correspondence

Correspondence1942

Re: A population-based study of congenitaldiaphragmatic hernia outcome in New South Wales andthe Australian Capital Territory, Australia, 1992-2001

Dear Editor,

In the recent article bA population-based study of

congenital diaphragmatic hernia outcome in New South

Wales and the Australian Capital Territory, Australia, 1992-

2001,Q the overall survival to hospital discharge was

reported at 56%.

As the Grace Centre for Newborn Care at The Child-

ren’s Hospital at Westmead and the Sydney Children’s

Hospital at Randwick, Sydney, were the 2 major partici-

pating surgical neonatal intensive care units, we have

reviewed the survival to hospital discharge of infants with

congenital diaphragmatic hernia in the last 5 years. We

examined the 2 epochs, from 1996 to 2000 and from 2001

to 2005. This revealed an increase in survival at the

Children’s Hospital at Westmead of these infants from 63%

to 82% (22/35 vs 32/39; v2 = 3.45; P = .06). Of interest,

this increase is also replicated at Sydney Children’s

Hospital (32/39). This increase in survival was in keeping

with published survival rates [1] and may be related to

changing ventilation practices [2].

Karen Walker

Department of Neonatology

The Children’s Hospital at Westmead

Sydney, NSW 2145, Australia

URL: www.chw.edu.au

Nadia Badawi




Department of Paediatrics

The University of Sydney


John Levison

Robert Halliday




Andrew J.A. Holland

Department of Academic Surgery


The University of Sydney


Gary Williams

Department of Paediatric Intensive Care

The Sydney Children’s Hospital at Randwick

Randwick, NSW 2031, Australia

Edward Shi

Department of Surgery

The Sydney Children’s Hospital at Randwick

Randwick, NSW 2031, Australia

doi:10.1016/j.jpedsurg.2006.09.004

References

[1] Colvin J, Bower C, Dickinson JE, et al. Outcomes of congenital

diaphragmatic hernia: a population-based study in Western Australia.

Pediatrics 2005;116(3):e356-63.

[2] Bohn D. Congenital diaphragmatic hernia. Am J Respir Crit Care Med

2002;166(7):911 -5.

To the Editor,

We read with great interest the article by Arbell et al

[1]. The points related to the problem of malrotation are

well taken. However, the discussion of Hirschsprung left

us with a significant number of doubts that we feel

deserve comment.

In our series of more than 1930 patients with anorectal

malformations, we frequently found a lack of ganglion cells

in the specimen of the most distal part of the bowel at the

point where the bowel is attached to the urinary tract

(fistula). This finding had absolutely no clinical implication.

During the last 25 years, we only have documented 3 cases

of real Hirschsprung disease associated with an anorectal

malformation (of 2000 operative and more than 5000

nonoperative patients seen in consultation, an incidence

similar to the general population). On the other hand,

constipation is a consistent problem in patients operated for

anorectal malformations.

As everybody knows, constipation does not necessarily

mean Hirschsprung disease. Patients with Hirschsprung

disease behave in a completely different way; they are prone

to develop enterocolitis when one attempts to produce stasis,

like in operations with patches or pouches that may work in

a patient without Hirschsprung (total colectomies after

familial polyposis or ulcerative colitis) but do not work as

well in patients with Hirschsprung. Patients with Hirsch-

sprung disease also often suffer from failure to thrive,

whereas constipated patients never seem to.

The authors did not mention the extent of the aganglio-

nosis, and they did not comment on other important

histologic findings relevant to the histologic diagnosis of

Hirschsprung disease (hyperthropic nerves and increased

activity of acetylcholinesterase). We wonder if the pathol-

ogy they observed is consistent with the pathology of any

fistula site specimens and not Hirschsprung at all.

The authors used the transition zone for the anastomosis,

with the specific goal of producing stasis, because the

patient suffered from chronic diarrhea. That kind of

Correspondence 1943

procedure in a patient with Hirschsprung usually results

in enterocolitis.

Sincerely,

Alberto Pena

Marc Levitt

Cincinnati Childrens Hospital Medical Center

Cincinnati, OH 45229, USA

E-mail addresses: [email protected]

[email protected]

doi:10.1016/j.jpedsurg.2006.09.003

Reference

[1] Arbell D, Gross E, Orkin B, et al. Imperforate anus, malrotation, and

Hirschsprung’s disease: a rare and important association. J Pediatr Surg

2006;41:1335-7.

Reply to Dr Pena and Dr Levitt

To the Editor,

We would like to thank Dr Pena and Dr Levitt for their

sincere questions and kind remarks. In the case described,

Hirschsprung disease was actively sought after because of

the malrotation and imperforate anus. The aganglionosis

with hypertrophic nerves (acetylcholine esterase was not

examined) extended for about 4 to 5 cm proximal to the

fistula, with frozen sections at operation confirmed by later

paraffin specimens. Therefore, this does not seem to be the

case of fistula site specimen.

As mentioned in the article, the child suffered from short

bowel including loss of a significant part of his colon. As

Dr Pena teaches, a constipated child with anorectal mal-

formation is easier to treat than a child with diarrhea, and

that was the reason the transitional zone, as seen by frozen

section pathology, was chosen. In fact, after the operation

(as mentioned in the article), the child suffered from

diarrhea that gradually resolved.

As Dr Pena and Dr Levitt noted, the coexistence of

Hirschsprung disease with anorectal malformation is rare,

but it exists. We hope we have satisfied their valid questions

about verifying true Hirschsprung disease in this case.

Because they have vast and unparalleled experience in these

cases, we would like to learn from them on what happens in

postoperative cases where an aganglionotic segment is used

for anorectal malformation repair.

Sincerely,

Dan Arbell

Raphael Udassin

Department of Pediatric Surgery

Hadassah University Hospital

PO Box 12000

11-91120 Jerusalem, Israel

Email address: [email protected]

doi:10.1016/j.jpedsurg.2006.09.002

correspondence

Documents