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Pediatr Surg Int (2011) 27:399–405

DOI 10.1007/s00383-010-2808-x

ORIGINAL ARTICLE

Abdominal compartment syndrome in childhood: diagnostics,


therapy and survival rate
Gerhard Steinau • Torsten Kaussen •
Beate Bolten • Alexander Schachtrupp •
Ulf P. Neumann • Joachim Conze • Gabriele Boehm

Accepted: 10 November 2010 / Published online: 5 December 2010


! Springer-Verlag 2010

Abstract remaining six cases (21.4%) was sepsis with multiorgan


Purpose The abdominal compartment syndrome (ACS) in failure.
childhood is a rare but dire disease if diagnosed delayed and Conclusion Our results suggest that early establishment
treated improperly. The mortality amounts up to 60% (Beck of the specific diagnosis of ACS followed by swift therapy
et al. in Pediatr Crit Care Med 2:51–56, 2001). ACS is defined with reduction of intraabdominal hypertension is essential
by a sustained rise of the intraabdominal pressure (IAP) in order to further reduce the high mortality rate associated
together with newly developed organ dysfunction. The pres- with this condition.
ent study reports on 28 children with ACS to evaluate its
potential role in the diagnosis, treatment and outcome of ACS. Keywords Abdominal compartment syndrome !
Methods Retrospectively, medical reports and outcome Diagnostics ! Therapy ! Survival rate ! Children
of 28 children were evaluated who underwent surgical
treatment for ACS. The diagnosis of ACS was established
by clinical signs, intravesical pressure-measurements and Introduction
concurrent organ dysfunction.
Results Primary ACS was found in 25 children (89.3%) The abdominal compartment syndrome (ACS) is rare in
predominantly resulting from polytrauma and peritonitis. childhood and presents a life-threatening entity with a
Three children presented secondary ACS with sepsis (2 lethality of up to 60% [1]. The presence of inverse pressure
cases) and combustion (1 case) being the underlying ratios between thoracic and abdominal cavity was first
causative diseases. Therapy of choice was the decom- described by Marey in 1864 published by Light [2]. ACS is
pression of the abdominal cavity with implantation of an defined by a pathological increase of the intraabdominal
absorbable Vicryl" mesh. In 18 cases the abdominal cavity pressure (IAP) [3] with consecutive dysfunction of one or
could be closed later, while in the other ten cases granu- several organ systems leading to adverse hemodynamic,
lation of the mesh was allowed. The overall survival rate respiratory and renal effects [4]. Referring to the WSACS
was 78.6% (22 of 28 children). The cause of death in the definitions (http://www.WSACS.org), primary, secondary
and tertiary ACS are distinguished. Specific diagnostic
means and immediate therapeutic intervention are neces-
G. Steinau (&) ! B. Bolten ! A. Schachtrupp !
sary to reduce the IAP.
U. P. Neumann ! J. Conze ! G. Boehm
Department of Surgery,
University Hospital Aachen, Aachen, Germany
e-mail: gsteinau@ukaachen.de Materials and methods
J. Conze
e-mail: jconze@ukaachen.de In this retrospective study the medical records of all chil-
dren with diagnosis ACS (age under 18 years) between
T. Kaussen
Department of Pediatrics, 1998 and 2008 were examined. 28 children were identified
Dritter Orden Passau, Passau, Germany and enclosed.

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Statistics Prematurity and gestational age

The statistical evaluation was done using the statistical By definition, prematurity means birth before completion
software programme SAS. To the estimate of dimensions of 37th week of gestation (WOG). Within the age groups of
considering the censored data the LIFETEST routine the newborns and infants 3 (37.5%) of the children had
integrated in SAS was used. As a product limit assessor the been born before the 37th WOG. The most premature
method of Kaplan–Meier found use. newborn was born in the 23rd ? 3 WOG and the most
mature newborn during the 37th WOG.

Results Malformations and genetic disorders

Age distribution Medical histories of 26 (92.9%) delivered information about


the presence or absence of concomitant malformations or
The study group consisted of 28 children and was divided genetic disorders. 14 children (53.8%) showed none, and the
into four sub-groups dependent on their age (newborn, other 12 children (46.2%) at least one malformation or
babies, toddler and school children). Despite the newborn genetic disorder. With regard to the last-mentioned children,
life period lasts as short as 30 days postnatal, 10.7% of all 6 children (50%) had only one, 3 children (25%) two, and 3
investigated children belonged to the newborn group (3 of children (25%) three malformations or genetic disorders,
28 children; aged 19.2 days). As underlying cause in all respectively. An overview shows (Table 1).
these children necrotizing enterocolitis (NEC) was diag-
nosed. The group of babies (2–12th life month) comprised Diagnostics
five children (17.9%; aged 3.0 ± 1.2 months) suffered
from NEC, peritonitis, intestinal perforation and ileus as Within the first years of the above-mentioned observational
ACS-inducing entities. The toddlers (2nd–6th year) pre- period, the diagnosis of an ACS was made exclusively by
sented the smallest group of two children (7.1%, aged
28.5 ± 0.7 months), in which combustion and polytrauma Table 1 Number of malformations, respectively, genetical diseases
had led to ACS. The strongest group was the school chil-
Genetic disorder Frequency
dren (7th–18th year) with 18 (64.3%) children. The mean
age of this group was 12.9 (±3.6) years. Polytrauma, Cerebral
peritonitis, pancreatitis and intraabdominal bleeding had Meningomyelocele 1
led to the ACS (Fig. 1). Arnold-Chiari syndrome 1
Mental retardation of unknown cause 1
Gender distribution Pulmonary
Unilateral lung hypoplasia 1
The over-all gender distribution was even with 14 girls Cardiac
(50.0%) and 14 boys (50.0%). Within the age group of Persistent ductus botalli 3
newborns the female children with 66.7% were outbal- Ventricular septal defect 1
ancing the male (33.3%). Within the other age groups the Morbus fallot 1
female-to-male ratio was nearly even. Diaphragm
Unilateral diaphragmatic agenesia 1
Gastrointestinal tract
20 Mesenterium commune 2
Colonic atresia 1
16
Esophageal atresia Vogt IIIb 1
12 Gallbladder agenesia 1
Hirschsprung’s disease 1
8
Renal
4 Double renal system 2
Complex syndromes
0
newborn babies toddlers school OEIS 1
children Genetic disease
Mucoviscidosis 1
Fig. 1 Mean age within each of the four age groups

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Pediatr Surg Int (2011) 27:399–405 401

Table 2 Diseases and number which were caused for ACS


Primary
Peritonitis 7 Post appendectomy 3
Anastomotic leak following right hemicolectomy 1
Anastomotic leak following appendectomy 1
Traumatic small bowel perforation 1
Perforated appendicitis 1
Polytrauma 7 Nephrectomy, splenectomy 1
Splenic rupture, liver contusion 1
Retroperitoneal hematoma following rupture of left internal iliac vein and artery 1
Tearing of V. cava inferior, liver veins, splenic capsule 1
Splenektomie for splenic rupture, cholezystectomie, 1
liver rupture, retroperitoneal hematoma
Splenic and liver rupture, splenectomy, 1
Partial liver resection 1
Liver rupture grades 3–4 1
Perforation 2 Duodenal perforation due to bowel ischemia 1
Following resection of neuroblastoma
Ileal perforation following multiple laparotomies 4
NEC 4 With ischemia of small and large bowel 1
Pancreatitis 1 Following appendectomy
Intraabdominal bleeding, retroperitoneal hematoma 1 Anticoagulation after acute paraplegia 1
Ileus 3 Ileus due to adhesions following surgery for enterothorax 1
Following repair of volvulus and small bowel perforation 1
Adhesions 1
Secondary
Sepsis 2 Following abortion with sepsis 1
Empyema of the knee joint 1
Burn 1 8% burn of body surface area, IIa–b 1

clinical means as, for example, abdominal distension, aci- primary ACS 50% resulted from polytrauma and
dosis and otherwise unexplainable decrease in urine output, peritonitis, while 32.1% (9/28) were due to bowel
respectively. The bladder was filled via an urinary catheter perforation or ileus. In 7.1% of cases (2/28) the
with 1 ml of NaCl/kg bodyweight while the patient was underlying causes were pancreatitis (1/28) and intra-
positioned supine. A three-way stopcock was established and abdominal bleeding (1/28) leading to the appearance of
connected to an infusion set filled with NaCl in order to build retroperitoneal hematoma. Three children (10.7%)
a water column which served as indirect IAP indicator (scale: presented secondary ACS with sepsis (2 cases) and
mm; reference point: symphysis [=zero point]). If the IAP lay combustion (1 case) being the underlying diseases
repeatedly above 12 mmHg and at least one organ showed (Table 2).
dysfunction, a diagnosis of ACS was made. Preoperatively,
the mean IAD was 18.5 (±3.1) mmHg, with a postoperative Previous abdominal surgery
drop down to a mean of 9.3 (±1.2) mmHg. The diagnosis
‘‘ACS’’ was defined as repeatedly measured elevated intra- For 13 children (46.4%) decompressive surgery was their
abdominal hypertension ([12 mmHg) accompanied by at first intraabdominal intervention, while 15 children
least one organ dysfunction [6]. (53.6%) had undergone at least one abdominal operation
before. Eight of them (53.3%) had been previously oper-
Primary and secondary ACS ated once, four patients twice (26.7%) and three children
thrice (20%). Severity of operations was distinguished into
Primary ACS clearly predominated the study group and small, middle and major interventions (for details see
was found in 25 children (89.3%). Within the group of Table 3).

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Table 3 Classification in small, medium and major operations


Minor
5 Appendectomy
3 Second look
1 Abdominal closure
1 Exploration and refashioning of ileostoma
Middle
2 Evacuation of retroperitoneal hematoma
1 Revision for ileus and peritonitis
1 Adhaesiolysis
1 Hysterectomy
1 Appendectomy and adhaesiolysis
1 Partial jejunal resection
1 Closure of small bowel fistula Fig. 2 Implantation of a Vicryl" mesh in the abdominal wall by a
1 Debridement of burn wounds three months-aged girl after decompression laparotomy because of
ACS
1 Refashioning of jejunostomy
Major
8 Bowel resection day of admission, the other five within 9 days of
1 Repair of bladder exstrophy, cecal reconstruction and hospitalization.
ascendostoma
1 Right hemicolectomy Long-term results
1 Partial pancreatic necrosectomy, splenectomy
1 Excision of retroperitoneal neuroblastoma Median follow-up time was 6.8 years (range 2.2–10 years).
Further surgical interventions during follow-up included
Surgery incisional hernia repair 6 cases (27.3%), closure of a stoma
(one case) and relaparotomy for ileus due to adhesions (one
In all children ACS decompression of the abdominal cavity case).
was carried out by performing a transverse laparotomy and A questionnaire was sent to the parents of 22 surviving
creating a laparostoma by implantation of an absorbable children with a resulting response rate of 72.7% (16/22).
mesh, which was fixed to the fascial layer using an absorb- According to the answers given in the questionnaire the
able running suture (Fig. 2). Depending on the postoperative scar area keeps discomforting 10 of 16 children physio-
course, this mesh either was removed while reconnecting logically or psychologically (62.5%). Of those, subjective
fascial layers directly, or the mesh was left in situ until impediment degree was put at 100% by five children
granulation tissue had overgrown the whole implant. In 18 (31.3%), at 80% by one child (6.3%) and at 50% by two
children direct closure was carried out, the remaining ten patients (12.5%).
wounds healed by secondary intention. In two children an
additional split skin graft was necessary. The median time
until closure of the abdominal cavity could be performed was Discussion
53 days (range 10–63 days). In six children repeated
Vicryl" mesh reduction was needed to minimize the wound Few and just low-evidenced studies exist reporting exclu-
surface and tension prior to the final surgical closure. sively about ACS in childhood. Released results corre-
The most frequent complication (6/28) observed was spond to our findings, although a direct comparison is not
formation of an enterocutaneous fistula (21.4%). Fistulae possible on account of the different study designs [5–9].
were either oversewn or excised. In two children a mesh ACS occurs at any age in children. A division into different
tear occurred at the junction with the fascia and made re- age groups has not been carried out yet by any of the
adaptation necessary. named authors. All cited reports describe a predominance
of the male gender, being stated at 61.8%.
Lethality The presence of associated malformations in children
with ACS is mentioned only in single reports. Diaz found
22 of 28 children (78.6%) survived. Underlying cause of malformations in 40% of children with ACS [1, 5]. This is
death in the other six cases (22.4%) was sepsis with in accordance with our own results, which showed the
multi-organ failure (MOF). Two children died on the occurrence of malformations or genetic disorders to be

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Pediatr Surg Int (2011) 27:399–405 403

46.1%. Literature research [1, 5–9] revealed the primary than 12 mmHg combined with at least one organ
ACS to occur nearly twice as frequently as secondary and dysfunction.
tertiary ACS together (62.1 vs. 37.9%). In adults, the intravesical pressure measurement is
Retrospectively, further distinction between secondary considered as gold standard for the indirect IAP measure-
and tertiary ACS could not be worked out against the ment. Using this intermittent method which was first
background of insufficient data. Our own data revealed a described by Bertram et al. [4], a measuring volume of
higher ratio in favor of the primary ACS with a share of 50 ml sterile saline is inserted. Against that, the recom-
89%. Due to our complete dataset a differentiation between mended volumes in children vary between 1 ml/kg [13–15]
primary and secondary ACS was well possible. In the lit- and 2 ml/kg of body weight [16]. Concerning our study a
erature only few studies did carry out a division into pri- filling amount of 1 ml/kg of body weight was enough to
mary and secondary ACS [5–9]. reliably determine the IAP. This is in accordance with data
Primary ACS defines all forms of ACS resulting from from Suominen who proved high correlation to the directly
intraabdominal diseases and entities. Against that, sec- measured IAP when filling the bladder with 1 ml/kg of
ondary ACS derives from disarrangements arising from body weight [15]. As soon as measurements are performed
outside the abdominal cavity including pneumonia, extra- in fixed or traumatized bladders untrustworthy pressure
abdominal trauma, burns with capillary leak syndrome or heads result [12]. Other situations rendering this technique
the need of high-fluid resuscitation, for example [10]. unreliably are body positions other than supine, pre-exist-
Tertiary ACS is defined as recurrent ACS following an ing peritoneal adhesions or preceding abdominal packing
initially successful treatment of primary ACS, e.g. after [12, 17]. Possible risks of bladder pressure measurements
decompressive laparotomy [4]. frequently pointed out by critics are damage and infection
Concerning the diagnosis of ACS or intraabdominal of the urinary tract. Nevertheless, Malbrain found the
hypertension (IAH) no generally valid approach exists infection risk not to be raised when using a closed mea-
which was demonstrated by data resulting from a ques- suring system with three-way stopcock [12].
tionnaire sent to members of the Society of Critical Care A huge number of causes are considered to be causative
[11]. 20% of the responders base their diagnosis IAH for the development of IAH and ACS. Our own evaluation
exclusively on clinical signs, 7.2% strictly use intravesical revealed peritonitis and polytrauma to be predominant with
pressure-measurements and about 70% a combination of each 25%. This is noteworthy in so far as in the present
both. The remaining 2.8% use other methods. According to literature peritonitis was not looked upon as an origin of an
the WSACS (World Society of the Abdominal Compart- ACS, yet. This might be explain by the fact that authors all
ment Syndrome) an ACS is defined as rise of the IAP illnesses which first lead to peritonitis and after that to ACS
above 20 mmHg with or without an APP (abdominal per- deem causative for the development of IAH and ACS. In
fusion pressure) less than 60 mmHg in combination with a their opinion, also peritonitis is just a consequence of
new onset of organ dysfunction [12, 13]. There is no evi- another disarrangement and builds an intermediate stage on
dence whether the above-mentioned criteria also apply for the way to an ACS. Polytrauma as an underlying cause for
physiological conditions in children. Several authors sug- ACS was also mentioned by other investigators: deCou
gest IAH-limits as low as 10–15 mmHg to be detrimental reported three exemplary cases [18] and Neville found a
in childhood and to build the critical pressure head at which share of 13% being traumatized before developing an ACS
regularly a switch from IAH to ACS in pediatric patients [9]. Beck [1] analyzed medical records of 1762 pediatric
can be observed [1, 5, 6]. According to Eijke [6] we patients who were sent to ICU and revealed a trauma-
therefore defined pediatric ACS as a lasting IAH higher induced ACS-incidence of 0.7% related to patients who

Table 4 Causes for primary ACS


Beck [1] DeCou [18] Diaz [5] Eijke [6] Kawar [19] Neville [9] Aachen

Abd. bleeding 2
Bowel perforation 1 2 4 2
Ileus 1 2 1 3
Abd. trauma 1 2 1 1 7
Abd. tumor 2 2 1 1
Peritonitis excluding bowel perforation 4 1 1 7
NEC/colitis 1 1 15 4
Pancreatitis 1

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Table 5 Causes for secondary ACS


Beck [1] DeCou [18] Diaz [5] Eijke [6] Jensen [16] Maxwell [8] Morrell [20] Aachen

Cerebral disease 4
Polytrauma 1 3 1 1 1
Combustion 2 3 1
Sepsis 3 3 2
Cardiac disease 2
Pneumonia 1

Fig. 3 Flow sheet for


Surveillance of patients at risk for 12 h, intervals at 1-2 h
monitoring in risk patients

>20 mmHg
<12 mmHg 12-15 mmHg 16-20 mmHg and
or
No organ dysfunction >16 mmHg
with organ
dysfunction
24 h IAD
monitoring: conservative therapy and IAP-monitoring
every 4-6 h

ACS

<12 mmHg >12 mmHg Secondary or


primary
tertiary

Stop Start yes conservative


monitoring at top operative
therapy
decompression
exhausted?

no

no
success conservative therapy
Monitor IAP every 4 h
Aim: APP >60 mmHg
successful

<20 mmHg > 20 mmHg

Attempt
abdominal closure

<12 mmHg >12 mmH g

stop
measurement

have had an accident (3/406) and an incidence of 0.2% to be the main origin (56.6%) [9]. According to the sec-
based on all patients admitted to the ICU (3/1762). Of all ondary ACS, a comparably wide range of possibly under-
23 children who developed ACS and had to be decom- lying reasons has been published. Summarized infection
pressed by laparotomy in his cohorte, Neville stated NEC and sepsis account for the vast majority of perpetrators,

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Pediatr Surg Int (2011) 27:399–405 405

followed by combustion and trauma (Tables 4, 5). The 8. Maxwell RA, Fabian TC, Croce MA, Davis KA (1999) Sec-
diagnosis of an ACS requires the appropriate clinical signs ondary abdominal compartment syndrome: in underappreciated
manifestation of serve hemorrhagic shock. J Trauma 47:995–999
and a rise in bladder pressure above 12 mmHg [4, 13]. 9. Neville HL, Lally KP, Cox CS (2000) Emergent abdominal
Decision making after recognition of ACS was facili- decompression with patch abdominoplasty in the pediatric
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Fox P, Pallua N (2005) Ischaemic necrosis of small and generous
Kimball and DeLaet. The most important difference intestine in a 2-year old child with 20% partial thickness burns.
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cerning the therapy of choice in cases of ascertained ACS, Johnston C, Day IT, Jackson PR, Payne M, Barton RG (2006)
Survey of intensive care physicians on the recognition and
both authors argument controversially. While Kimball management of intra abdominal hypertension and abdominal
recommends an immediate decompression when ACS is compartment syndrome. Crit Care Med 34:2340–2348
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