

abd

omin

ald

iste

ntio

nL

igat

ure

Liv

ed12

M–

427

00B

iliou

svo

mit

ing,

abd

omin

ald

iste

ntio

nL

igat

ure

Liv

ed13

Yam

asat

oet

al.12

1987

M39

336

78B

iliou

svo

mit

ing,

abd

omin

ald

iste

ntio

nSt

ump

Liv

ed14

Cha

ndra

etal

.119

89–

––

––

Lig

atur

eL

ived

15–

–4

––

Lig

atur

eL

ived

16C

ham

pman

-She

ath

etal

.319

96M

–3

2070

Bili

ous

vom

itin

g,ab

dom

inal

dis

tent

ion

Stum

pL

ived

17W

atan

abe

etal

.1320

07F

2519

798

Abd

omin

ald

iste

ntio

nR

ing

Liv

ed18

Pres

ent

case

2007

F37

319

95B

iliou

svo

mit

ing,

abd

omin

ald

iste

ntio

nL

igat

ure

and

clip

Liv

ed

Figure 1 The base of the umbilical cord was ligated usinga silk ligature. An umbilical ligation clip was also usedapproximately 3 cm from the enlarged base of theumbilical cord. The pinhole from which the fluidsimilar to intestinal fluid was discharged was notedamong those ligations.

Figure 2 Fistulography demonstrated the fistula to beconnected to the small intestine.

K. Asabe et al.

620 © 2008 The AuthorsJournal compilation © 2008 Japan Society of Obstetrics and Gynecology

abdominal wall defect was approximately 1.5 cm. Thenecrotic and perforated ileum in the omphalocele wasremoved. After resecting the affected ileum, end-to-end anastomosis was done. The umbilical defect wasclosed. Oral feeding was thereafter begum on theseventh day and was also gradually increased to anormal formula diet. The patient’s recovery wasuncomplicated.

Discussion

The definition for the conditions in which some portionof the viscera of the newborn infant lies outside theanterior abdominal cavity – that is, an exomphalos oromphalocele – is usually accepted as being a herniationof abdominal contents through an open umbilical ringinto the base of the umbilical cord3,4 and covering ofthese viscera with a membrane sac. However, gas-troschisis is a condition in which the principal featuresare the extra-umbilical localization of the defect andthe absence of a membranous sac covering the eviscer-ated mass of the intestines.5

As the fetus grows in pregnancy, the intestines growand get longer and project from the abdomen into theumbilical cord. This growth takes place from the sixthto the tenth week of pregnancy. Normally, the intes-tines return rapidly into the abdomen by the eleventhweek of pregnancy. If this fails to happen, then anomphalocele can develop.

Exomphalos major or minor is an easily recognizedanomaly. The umbilical cord arises from the apex orinferior surface of the intact membrane. If the ompha-locele is occult or too small, it may be overlooked andin such circumstances a ligature which is applied closeto the abdominal wall may occlude or transect a loop ofthe intestine and thus create an iatrogenic acute intes-tinal obstruction or fecal fistulae at the umbilicus.11 Thenormal full-term umbilical cord measures 45–75 cm,with a mean of approximately 60 cm and a diameter of1–2 cm.13 In fact, the hernia ring of the present case wasnormal size, and it was thus extremely difficult to rec-ognize such a hernia into the umbilical cord. Eighteensuch cases prior to the present case have been reportedin published work1,3,6–14 and it is emphasized that anenlargement of the cord at its base should alert thephysician to the possibility of a small omphalocele andthat the cord should be clamped, tied or cut distal toany such swelling. Landor et al. revealed two cases ofomphalocele in which the ileum was accidentallyclamped, thus causing an intestinal obstruction.9 Eck-stein reported 100 cases of omphalocele. His series also

included one infant with an appendical–umbilicalfistula due to the clamping of the appendix along witha small unrecognized omphalocele.10

The sex distribution of previous cases showed sixboys, four girls and eight unknown. The age at the timeof operation ranged 0–19 days. The symptoms of mostcases revealed abdominal distention (10 cases) andbilious vomiting (seven cases). The methods of cordclumping included five stumps, five ligatures, one tap,one ring and one clip. Haultain observed two caseswhere several loops of small bowel had been cut acrosswith cutting of the cord.7 The portions of the entrappedalimentary tract into the clamped small omphaloceleincluded 14 small intestines with eight ileums, twoMeckel’s diverticula, one appendix and one ileocecal.Four deaths were reported among the 16 patientsexcluding two with an unknown outcome. These fatalcases were all reported before 1963. Since then, allsimilar cases have been reported to survive (Table 1).

Some investigators emphasize that, in minor formsof exomphalos, the diagnosis is not always immediatelyobvious. The presence of an unusually large, thick orbroad umbilical cord should immediately alert physi-cians to the possibility that a small or occult ompha-locele may be present.3,6,9,11 They asserted that theumbilical cord should be routinely clamped at least5 cm from the abdominal wall to prevent any possibil-ity of iatrogenic damage to the bowel.3,6,11 Therefore, insuch instances the umbilical clamp, tie or cut should beplaced distal to the swelling and efforts be made toobtain a definitive diagnosis without delay.

References

1. Chandra S, Bhatnagar V, Rohatgi M. Management of ompha-locele with bowel pathology-primary or iatrogenic. IndianPediatr 1989; 26: 713–715.

2. Stein JL, Gerber A. Congenital omphacele: A report of fourcases. J Pediatr 1939; 14: 89–91.

3. Champman-Sheath P, Wilcox D, Mok Q, Drake D. Lesson ofthe week: Iatrogenic ileal obstruction: A complication ofumbilical cord clamping. BMJ 1996; 313: 613–614.

4. Jones PG. Exomphalos (Syn. Omphalocele). A review of 45cases. Arch Dis Child 1963; 38: 180–187.

5. Bax NMA. Exomphalos and gastroschisis. In: Freeman NV,Burge DM, Griffiths DM, Malone PSJ (eds). Surgery of theNewborn, 1st edn. New York: Churchill Livingstone, 1994;301–320.

6. Vassy LE, Boles ET. Iatrogenic ileal atresia secondary toclamping of an occult omphalocele. J Pediatr Surg 1975; 10:797–800.

7. Dott NM. Clinical record of a case of exomphalos, illustratingthe embryonic type and its surgical treatment. Edinburgh MedJ 1932; 39: 105–108.

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8. Hollenberg HG, Rock L. Amniotic hernia. Surgery 1948; 23:363–368.

9. Landor JH, Armstrong JH, Dickerson OB, Westerfeld RA.Neonatal obstruction of bowel caused by accidental clampingof small omphalocele: Report of two cases. South Med J 1963;56: 1236–1238.

10. Eckstein HB. Exomphalos – A review of 100 cases. Br J Surg1963; 50: 405–410.

11. Yaday K, Mengi Y. Iatrogenic obstruction of occult omphalo-cele. Indian Pediatr 1981; 18: 832–835.

12. Rushton DI. Pathology of placenta. In: Wigglesworth JS,Singer DB (eds). Textbook of Fetal and Perinatal Pathology, 1stedn. Boston: Blackwell Scientific Publications, 1991; 161–219.

13. Yamasato M, Hokama A, Muto Y et al. Intestinal-umbilicalfistula secondary to accidental clamping of an occult ompha-locele. A case report. Ryukyu Med J 1987; 10: 112–115.

14. Watanabe M, Komuro H, Kaneko M et al. Ileal obstructioncaused by usage of an umbilical ligation ring in an extremelylow-birth-weight infant: A case report. J Jpn Soc Pediatr Surg2007; 43: 58–61.

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622 © 2008 The AuthorsJournal compilation © 2008 Japan Society of Obstetrics and Gynecology