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 Ó Springer-Verlag 2010
Abstract Purpose The abdominal compartment syndrome (ACS) in childhood is a rare but dire disease if diagnosed delayed and treated improperly. The mortality amounts up to 60% (Beck et al. in Pediatr Crit Care Med 2:51–56, 2001). ACS is defined by a sustained rise of the intraabdominal pressure (IAP) together with newly developed organ dysfunction. The present study reports on 28 children with ACS to evaluate its potential role in the diagnosis, treatment and outcome of ACS. Methods Retrospectively, medical reports and outcome of 28 children were evaluated who underwent surgical treatment for ACS. The diagnosis of ACS was established by clinical signs, intravesical pressure-measurements and concurrent organ dysfunction. Results Primary ACS was found in 25 children (89.3%) predominantly resulting from polytrauma and peritonitis. Three children presented secondary ACS with sepsis (2 cases) and combustion (1 case) being the underlying causative diseases. Therapy of choice was the decompression of the abdominal cavity with implantation of an absorbable VicrylÒ mesh. In 18 cases the abdominal cavity could be closed later, while in the other ten cases granulation of the mesh was allowed. The overall survival rate was 78.6% (22 of 28 children). The cause of death in the G. Steinau (&) B. Bolten A. Schachtrupp U. P. Neumann J. Conze G. Boehm Department of Surgery, University Hospital Aachen, Aachen, Germany e-mail:
[email protected] J. Conze e-mail:
[email protected] T. Kaussen Department of Pediatrics, Dritter Orden Passau, Passau, Germany
remaining six cases (21.4%) was sepsis with multiorgan failure. Conclusion Our results suggest that early establishment of the specific diagnosis of ACS followed by swift therapy with reduction of intraabdominal hypertension is essential in order to further reduce the high mortality rate associated with this condition. Keywords Abdominal compartment syndrome Diagnostics Therapy Survival rate Children
Introduction The abdominal compartment syndrome (ACS) is rare in childhood and presents a life-threatening entity with a lethality of up to 60% [1]. The presence of inverse pressure ratios between thoracic and abdominal cavity was first described by Marey in 1864 published by Light [2]. ACS is defined by a pathological increase of the intraabdominal pressure (IAP) [3] with consecutive dysfunction of one or several organ systems leading to adverse hemodynamic, respiratory and renal effects [4]. Referring to the WSACS definitions (http://www.WSACS.org), primary, secondary and tertiary ACS are distinguished. Specific diagnostic means and immediate therapeutic intervention are necessary to reduce the IAP.
Materials and methods In this retrospective study the medical records of all children with diagnosis ACS (age under 18 years) between 1998 and 2008 were examined. 28 children were identified and enclosed.
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Statistics
Prematurity and gestational age
The statistical evaluation was done using the statistical software programme SAS. To the estimate of dimensions considering the censored data the LIFETEST routine integrated in SAS was used. As a product limit assessor the method of Kaplan–Meier found use.
By definition, prematurity means birth before completion of 37th week of gestation (WOG). Within the age groups of the newborns and infants 3 (37.5%) of the children had been born before the 37th WOG. The most premature 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 genetic disorders. 14 children (53.8%) showed none, and the other 12 children (46.2%) at least one malformation or genetic disorder. With regard to the last-mentioned children, 6 children (50%) had only one, 3 children (25%) two, and 3 children (25%) three malformations or genetic disorders, respectively. An overview shows (Table 1).
The study group consisted of 28 children and was divided into four sub-groups dependent on their age (newborn, babies, toddler and school children). Despite the newborn life period lasts as short as 30 days postnatal, 10.7% of all investigated children belonged to the newborn group (3 of 28 children; aged 19.2 days). As underlying cause in all these children necrotizing enterocolitis (NEC) was diagnosed. The group of babies (2–12th life month) comprised five children (17.9%; aged 3.0 ± 1.2 months) suffered from NEC, peritonitis, intestinal perforation and ileus as ACS-inducing entities. The toddlers (2nd–6th year) presented the smallest group of two children (7.1%, aged 28.5 ± 0.7 months), in which combustion and polytrauma had led to ACS. The strongest group was the school children (7th–18th year) with 18 (64.3%) children. The mean age of this group was 12.9 (±3.6) years. Polytrauma, peritonitis, pancreatitis and intraabdominal bleeding had led to the ACS (Fig. 1).
Diagnostics Within the first years of the above-mentioned observational period, the diagnosis of an ACS was made exclusively by Table 1 Number of malformations, respectively, genetical diseases Genetic disorder
Frequency
Cerebral Meningomyelocele
1
Arnold-Chiari syndrome Mental retardation of unknown cause
1 1
Gender distribution
Pulmonary
The over-all gender distribution was even with 14 girls (50.0%) and 14 boys (50.0%). Within the age group of newborns the female children with 66.7% were outbalancing the male (33.3%). Within the other age groups the female-to-male ratio was nearly even.
Cardiac
Unilateral lung hypoplasia
1
Persistent ductus botalli
3
Ventricular septal defect
1
Morbus fallot
1
Diaphragm Unilateral diaphragmatic agenesia
1
Gastrointestinal tract 20 16 12 8
2
Colonic atresia
1
Esophageal atresia Vogt IIIb
1
Gallbladder agenesia
1
Hirschsprung’s disease
1
Renal Double renal system
4
2
Complex syndromes
0 newborn
babies
toddlers
school children
Fig. 1 Mean age within each of the four age groups
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Mesenterium commune
OEIS
1
Genetic disease Mucoviscidosis
1
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Table 2 Diseases and number which were caused for ACS Primary Peritonitis
Polytrauma
7
7
Post appendectomy
3
Anastomotic leak following right hemicolectomy
1
Anastomotic leak following appendectomy
1
Traumatic small bowel perforation
1
Perforated appendicitis
1
Nephrectomy, splenectomy
1
Splenic rupture, liver contusion
1
Retroperitoneal hematoma following rupture of left internal iliac vein and artery 1
Perforation
2
Tearing of V. cava inferior, liver veins, splenic capsule
1
Splenektomie for splenic rupture, cholezystectomie, liver rupture, retroperitoneal hematoma Splenic and liver rupture, splenectomy,
1
Partial liver resection
1
Liver rupture grades 3–4
1
Duodenal perforation due to bowel ischemia
1
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 Ileus
3
Anticoagulation after acute paraplegia
1
Ileus due to adhesions following surgery for enterothorax
1
Following repair of volvulus and small bowel perforation
1
Adhesions
1
Secondary Sepsis Burn
2 1
Following abortion with sepsis
1
Empyema of the knee joint
1
8% burn of body surface area, IIa–b
1
clinical means as, for example, abdominal distension, acidosis and otherwise unexplainable decrease in urine output, respectively. The bladder was filled via an urinary catheter with 1 ml of NaCl/kg bodyweight while the patient was positioned supine. A three-way stopcock was established and connected to an infusion set filled with NaCl in order to build a water column which served as indirect IAP indicator (scale: mm; reference point: symphysis [=zero point]). If the IAP lay repeatedly above 12 mmHg and at least one organ showed dysfunction, a diagnosis of ACS was made. Preoperatively, the mean IAD was 18.5 (±3.1) mmHg, with a postoperative drop down to a mean of 9.3 (±1.2) mmHg. The diagnosis ‘‘ACS’’ was defined as repeatedly measured elevated intraabdominal hypertension ([12 mmHg) accompanied by at least one organ dysfunction [6]. Primary and secondary ACS Primary ACS clearly predominated the study group and was found in 25 children (89.3%). Within the group of
primary ACS 50% resulted from polytrauma and peritonitis, while 32.1% (9/28) were due to bowel perforation or ileus. In 7.1% of cases (2/28) the underlying causes were pancreatitis (1/28) and intraabdominal bleeding (1/28) leading to the appearance of retroperitoneal hematoma. Three children (10.7%) presented secondary ACS with sepsis (2 cases) and combustion (1 case) being the underlying diseases (Table 2). Previous abdominal surgery For 13 children (46.4%) decompressive surgery was their first intraabdominal intervention, while 15 children (53.6%) had undergone at least one abdominal operation before. Eight of them (53.3%) had been previously operated once, four patients twice (26.7%) and three children thrice (20%). Severity of operations was distinguished into small, middle and major interventions (for details see 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 1 Debridement of burn wounds 1 Refashioning of jejunostomy Major 8 Bowel resection 1 Repair of bladder exstrophy, cecal reconstruction and ascendostoma 1 Right hemicolectomy
Fig. 2 Implantation of a VicrylÒ mesh in the abdominal wall by a three months-aged girl after decompression laparotomy because of ACS
day of admission, the other five within 9 days of hospitalization. Long-term results
1 Partial pancreatic necrosectomy, splenectomy 1 Excision of retroperitoneal neuroblastoma
Surgery In all children ACS decompression of the abdominal cavity was carried out by performing a transverse laparotomy and creating a laparostoma by implantation of an absorbable mesh, which was fixed to the fascial layer using an absorbable running suture (Fig. 2). Depending on the postoperative course, this mesh either was removed while reconnecting fascial layers directly, or the mesh was left in situ until granulation tissue had overgrown the whole implant. In 18 children direct closure was carried out, the remaining ten 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 53 days (range 10–63 days). In six children repeated VicrylÒ mesh reduction was needed to minimize the wound surface and tension prior to the final surgical closure. The most frequent complication (6/28) observed was formation of an enterocutaneous fistula (21.4%). Fistulae were either oversewn or excised. In two children a mesh tear occurred at the junction with the fascia and made readaptation necessary. Lethality 22 of 28 children (78.6%) survived. Underlying cause of death in the other six cases (22.4%) was sepsis with multi-organ failure (MOF). Two children died on the
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Median follow-up time was 6.8 years (range 2.2–10 years). Further surgical interventions during follow-up included incisional hernia repair 6 cases (27.3%), closure of a stoma (one case) and relaparotomy for ileus due to adhesions (one case). A questionnaire was sent to the parents of 22 surviving children with a resulting response rate of 72.7% (16/22). According to the answers given in the questionnaire the scar area keeps discomforting 10 of 16 children physiologically or psychologically (62.5%). Of those, subjective impediment degree was put at 100% by five children (31.3%), at 80% by one child (6.3%) and at 50% by two patients (12.5%).
Discussion Few and just low-evidenced studies exist reporting exclusively about ACS in childhood. Released results correspond to our findings, although a direct comparison is not possible on account of the different study designs [5–9]. ACS occurs at any age in children. A division into different age groups has not been carried out yet by any of the named authors. All cited reports describe a predominance of the male gender, being stated at 61.8%. The presence of associated malformations in children with ACS is mentioned only in single reports. Diaz found malformations in 40% of children with ACS [1, 5]. This is in accordance with our own results, which showed the occurrence of malformations or genetic disorders to be
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46.1%. Literature research [1, 5–9] revealed the primary ACS to occur nearly twice as frequently as secondary and tertiary ACS together (62.1 vs. 37.9%). Retrospectively, further distinction between secondary and tertiary ACS could not be worked out against the background of insufficient data. Our own data revealed a higher ratio in favor of the primary ACS with a share of 89%. Due to our complete dataset a differentiation between primary and secondary ACS was well possible. In the literature only few studies did carry out a division into primary and secondary ACS [5–9]. Primary ACS defines all forms of ACS resulting from intraabdominal diseases and entities. Against that, secondary ACS derives from disarrangements arising from outside the abdominal cavity including pneumonia, extraabdominal trauma, burns with capillary leak syndrome or the need of high-fluid resuscitation, for example [10]. Tertiary ACS is defined as recurrent ACS following an initially successful treatment of primary ACS, e.g. after decompressive laparotomy [4]. Concerning the diagnosis of ACS or intraabdominal hypertension (IAH) no generally valid approach exists which was demonstrated by data resulting from a questionnaire sent to members of the Society of Critical Care [11]. 20% of the responders base their diagnosis IAH exclusively on clinical signs, 7.2% strictly use intravesical pressure-measurements and about 70% a combination of both. The remaining 2.8% use other methods. According to the WSACS (World Society of the Abdominal Compartment Syndrome) an ACS is defined as rise of the IAP above 20 mmHg with or without an APP (abdominal perfusion pressure) less than 60 mmHg in combination with a new onset of organ dysfunction [12, 13]. There is no evidence whether the above-mentioned criteria also apply for physiological conditions in children. Several authors suggest IAH-limits as low as 10–15 mmHg to be detrimental in childhood and to build the critical pressure head at which regularly a switch from IAH to ACS in pediatric patients can be observed [1, 5, 6]. According to Eijke [6] we therefore defined pediatric ACS as a lasting IAH higher
than 12 mmHg combined with at least one organ dysfunction. In adults, the intravesical pressure measurement is considered as gold standard for the indirect IAP measurement. Using this intermittent method which was first described by Bertram et al. [4], a measuring volume of 50 ml sterile saline is inserted. Against that, the recommended volumes in children vary between 1 ml/kg [13–15] and 2 ml/kg of body weight [16]. Concerning our study a filling amount of 1 ml/kg of body weight was enough to reliably determine the IAP. This is in accordance with data from Suominen who proved high correlation to the directly measured IAP when filling the bladder with 1 ml/kg of body weight [15]. As soon as measurements are performed in fixed or traumatized bladders untrustworthy pressure heads result [12]. Other situations rendering this technique unreliably are body positions other than supine, pre-existing peritoneal adhesions or preceding abdominal packing [12, 17]. Possible risks of bladder pressure measurements frequently pointed out by critics are damage and infection of the urinary tract. Nevertheless, Malbrain found the infection risk not to be raised when using a closed measuring system with three-way stopcock [12]. A huge number of causes are considered to be causative for the development of IAH and ACS. Our own evaluation revealed peritonitis and polytrauma to be predominant with each 25%. This is noteworthy in so far as in the present literature peritonitis was not looked upon as an origin of an ACS, yet. This might be explain by the fact that authors all illnesses which first lead to peritonitis and after that to ACS deem causative for the development of IAH and ACS. In their opinion, also peritonitis is just a consequence of another disarrangement and builds an intermediate stage on the way to an ACS. Polytrauma as an underlying cause for ACS was also mentioned by other investigators: deCou reported three exemplary cases [18] and Neville found a share of 13% being traumatized before developing an ACS [9]. Beck [1] analyzed medical records of 1762 pediatric patients who were sent to ICU and revealed a traumainduced ACS-incidence of 0.7% related to patients who
Table 4 Causes for primary ACS Beck [1] Abd. bleeding
2
Bowel perforation
1
Ileus
1
Abd. trauma
1
Abd. tumor 4
NEC/colitis
1
Diaz [5]
Eijke [6]
Kawar [19]
2 2 2 2
Peritonitis excluding bowel perforation Pancreatitis
DeCou [18]
1 2 1
Aachen
4
2
1
3
1
7
1 1
Neville [9]
1 1
7 15
4 1
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Table 5 Causes for secondary ACS Beck [1] Cerebral disease
DeCou [18]
Diaz [5]
Eijke [6]
Jensen [16]
Maxwell [8]
Morrell [20]
3
1
1
1
Aachen
4
Polytrauma
1
Combustion
2
Sepsis
3
3
1
3
Cardiac disease
2
Pneumonia
1
Fig. 3 Flow sheet for monitoring in risk patients
2
Surveillance of patients at risk for 12 h, intervals at 1-2 h
20 mmHg or >16 mmHg with organ dysfunction
16-20 mmHg and No organ dysfunction
conservative therapy and IAP-monitoring ACS
12 mmHg
Stop monitoring
Start at top
Secondary or tertiary
primary
operative decompression
yes
conservative therapy exhausted? no
no success successful
60 mmHg
> 20 mmHg
Attempt abdominal closure
12 mmH g
stop measurement
have had an accident (3/406) and an incidence of 0.2% based on all patients admitted to the ICU (3/1762). Of all 23 children who developed ACS and had to be decompressed by laparotomy in his cohorte, Neville stated NEC
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to be the main origin (56.6%) [9]. According to the secondary ACS, a comparably wide range of possibly underlying reasons has been published. Summarized infection and sepsis account for the vast majority of perpetrators,
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followed by combustion and trauma (Tables 4, 5). The diagnosis of an ACS requires the appropriate clinical signs and a rise in bladder pressure above 12 mmHg [4, 13]. Decision making after recognition of ACS was facilitated by a therapeutic algorithm (Fig. 3) which was developed and adapted from recommendations provided by Kimball and DeLaet. The most important difference between both authors lies in the therapy following the diagnosis of an ACS. With respect to their advices concerning the therapy of choice in cases of ascertained ACS, both authors argument controversially. While Kimball recommends an immediate decompression when ACS is diagnosed (irrespective of the ACS being primary, secondary or tertiary), DeLaet prefers a less invasive management and suggests to proceed conservative therapy in cases of secondary and tertiary ACS, if reasonable [21, 22]. Efficient therapeutic intervention for ACS consists of an immediate decompression of the abdomen and correction of the underlying cause, if conservative therapy options foreseeable deliver frustrating results. The implantation of absorbable mesh (e.g. VicrylÒ) has proven itself so far as meshes can be removed as soon as patients broadly become reconvalescent or alternatively may remain in situ till resorption with secondary granulation has achieved [4, 23]. Cheatham investigated adults with necessity of abdominal decompression and revealed no negative influence by laparotomy [24].
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