A comparison of the measurement properties of the Juvenile Arthritis Functional Assessment Scale with the childhood health assessment questionnaire in daily practice

We compared the measurement properties of a performance test (Juvenile Arthritis Functional Assessment Scale; JAFAS) with a questionnaire-based instrument (Childhood Health Assessment Questionnaire; CHAQ) to measure functional ability in patients with juvenile idiopathic arthritis on the level of individual items. In 28 consecutive children visiting an outpatient paediatrics clinic, the JAFAS (range 0–20) and CHAQ (range 0–3) were applied, and measures of disease activity and joint range of motion (ROM) were determined. Twenty-eight children with a median age of 10 years and median disease duration of 3.2 years were included. The median JAFAS score was 0, and the median CHAQ score was 0.125. Cronbach’s alpha was 0.92 for the JAFAS and 0.96 for the CHAQ. The Spearman correlation coefficient between the JAFAS and the CHAQ was 0.55 (P < 0.01). With six out of ten items, the JAFAS classified the child as less disabled than with corresponding CHAQ activities. Overall, associations with measures of disease activity and ROM were higher for the CHAQ than for the JAFAS. A performance test (JAFAS) does not appear to have an added benefit over the questionnaire-based assessment (CHAQ) of physical function in a cross-sectional study.
Introduction
In patients with juvenile idiopathic arthritis (JIA), functional disability can both be evaluated by means of questionnaires and observed performance tests. In a previous study [1], the internal consistency, construct validity and responsiveness of a questionnaire-based instrument, the Childhood Health Assessment Questionnaire [2] (CHAQ), proved to be somewhat better than those of an observed performance test, the Juvenile Arthritis Functional Assessment Scale [3] (JAFAS). As performance tests are time consuming and require specific equipment and trained assessors, it is relevant to know whether they have an added benefit over a questionnaire-based instrument.
In the abovementioned, both measures displayed a floor effect [1]. As minor average disability in patients with JIA is nowadays a reality, identifying those tasks that can discriminate among lower levels of disability becomes all the more important. In addition, as performance tests are time consuming and require specific equipment and trained assessors, it is relevant to know whether they have an added benefit over a questionnaire-based instrument.
The aim of the present study was therefore to compare the measurement properties of the JAFAS and the CHAQ in an unselected population of children with JIA on the level of individual items.
Materials and methods
Study design and patient recruitment
Between January 2001 and April 2002, 34 consecutive children with JIA were recruited according to the following criteria: age between 7 and 12 years, diagnosis JIA [4] and no other medical conditions interfering with functional ability. The patients were visiting the outpatient paediatric rheumatology clinic of the Leiden University Medical Centre. The clinic, which has two part-time working paediatric rheumatologists, is a tertiary referral centre for children with rheumatic diseases from the Leiden district and surrounding area (1 million inhabitants). The Medical Ethics Committee approved the study, and all patients and their parents gave written informed consent.
Assessment methods
The JAFAS (range 0–20) was developed as an objective measure of functional ability in children with rheumatic diseases between 7 and 18 years. With the JAFAS, the observed time needed to perform ten activities is compared with a standard ‘criterion’ time. The JAFAS was administered by one well-trained paediatric physical therapist (Bekkering).
The CHAQ, including 30 activities in eight different domains, with a total score ranging from 0 (no limitation) to 3 (maximal limitation), was completed by interviewing the children.
Disease activity was measured by the erythrocyte sedimentation range (ESR) and joint counts on swollen (JC-swollen) or tender joints (JC-tender), concerning 28 joints included in the Fuchs score [5] plus the ankles (range 0–30). The feeling of well-being and the presence of pain were determined by 15-cm Visual Analogue Scales (VAS), with anchors of ‘no pain/no discomfort’ on the left and ‘very severe pain/severe discomfort’ on the right (pain-VAS and VAS-well-being; final scores converted to scores ranged from 0 to 3). A similar VAS was used for the physician’s evaluation of disease activity (VAS-paediatrician; score range 0–3). Limitation in range of motion (ROM) was determined by a joint count on in motion-restricted joints (JC-limitation; range 0–30) and the paediatric Escola de Paulista de Medicina ROM scale [6] (pEPM-ROM; score range 0–6).
Statistical analysis
The ten JAFAS items were linked with nine corresponding CHAQ items (CHAQ-9). All ten items had a counterpart in the CHAQ; however, the JAFAS items ‘get in’ and ‘get out of bed’ matched only one item in the CHAQ (get in and out of the bed). Associations between the JAFAS, the CHAQ-total and CHAQ-9 scores were determined by means of Spearman correlation coefficients (rs). To test the concordance between individual JAFAS and CHAQ-9 items, the score on every item was dichotomised into not limited (0) or limited (≥1) and mutually compared with Cohen’s Kappa (κ value greater than 0.80 is considered as good) [7]. Internal reliability of the JAFAS, CHAQ-total and CHAQ-9 was determined by calculating Cronbach’s α (α value of 0.85 is considered good) [8] and item–total correlations. In addition, Spearman correlation coefficients of the JAFAS, CHAQ-total and CHAQ-9 scores with measures of disease activity and ROM were computed.
Results
Characteristics of the patients
From the 33 eligible children who visited the outpatient paediatric rheumatology clinic in the study period, two children refused to participate, and three did not fulfil the inclusion criteria (two children had serious mental retardation and one child had the attention deficit hyperactivity disorder). Thus, 28 children, 12 boys and 16 girls, were included. Their median age was 10.0 years (range 7.3–12.8), and the median disease duration was 3.3 years (range 0.1–10.2). A majority of the children had a polyarticular (nine patients) or oligoarticular pattern (11 patients) of joint involvement. Systemic onset JIA (three patients), arthritis and psoriasis (four patients) and enthesitis type JIA (one patient) were less frequently seen. Twenty (71%) children used anti-rheumatic medication, of whom 17 (68%) used disease-modifying anti-rheumatic drugs and three (11%) used oral corticosteroids.
The median scores of the ESR (7.7 mm/h, range 2–54), the JC-swollen (1.0, range 0–28), the JC-tender (0.8, range 0–8), the VAS-well-being (0.2, range 0–2.5), VAS-pain (0.1, range 0–1.5) and VAS-paediatrician (0.2, range 0–2.7) point at a relatively low level of disease activity. With respect to joint ROM, the median JC-limitation score was 1.0 (range 0–17), and the median EPM-ROM score was 0. 5 (range 0–19.5).
The median scores of the JAFAS (0, range 0–13) and CHAQ (0.125, range 0–2.6) indicate, on average, the presence of no and very little functional disability, respectively. The frequency distributions (Fig. 1) of the JAFAS and the CHAQ show that according to the JAFAS, 18 out of 28 patients (65%) had no limitations, whereas according to the CHAQ, 13 out of 28 patients (47%) had no functional disability.
Fig. 1Frequencies of JAFAS and CHAQ scores. No disability JAFAS = 0, CHAQ = 0. Mild disability JAFAS = 1–3, CHAQ = 0–0.5. Moderate disability JAFAS = 4–9, CHAQ = 0.6–1.5. Severe disability JAFAS = 10–20, CHAQ = 1.6–3.0
Reliability and validity
With respect to internal reliability, Cronbach’s α was 0.91 for the JAFAS, 0.96 for the CHAQ-total score and 0.92 for the CHAQ-9. The item–total correlation was moderate (≥0.60; p < 0.01) for two out of ten JAFAS and six out of nine matching CHAQ tasks. Besides these six items (dressing, pull on socks, cutting meat, bend down, walk outdoors and climb stairs) selected in this study, the items reach for object, writing, turn door key or water tab, and running were frequently scored as difficult and showed good item–total correlations.
Concerning the agreement between the two instruments, the JAFAS total score correlated moderately well with both the CHAQ-total score (r = 0.55, p < 0.01) and the computed score of the corresponding CHAQ-9 items (r = 0.56, p < 0.01). On the individual item level, there was excellent agreement [7] (κ > 0.80) with respect to tasks 2 (pull shirt or sweater over head) and 8 (from standing position sit on floor, then stand up). The results of the internal reliability statistics and the associations between the total scores and the corresponding item scores of JAFAS and CHAQ are presented in Table 1.
Table 1JAFAS and CHAQ scores and their associations (Spearman correlation coefficients) in 28 patients with JIAItemJAFASNumber of patients limited in activityInternal reliability, item–total correlationCHAQNumber of patients limited in activityInternal reliability, item–total correlationConcordance among JAFAS and CHAQ itemsNumber of concordant pairsCHAQ /JAFASbCohen’s κ1Button shirt/blouse90.85**Dress, including tying shoelaces and doing buttons70.76**203/50.30 ns2Pull shirt or sweater over head10.38*Pull on sweater over head10.34 ns280/01.00**3Pull on both socks20.49**Pull on socks70.64**216/10.13 ns4Cut food with knife and fork40.70**Cutting meat70.79**215/20.23 ns5Get into bed00.00Getting in and out of bed30.47*244/0a6Get out of bed00.00244/0a7Pick something up off floor from standing position10.38*Bend down to pick up clothing or a piece of paper50.62**244/00.29*8From standing position sit on floor, then stand up20.52**Stand up from a low chair or floor20.51**280/01.00**9Walk 50 feet without assistance10.38*Walk outdoors on flat ground50.67**244/00.29*10Walk up flight of 5 steps10.38*Climb up five steps70.69**226/00.20 nsTotal scoresCronbach’s αCronbach’s αSpearman’s r10 JAFAS items140.919 CHAQ items180.920.56**total CHAQ score180.960.55***p < 0.05, **p < 0.01aNo statistics are computed because of a constant factorbNumber of pairs with CHAQ ≥ 1 and JAFAS = 0/Number of pairs with JAFAS ≥ 1 and CHAQ = 0
Construct validity
The relationship between the JAFAS, CHAQ, CHAQ-9 and measures of disease activity, pain, swelling and limited range of joint motion are shown in Table 2. Both the JAFAS and the CHAQ scores correlated moderately to well with the VAS-paediatrician, JC-swollen, JC-limited joints, pEPM-ROM and CHAQ-pain. Neither the JAFAS nor the CHAQ scores were significantly associated with the JC-tender. The CHAQ showed significant associations with the ESR and CHAQ well-being, whereas the JAFAS did not.
Table 2Spearman correlation coefficients between JAFAS, CHAQ and other measures in 28 patients JAFASCHAQCHAQ (9 items)VAS-paediatrician0.41*0.56**0.34 nsESR0.37 ns0.62*0.75**Joint count on swollen joints0.47*0.65**0.48*Joint count on tender joints0.07 ns0.41*0.09 nsJoint count on limited joints0.44*0.64**0.59**Paediatric EPM-ROM0.50**0.73**0.88**CHAQ–pain0.38*0.69**0.55**CHAQ-well-being0.20 ns0.44*0.48**p < 0.05, **p < 0.01
Discussion
In parallel with an earlier publication [1], this study demonstrated no advantages of a performance test (JAFAS) as opposed to a questionnaire (CHAQ) to measure functional disability in children with JIA. We found modest correlations between the two instruments and the floor effect with the CHAQ being smaller than with the JAFAS. Moreover, the CHAQ showed a better internal reliability and stronger associations with measures of disease activity and joint ROM than the JAFAS. Tennant et al. [1] reported similar results regarding the internal reliability and validity, in addition to a smaller responsiveness of the JAFAS.
Possible explanations for the discrepancy between the JAFAS and CHAQ are that the JAFAS is concerned with performance on one specific time point and the speed of performance, whereas the CHAQ refers to the last week and the experience of difficulties, including the need for aids, appliances or other persons. Discordance between observed and reported functional disability has been reported earlier in both children with JIA [9] as well in adults with rheumatoid arthritis [10].
The relatively small number of patients and the lack of distribution of the scores over the full ranges of the various outcome measures could limit the external validity of this study. However, the observed low level of functional disability is consistent with results of previous studies in paediatric rheumatology [11].
The children’s version of the CHAQ was originally designed and validated with the questionnaire self-administrated by the patients. In this study, the questions were read out and filled in by the investigator to ensure appropriate completion. Although it is likely that with this method, the same results are obtained as with self-administration, a possible influence on the final scoring cannot be totally ruled out.
Given the large improvements in medical treatment of JIA and considering the persisting need for valid and responsive measures in clinical trials, a further elaboration of the tasks included in measures of functional ability, reflecting relevant activities in daily life, is needed. Lam et al. [12] showed that by utilizing new response scales as well as adding more challenging questions than those posed by the original Health Assessment Questionnaire (HAQ), the floor effect could be reduced and the sensitivity enhanced. In addition, the excellent internal reliability of the CHAQ-9 score as found in the present study and the observation that some JAFAS and selected CHAQ items were not or only marginally contributing to the final scores suggest that with both instruments, there are opportunities for a reduction in the number of items, in parallel with the recently developed short version of the HAQ in adult rheumatoid arthritis patients [13]. Which items should be included has to be further examined, as the nine CHAQ items this study focusses on are only selected because they corresponded with the JAFAS. With any future research, the conduction of large, prospective follow-up studies is to be advised.

questionnaire
juvenile idiopathic arthritis
activities of daily living
disability evaluation


Preconception Healthcare: What Women Know and Believe

Objectives: The objectives of this study were to determine if women realize the importance of optimizing their health prior to a pregnancy, whether the pregnancy is planned or not; and to evaluate their knowledge level and beliefs about preconception healthcare. Additionally, we sought to understand how and when women wanted to receive information on preconception health. Methods: A survey study was performed using consecutive patients presenting to primary care practices for an annual well-woman exam. Patients were recruited based on appointment type and willingness to complete the survey at the time of their appointment, but prior to being seen by the physician. Results: A total of 499 women completed the survey. Nearly all women (98.6%) realized the importance of optimizing their health prior to a pregnancy, and realized the best time to receive information about preconception health is before conception. The vast majority of patients surveyed (95.3%) preferred to receive information about preconception health from their primary care physician. Only 39% of women could recall their physician ever discussing this topic. The population studied revealed some significant knowledge deficiencies about factors that may threaten the health of mother or fetus. Conclusions: A majority of women do understand the importance of optimizing their health prior to conception, and look to their Primary care physician as their preferred source for such information. Study participants demonstrated deficiencies in their knowledge of risk factors that impact maternal and fetal health suggesting that physicians are not addressing preconception healthcare during routine care.
Introduction
Preconception care is defined as the promotion of the health and well being of a woman and her partner before pregnancy [1]. The goal of the preconception visit is to identify medical and social conditions that may put the mother or fetus at risk. The concepts of preconception care have been articulated for over a decade—but unfortunately have not become part of the routine practice. Although many studies [2–9] document the effectiveness of interventions targeted to increase awareness of preconception folic acid supplementation, however, little evidence links comprehensive preconception health promotion to improved pregnancy outcomes. Only one study using data gathered more than 10 years ago demonstrated a greater likelihood of pregnancy intendedness in a low-income cohort of woman exposed to information on preconception health during routine family planning visits at a community health department [10]. Studies in the United Kingdom [11, 12] of knowledge and attitudes toward preconception care among primary health care teams show widespread consensus among the healthcare workers of the importance of the topic. One of these studies included the attitudes of women of childbearing age and noted that the view of the importance of preconception care was less strongly held by the female population studied [11]. Other studies have focused on more specific topics, such as rubella immunity, folic acid supplementation and glycemic control in diabetics [13–15]. Approximately 2% to 3% of all pregnancies result in a neonate with a serious genetic disease or a birth defect that can cause disabilities, mental retardation, and in some cases early death [16]. We are unaware of additional studies assessing the general knowledge and beliefs that women possess about optimizing their health prior to conception, or their preferences for obtaining such information.
Methods
A survey study of consecutive patients presenting to primary care practices for an annual well-woman exam was performed in accord with prevailing ethical principles. The selected primary care practices, occupying the same building at the Mayo Clinic Arizona, represented a women’s health general internal medicine practice (5 physicians) and a family medicine practice (9 faculty physicians and 18 family medicine residents). Women were recruited for the study by nursing staff working within the practices, with permission and informed consent completed prior to distributing the survey tool. The Mayo Clinic IRB approved the study. Patients were recruited based on appointment type (e.g., well woman exam, annual Pap exam, and annual preventive medicine exam) and consenting to complete the survey questionnaire at the time of their appointment, but prior to being seen by the physician. The enrollment period was between August 2004 and July 2005. Women were considered eligible for the study if they were between the ages of 18 and 45 years, understood English, and gave their permission. The survey instrument was a four-page questionnaire and required approximately 10 minutes to complete. The survey included questions about demographics, pregnancy intendedness, knowledge and attitudes about preconception care, and personal preferences about sources of health information about preconception care.
Data from each survey was entered into a database at the Research Survey Center, Mayo Clinic, Rochester, Minnesota, and the aggregate data made available for analysis to the research team.
Results
A total of 570 women were invited to participate in the study; 58 declined and 13 did not meet eligibility criteria, leaving 499 women who completed surveys for data analysis. The demographic profile of the study population is shown in Table 1. In this study population, the majority (70.6%) of women were not currently attempting to conceive as noted in Table 2. Approximately 5% were actively trying to conceive, with 13.5% and 8.4% considering a pregnancy in either the next 1–2 or 3–5 years respectively. Interestingly, of the women who had previously been pregnant, pregnancies had actually been planned in only 47.2% of instances. Nearly all women in the study (98.6%) realized the importance of optimizing their health prior to a pregnancy, again as noted in Table 2. However, only 39% could ever recall their physician discussing preconception health. The majority of the women in this study population who were interested in preconception health education preferred the information prior to a pregnancy (74.8%) or at the time of their annual medical exam (11.9%), as displayed in Fig. 1.Table 1Study population demographic (n=499)Age Range: 18 to 45 years  18 to 25 years24%  26 to 35 years30%  36 to 45 years46% Mean: 33 yearsEthnicity White84.8% Asian3.6% African-American1.3% Native American1.4% Other9.0%Education (highest level) 11th grade or less0.4% Graduated High School7.5% Some college or technical school33.0% Graduated college38.5% Some graduate work6.7% Graduate degree14.0%Household income Less than $25,00010.5% $26,000 to $50,00019.7% $51,000 to $75,00018.6% $76,000 to $99,00013.0% $100,000 to $125,00013.2% $126,000 to $150,0006.8% $151,000 to $200,0006.4% Greater than $200,00011.8%Table 2Conception history and planningPlans about getting pregnant No plans at present time70.6% Currently trying4.6% Considering in next 1 to 2 years13.5% Considering in next 3 to 5 years8.4% Have tried, unable to get pregnant2.8%Ever been pregnant? Yes50.7% No49.3%If ever pregnant, where previous pregnancies planned? Yes47.2% No52.8%Affect of optimizing health of mother and pregnancy? Has a good effect on the pregnancy98.6% Has no effect on the pregnancy0.8% Has a bad effect on the pregnancy0.6%Doctor ever spoken to you about preconception health? Yes39.0% No61.0%Are you interested in receiving preconception health education? Very interested34.8% Somewhat interested21.6% Unsure10.1% Not at all interested33.5%If interested or unsure about education, when would you prefer?` At the time I become pregnant7.6% Before I try to get pregnant74.8% During pregnancy0.7% Every time I get an annual medical exam11.9% Unsure5.0%Fig. 1Are you interested in preconception health education?
The women who were interested in preconception health education, or unsure of such interest, were asked their preferences for sources for such information (Table 3 and Fig. 1). The vast majority preferred their physician, either a primary care physician (51.3%) or Obstetrician/gynecologist (44.0%). Only a fraction would primarily seek their information from sources other than their physician.
The survey included questions about patient awareness of key preconception risk factors which may influence the outcome of a future pregnancy. The study population demonstrated high awareness of certain risk factors, such as tobacco, alcohol, drug use, and domestic abuse (Table 4). There were, however, opportunities for improvement in basic understanding of the risk to maternal/fetal health as it relates to fish consumption, exposure to cat litter, and the impact of family and/or genetic history.
Discussion
Preconception care is the primary prevention of maternal and perinatal morbidity and mortality. It is an important issue in women’s health that is easily overlooked by physicians and patients. The results of this study demonstrate that the vast majority of study participants understood the importance of preconception healthcare and realized that it should be obtained prior to conception. Interestingly, many of the women who expressed an interest in receiving information about preconception healthcare perceived the annual exam as the appropriate venue for exploring this topic. The study participants showed a strong preference for obtaining information about preconception healthcare from their personal physician and the majority eschewed the use of technology such as the Internet as a source of such information. The study revealed that a large percentage of women expected their primary care physician or OB/GYN physician to address preconception healthcare with them.Table 3Patient preferences for sources of preconception information (Percentage ranking the choice as first preference)Primary care physician51.3%Obstetrician/gynecologist44.0%Family, friends0.3%Magazine, newspaper0.3%Internet/world wide web3.1%Other0.7%
These data demonstrate that the study population exhibited gaps in knowledge about specific preconception health topics, but confirmed previous findings that women do have an increased awareness of the importance of folic acid supplementation [2–9]. Additionally, the study population showed a high awareness of the risks associated with tobacco, alcohol and drug use, but were much less aware of the risks to fetal health associated with fish consumption and exposure to cat litter. Such findings point out the need to continue efforts at increasing public awareness of other modifiable risks to fetal health. Unfortunately, fewer than 40% of the women surveyed recalled discussing preconception healthcare with their physician at the time of their annual exam.
Clearly, there is a gap in patient expectation and delivery of healthcare services in this population of educated middle class women. This study only surveyed women who had chosen to obtain their primary care in Internal Medicine and Family Medicine practices of a private health care facility, suggesting that there may be obstacles to delivering preconception care even in an academic outpatient setting. Potential explanations for this gap in the delivery of preconception healthcare may include: age of patients studied, time constraints in the outpatient setting or insufficient training and/or content knowledge in preconception healthcare of the physicians. This study demonstrates that an opportunity to better understand the gap in care delivery and to evaluate possible solutions in private middle class settings. More exposure on the part of residents to a curriculum on preconception healthcare may provide an opportunity for physicians in these specialty fields to improve their skills and the delivery of this much-needed care to women.Table 4Preconception health knowledge and opinions (Percentage awarea of risk factor potentially affecting a pregnancy)Consumption of certain fish54.2%Exposure to cat liter64.9%Folic acid use79.6%Impact of family and/or genetic history84.1%Infectious diseases (need to screen for)89.3%Immunizations (up to date)91.2%Alcohol use95.8%Abuse (verbal, sexual and/or physical)97.2%Medication use (prescription and nonprescription)97.4%Tobacco use98.2%Illicit drug use98.8%aAgreed or strongly agreed on a five point Likert scale
One of the limitations of our study was the homogeneity of our patient population. The majority of our study participants were middle class, Caucasian and had at least some college education. Our findings may not be broadly applicable to women of other socioeconomic backgrounds, ethnicity and educational levels. The lack of diversity of our population reflects the demographics of this community and further study in other populations should be pursued.
As all women of reproductive age and potential presenting for continuing care in the primary care setting are candidates for preconception care, the essential and critical role of primary care physicians and providers in the provision of preconception care is apparent. The Centers for Disease Control (CDC) has published their “Recommendations for Improving Preconception Health and Health Care” [17]. These recommendations were developed through a collaborative effort during which the CDC successfully aligned the missions of a number of its external partners and internal programs to ultimately draft these national recommendations for preconception care. These national recommendations can now serve as a roadmap for both graduate medical education and continuing medical education curricula to improve the knowledge and skill of the physician workforce in the delivery of comprehensive preconception health care.

preconception health
primary care
preconception care
women’s health


Factors Affecting Morbidity and Mortality of Roux-en-Y Gastric Bypass for Clinically Severe Obesity: An Analysis of 1,000 Consecutive Open Cases by a Single Surgeon

Introduction Determinants of perioperative risk for RYGB are not well defined.
Introduction
Obesity is currently the number one public health problem in the United States, affecting one-third of all Americans (http:/www.surgeongeneral.gov/topics/obesity). Approximately 5 to 8% have clinically severe or morbid obesity and are candidates for bariatric surgery.
This obesity epidemic has been accompanied by a geometric rise in the number of bariatric surgical procedures. The American Society for Bariatric Surgery (ASBS) estimates that the number of bariatric surgery procedures has increased from approximately 20,000 in 1996 to over 140,000 in 2004 (http://www.asbs.org). During this period, membership in the ASBS has also increased fivefold, suggesting that many more surgeons are performing these procedures. The most common bariatric procedure performed in the United States is Roux-en-Y gastric bypass (RYGB).1
The explosive growth of bariatric surgery has garnered much attention in the media, with much speculation about the risks, both short- and long-term, of RYGB. Unfortunately, to date, little information from large series is available concerning the operative mortality of RYGB and those factors that predict mortality. In 2002, Livingston et al.2 reported an operative mortality of 1.3% in 1,067 patients after open RYGB and found that only age over 55 years correlated with perioperative mortality. More recently, Fernandez et al.3 analyzed their patient cohort of 1,431 patients having open RYGB and found a 1.9% mortality, which was associated with age, weight, longer limb gastric bypass, and the occurrence of a leak or pulmonary embolism.
This report is a multivariate analysis of preoperative mortality and morbidity in 1,000 consecutive open RYGBs performed over a 5-year period by a single surgeon (LF) in at a single institution.
Materials and Methods
Bariatric Surgery Program The bariatric surgery program at St. Luke’s–Roosevelt Hospital Center in New York City was initiated in April 1999 and the first operation performed in June 1999. The basis of this report consists of 1,000 consecutive open RYGBs (primary cases and revisions) performed by a single surgeon, LF, between June 1999 and June 2004.
Clinical Protocol and Surgical Technique All patients were evaluated preoperatively and met generally accepted criteria outlined by the NIH at its Consensus Development Conference on Gastrointestinal Surgery for Severe Obesity.4 In addition, all patients were routinely evaluated by a registered dietitian experienced in the treatment of obesity. Mental health and other specialty consultations were only obtained if they were felt to be clinically indicated or were required by an insurance carrier. All patients were evaluated by an attending anesthesiologist before surgery.Routine preoperative studies included: electrocardiogram, chest x-ray, gallbladder ultrasonography, serum electrolytes, glucose, HbA1c, calcium, albumin, lipid profile, liver function tests, complete blood count, platelets, prothrombin time, partial thromboplastin time, INR, and urinalysis. Over time, additional preoperative studies, including serum insulin, iron, ferritin, vitamin B12, 25-OH vitamin D, and thiamine (vitamin B1) were added.RYGB was performed in a standard fashion with the following common elements: (1) open technique; (2) 20–30 ml pouch, nondivided stomach, TA 90B (US Surgical, Norwalk, CT, USA) applied twice; (3) hand-sewn two-layer retrocolic, antegastric gastrojejunostomy, 12 mm in length, tested with methylene blue under pressure intraoperatively; (4) side-to-side, functional end-to-end jejunojejunostomy with dispSAble GIA (US Surgical) or LC (Ethicon Endosurgery, Sommerville, NJ, USA) 75 mm staplers, and TA55 (US Surgical) or TX 60 (Ethicon Endosurgery) staplers and routinely oversewn with 3–0 silk Lembert sutures. The biliopancreatic limb-length measured 75 cm along the antimesenteric border for patients with BMI less than 50 and 150 cm for patients with BMI greater than or equal to 50. The Roux or alimentary limb-length was 150 cm in all patients. For the initial 14 cases, the stomach was divided using the GIA-100 stapler. This technique was abandoned and switched to the nondivided stomach after a stapler malfunction led to a leak.Closed suction drains were placed in all revisions, patients with BMI > 55, or if clinically indicated (e.g., identification of an intraoperative leak, technically difficult anastomosis). Drains were removed as clinically indicated. Fascia was closed with a running looped #1 PDS (Ethicon, Sommerville) and infiltrated with 0.25% bupivacaine. The subcutaneous space was drained with a #10 Jackson–Pratt drain and the skin was closed with a running subcuticular stitch.The gallbladder was removed if gallstones were documented by preoperative ultrasound. Incisional or umbilical hernias were primarily repaired if encountered, oftentimes through a separate periumbilical incision.Invasive monitoring was not used routinely and Foley catheters were only placed if patients required invasive hemodynamic monitoring or in the case of revisions or when patients had multiple prior abdominal operations. Nasogastric tubes were left in placed as clinically indicated (intraoperative leak identified) or in patients with BMI > 55. All patients received perioperative antibiotics for 24 h, cefazolin (2 g intravenously every 8 h for three total doses) or clindamycin (900 mg intravenously every 6 h for four doses). Prophylaxis against deep vein thrombosis consisted of 5,000 units of unfractionated heparin administered subcutaneously every 8 h and pneumatic compression stockings until ambulatory. All patients were given a PCA pump (patient-controlled analgesia) for pain. Patients were routinely cared for on a regular surgical floor, equipped for severely obese patients. Patients with significant SA or documented CAD were usually observed in the recovery room overnight, and only rarely admitted to the intensive care unit. On the morning of postoperative day 1, patients were routinely studied with gastrograffin upper-GI studies. If no leak was identified, they were given liquids for lunch and advanced to soft food (yogurt, apple sauce, and cottage cheese) for dinner and switched to oral pain medications. Drains were usually removed before discharge. Patients were discharged on POD #2 or #3 as indicated with the following medications: a codeine-derivative for pain, prenatal vitamins, iron polysaccharide, calcium citrate, ursodeoxycholic acid, if the gallbladder was present.After hospital discharge, patients were scheduled to be seen at 2 and 8 weeks, 6, 12, 18, 24 months, and yearly thereafter. All routine follow-up appointments included nutritional counseling and those after 2 weeks included laboratory studies [serum electrolytes, glucose, HbA1c, calcium, albumin, lipid profile, liver function tests, complete blood count, platelets, serum insulin, iron, ferritin, vitamin B12, 25-OH vitamin D, and thiamine (vitamin B1)]. Follow-up was 74% at 1 year, 68% at 2 years, 59% at 3 years, 53% at 4 years, and 48% at 5 years.
Clinical Data and Data Analysis Clinical and laboratory data were PC-based database prospectively maintained since the program’s inception in 1999. Data collected included: age, sex, height weight, BMI, race/ethnicity, payer status, obesity-related comorbidities, operative procedure, duration of stay, mortality, major complications, and death. Complications were classified as systemic (prolonged intubation, deep venous thrombosis, pulmonary embolism, and myocardial infarction/fatal arrhythmia) or technical (incisional hernia, intestinal obstruction, leak/perforation, dehiscence, GI bleeding, anastomotic stricture, and anastomotic ulcer). Deaths were analyzed with respect to BMI, demographics, comorbidities, and complications.Superficial wound infections were not included; the incidence of urinary tract infections was not tracked. Nutritional complications were not evaluated because all patients were routinely maintained on vitamins, iron, and calcium supplements postoperatively; it would be impossible to determine the true incidence of any of these nutritional deficiencies.Univariate analyses and logistic regression with SPSS 11.0 were used to determine significance.
Results
The population consisted of 854 women and 146 men. Their demographic characteristics are summarized in Table 1. The prevalence of obesity-related comorbid conditions is shown in Table 2.
Table 1Demographic Characteristics by Sex and Race WomenMenTotalp-ValueAge (years)38 ± 1.0 (15–73)40 ± 11.9 (15–65)38 ± 11.17 (15–73)0.064Weight (kg)134 ± 27 (84–263)170 ± 43 (81–345)139 ± 33 (82–345)<0.01BMI (kg/m2)51 ± 10 (35–1,000)55 ± 13 (24–116)51 ± 10 (24–116)<0.01Caucasian(28%) 238/853(53%) 78/14732%<0.01African–American(30%) 253/853(20%) 29/14728%0.01Hispanic(42%)358/853(27%)40/14740%<0.01Other(0.5%) 4/853(0%) 0/1470.4%0.41Table 2Prevalence of Obesity-Related Comorbid Conditions by Sex Women (N = 853) (%)Men (N = 147) (%)Total (%)p-ValueType II diabetes mellitus177 (21)54 (37)23<0.01Hypertension310 (36)79 (54)39<0.01CAD/CHF29 (3)26 (18)6<0.01Dyslipidemia376 (44)80 (54)470.02Sleep apnea172 (20)63 (43)24<0.01Asthma135 (16)16 (11)150.12Dyspnea on exertion811 (95)127 (86)48<0.01GERD513 (60)77 (52)590.08Osteoarthritis791 (93)126 (87)92<0.01Urinary stress incontinence430/(50)5 (3)44<0.01Irregular menses276 (32)NA32NACAD/CHF coronary artery disease/congestive heart failure, GERD gastroesophageal reflux disease
The most common comorbidites encountered were dyspnea on exertion (94%), joint pain/arthritis (92%), and gastroesophageal reflux disease (59%). The comorbidites typically associated with systemic disease included hypertension (HTN, 39%), obstructive sleep apnea (SA, 24%), dyslipidemia (46%), and asthma (15%). Approximately 23% of the patient population suffered from type II diabetes mellitus (DM). At time of initial evaluation, 13.0% of this diabetic patient subset had a prior history of insulin-dependent DM, 57.6% had noninsulin dependent DM, and 23% had a previous diagnosis of DM intermittently controlled on diet or were newly diagnosed with DM during their preoperative evaluation. Six percent of the patient population had angiographically documented histories of coronary artery disease (CAD) but were deemed suitable risk by their respective specialists.
Procedures and Duration of Hospital Stay There were 966 primary RYGB and 34 revisions of failed bariatric procedures (21—VBG, 5—RYGB, 8—other; all performed at outside institutions) to RYGB. The median length of stay for primary procedures was 2.4 days compared to 3.7 days for revisions. Average (LOS) for all patients having primary RYGB was 3.8 days with 87% of the group leaving in 3 days or less.
Complications Overall, 91% of the procedures were without systemic or technical complications.The incidence of complications in relation to BMI is summarized in Table 3. Overall, systemic complications occurred rarely, but did not usually correlate with BMI. The most common technical complications were incisional hernia (3.5%), intestinal obstruction (1.9%), and leak/perforation (1.6%).
Table 3Incidence of Complications after RYGBComplicationBMI < 50 (%) (N = 481)BMI > 50 (%) (N = 515)Total (%)p-ValueSystemic complicationsProlonged intubation3 (0.6)8 (1.5)1.10.16 (NS)Deep venous thrombosis0 (0)2 (0.4)0.2NSPulmonary embolism0 (0)3 (0.6)0.3NSMI/fatal arrhythmia1 (0.2)1 (0.2)0.2NSTechnical complicationsIncisional hernia10 (2.1)25 (4.8)3.50.019Intestinal obstruction10 (2.1)9 (1.7)1.9NSLeak7 (1.5)9 (1.9)1.6NSDehiscence2 (0.4)2 (0.4)0.4NSGI bleeding requiring transfusion3 (0.6)6 (1.2)0.9NSAnastomotic ulcer2 (0.4)0 (0)0.2NSAnastomotic Stricture6 (1.2)2 (0.4)0.8NSDeath4 (0.8)11 (2.1)1.5.03Thirty-one patients (3.1%) required reoperation within 30 days of the original procedure. The indications for reoperation within 30 days were leak/perforation (11), intestinal obstruction (9), bleeding (4), rule-out leak (2), dehiscence (4), and subphrenic abscess (1).Indications for late operations (>30 days postoperatively) included incisional hernia repair in 35 patients, intestinal obstruction in 10 patients, and repair of leaks/perforations in five patients who were experiencing ongoing postoperative complications. No patients required reoperation for refractory anastomatic stricture or ulcer. One patient developed a gastrogastric fistula after repair of an early leak, which was treated expectantly since it was clinically insignificant. No late gastrogastric fistulae were identified.
Deaths Thirty-day mortality was 1.2%. Overall mortality attributable to surgery was 1.5%. Patients with late deaths due to unrelated events, such as motor vehicle accidents (N = 2) or drug overdoses (N = 1), were excluded and not classified as mortalities in the analysis. Mortality correlated with BMI, with four (0.8%) patients having a BMI < 50 dying compared to 11 (2.1%) patients with BMI ≥ 50 (p = 0.03) (Table 3).Causes of death after RYGB, along with their timing and relationship to BMI are shown in Table 4. Two patients died of fatal arrhythmias on POD #3 and #4. Both were males, ages 48 and 54, with BMIs > 50, DM, CAD, HTN, and SA. Autopsies were performed in both instances and no other precipitating factors were identified. One patient, a 43-year-old woman with a BMI 59, died of a pulmonary embolism (identified at autopsy) at home after 2 weeks.
Table 4Causes of Early and Late Deaths Related to BMICause of deathEarly (<30 days) (N = 11)Late (>30 days) (N = 4)BMI < 50BMI ≥ 50BMI < 50BMI ≥ 50MI/arrhythmia0200Pulmonary/PE0100MSOF 20 leak1212MSOF 20 bowel obstruction0100MSOF cause unknown1100Bleeding complications1200PE Pulmonary embolism, MSOF multisystem organ failureSix patients died from MSOF after postoperative leaks, three from the gastrojejunostomy and three from perforations of the distal small bowel within the common channel. Of these, four occurred and were diagnosed within 48 h of the initial RYGB and all were emergently explored. All four developed MSOF and expired between 14 and 211 days postoperatively. Of the remaining two deaths, one patient, 46-year-old woman with a BMI 79 and a history of chronic ventilator dependence due to a paralyzed left hemidiaphragm on chronic steroid therapy developed a late leak and multiple intestinal fistulae and eventually succumbed to MSOF. The other patient, a 43-year-old woman, BMI 43, had undergone a laparoscopic RYGB at another institution that was complicated by a strangulated internal hernia, massive intestinal gangrene, and short-bowel syndrome. She underwent a reversal of her RYGB with reconstruction of her GI tract, but developed multiple small bowel fistulae 2 weeks postoperatively and died of MSOF several months later.Three patients died from a severe systemic inflammatory response syndrome (SIRS) with MSOF accompanied by extremely high fevers, without any identifiable source. One was a 70-year-old woman with BMI 68 underwent reexploration for an early postoperative small bowel obstruction. The others were a 54-year-old woman with a BMI 47, DM, HTN, and SA and a 37-year-old male with BMI 94 and severe SA and HTN. Each developed SIRS and MSOF with temperatures of 105–107°F and hyperdynamic circulations. No intraabdominal or other sources were identified despite numerous cultures and radiologic studies.Three patients died of bleeding complications. A 43-year-old woman with a BMI 48 suffered progressive hypotension and tachycardia in the recovery room postoperatively. These symptoms were initially addressed with rehydration allowing the patient’s hematocrit to fall to a level where hypovolemic shock and resulting coagulopathy obscured efforts to surgically control or identify a single source. A 31-year-old woman with a BMI 50 was returned to the OR 4 h postoperatively for control of bleeding from the small bowel mesentery. After this second procedure, she developed SIRS, temperatures of 106°F, and MSOF, with no identifiable source of sepsis. A 54-year-old male with a BMI 56 and a history of HTN, CAD, and chronic atrial fibrillation suffered a postoperative myocardial infarction and developed a coagulopathy complicated by a massive intraspenic hematoma after restarting coumadin, which rapidly progressed to anuria and MSOF. Attempts to reverse his anticoagulation and control the bleeding angiographically were unsuccessful.The necessity for reoperation within 30 days of the original procedure was particularly ominous. Overall, the incidence of death after a second operative procedure within 30 days was 9/31 (29%). Two of the 16 patients with a BMI < 50 who required reoperation within 30 days died (12.5%) compared to 7 out of 15 patients with BMI ≥ 50 (47% p = 0.03). This is similar to the mortality for the entire series where 0.8% for patients with a BMI < 50 died compared to 2.1% for patients with BMI ≥ 50 (p = 0.03). However, among those 15 patients classified as operative deaths for the entire series, 9 (60%) died after their second operative procedure.Logistic regression demonstrated that CAD [LR 7.5 p < 0.01 (95% CI 2.2 to 25.3)], and SA [LR 3.3 p = 0.03 (95% CI 1.1 to 10.1)], followed by age [LR 1.06 p = 0.042 (95% CI 1.00 to 1.12)], were risk factors for death in all patients (Table 5). Although a small sample set, 12.7% of patients with CAD died (7/55) and 29% of patients with BMI > 50 and CAD and SA died.
Table 5Logistic Regression Evaluation of Patient Comorbidity as Predictors of Mortality p-ValueRelative Risk95% Confidence IntervalAge0.0421.0591.002–1.120BMI0.1301.0250.993–1.058Female0.6120.7290.215–2.474DM0.8520.8890.259–3.054HTN0.2572.0850.585–7.438CAD0.0017.4462.195–25.258Dyslipidemia0.0990.3590.106–1.211Asthma0.0703.0650.913–10.293SA0.0333.3421.104–10.115SOB0.6930.6440.073–5.725Although the average BMI of males was slightly greater than that of females (55.2 vs 51.2 kg/m2, p < 0.01), the two populations also differed in characteristics other than BMI. The male population had a significantly significant greater prevalence of DM, HTN, CAD, dyslipidemia, and SA. Females had a greater prevalence of pulmonary comorbidities, including asthma and dyspnea on exertion (Table 2).When logistic regression was performed specific to the subsets of sex, males with angiographically demonstrated CAD were 30 times more likely to die [LR 30.1 p = 0.028 (95% CI 1.4 to 631.4)]. Logistic regression did not identify CAD as a significant predictor when the analysis was limited to women. Predictors of death for women include age [LR 1.07 p = 0.033 (95 % CI 1.0 to 1.14) and SA [LR 4.1 p = 0.040 (95% CI 1.07 to 16.2)].Logistic regression was repeated specific to race. When limited to Caucasian patients only, Caucasians with CAD were 58 times more likely to die [LR 58.8 p < 0.01 (95% CI 4.8 to 716.9)] than those Caucasians without CAD. Increasing BMI was also significant among Caucasians. [LR 1.08 p = 0.02 (95% CI 1.01 to 1.16)]. Evaluation of African–American patients demonstrated that only SA was significant [LR 19.1 p = 0.03 (95% CI 1.29 to 282.8)]. Regression of the Hispanic population did not identify a specific factor. One-way ANOVA did not demonstrate any significant differences in prevalence of death or CAD between the three racial groups for the entire population or specific to sex.Hispanic patients had significantly less SA than either the Caucasian or African–American patients (18.1 vs 25.6 and 27.4%, respectively (p = 0.01)). When examined specifically in relation to sex, there were no differences among prevalence of SA in the males. African–American women, however, had the greatest prevalence of SA (26.4%) compared to Caucasian (18.9%) and Hispanic (15.7%) women (p < 0.01).
Discussion
The tremendous growth of bariatric surgery over the past several years has spawned much interest in its complications and mortality, first in the media, but most recently in the public health arena as well. Health and malpractice insurance carriers as well as governmental agencies and professional societies are evaluating the risks of bariatric surgery and the surgeons that perform it. In several states, insurance companies have stopped covering bariatric surgery at the same time that the Centers for Medicare and Medicaid Services have approved coverage for them. Several malpractice carriers have stopped issuing policies for surgeons performing bariatric procedures while others are categorizing bariatric surgery as a high-risk subspecialty area, similar to obstetrics and neurosurgery, and increasing premiums accordingly. In each of these instances, the overriding fear or consideration appears to be that the risks associated with bariatric surgery are excessively high.
Much of the information being utilized in this regard has come form series utilizing pooled data from multiple smaller series or government databases.1,5–9 Buchwald et al.5 performed a metaanalysis of 16,944 patients which included 7,074 patients that underwent gastric bypass (open and laparoscopic) in 44 studies with a 30-day mortality rate of 0.5%. Several authors have reported that complication rates of bariatric surgery were inversely correlated with case loads, reporting mortality rates of in the range of 0.1–0.5% for surgeons more than 100 or 150 cases per year.6–8 Flum et al.9 recently reported 30- and 90-day mortality rates of 2.0 and 2.8%, respectively in Medicare beneficiaries having bariatric surgery, with men having higher rates than women and those over 65 years of age having higher rates than those younger than 65. Many of these series lack data concerning BMI and comorbidities, making risk assessment difficult or impossible. In one large series from a single-center, Christou et al.10 reported a 30-day mortality of 0.4% in 1,035 patients undergoing RYGB, of whom 820 had open RYGB. No details about preexisting comorbidities or perioperative complications were given.
The results of these pooled series differ from those in several large series of open gastric bypasses2,3,11. Livingston et al.2 reported an operative mortality of 1.3% in 1,067 patients after open RYGB. In his series, mean BMI was 53.6 kg/m2, mean age 42.3 years, and the incidence of comorbidities was diabetes (23%), hypertension (48%), and sleep apnea (39%). Only male sex was predictive of severe life-threatening complications; mortality in patients over 55 years was significantly greater than in patients under 55 (3.5 vs 1.1%, p < 0.05). More recently, Fernandez et al.3 analyzed their patient cohort of 1,431 patients having open RYGB and found a 1.9% mortality, which was associated with age, weight, longer limb gastric bypass, and the occurrence of a leak or pulmonary embolism. In that series, the mean BMI was 53.3, mean age 40.7 years, and incidence of serious comorbidities was diabetes (19.5%), hypertension (51%), and sleep apnea (33%). In both of these series, the BMI was higher, the patients were older and the incidence of serious comorbidities, such as diabetes, hypertension, dyslipidemia, and sleep apnea, higher. Pories et al.11 reported a 1.5% perioperative mortality rate, with 0.8% dying of sepsis, 0.5% dying of pulmonary embolism, and 0.2% of an unknown cause. Additional information regarding the incidence of leaks, bowel obstructions and other complications was not reported as these were not the focus of the original paper.
Similarly, in the present series, the 30-day mortality rate was 1.2% in 1,000 patients with a mean BMI of 52 kg/m2. The prevalence of preoperative comorbidities was comparable to the larger series (diabetes—23%, hypertension—39%, dyslipidemia—46%, coronary artery disease/congestive heart failure—5.5%, sleep apnea—23.5%, and asthma—15%) and generally higher than in the pooled series. The incidence of leaks and postoperative small bowel obstruction in our series was comparable to the other series. The incidence of pulmonary embolism was less than those reported, perhaps related to the use of both pneumatic compression stockings and subcutaneous heparin and early ambulation with a shorter length of stay. Although the incidence of incisional hernia in our series was low (3.5%), this may well be affected by our suboptimal follow-up.
Multisystem organ failure accounted for 11 deaths in our series (73%). In six patients, this resulted from leaks or perforations; in one it followed a bowel obstruction, in two it followed postoperative hemorrhage and in two patients, the cause was never determined. In each of these instances, the complication was identified early and appropriate treatment and supportive care instituted. In four of the patients, the clinical course of SIRS and MSOF was characterized by extremely high temperatures (>105°F), with no apparent source ever identified. To our knowledge, this “syndrome” has not been reported, but may be due to the fact that the enormous adipose tissue stores in these patients may act as a “metabolic sink”, releasing cytokines and other mediators and perpetuating this extreme systemic inflammatory response. Two of the deaths were due to fatal arrhythmias, both in patients with known CAD, who were extensively evaluated preoperatively. The death due to pulmonary embolism occurred after discharge, even though the patient received prophylaxis with both heparin and pneumatic compression stockings in the hospital. The remaining death due to exsanguination was clearly preventable.
Although males were significantly heavier and had higher BMIs than women, sex was not an independent predictor of morbidity. However, the presence of angiographically documented coronary artery disease was particularly ominous in men. Males with angiographically demonstrated CAD were 30 times more likely to die. In women, predictors of death included age and SA, but not CAD. With respect to race, Caucasian males with BMI > 50 and CAD were most likely to die, whereas SA was a predictor in African–American men. There were no predictors in Hispanics. Despite the increased mortality in Caucasian males with CAD, these patients were not candidates for cardiac revascularization and extreme weight loss was the only intervention thought to make a beneficial health impact.
Based upon our data and those of others2,3,11, it appears that the risk of open RYGB is in the range of 1–2%. Risk appears to be adversely affected by increasing BMI and those factors with which it is often associated namely male sex and coronary artery disease. How this compares with the perioperative mortality after laparoscopic RYGB is still unclear because many series of patients having laparoscopic RYGB do not include patients with the highest BMIs, above 60 or 70 kg/m2, or patients having revisional surgery.
A perioperative mortality rate of 1.2% after RYGB compares favorably with that after other common surgical procedures. For example, perioperative mortality after elective surgery for abdominal aortic aneurysms has been reported at 3.1–4.7% overall, 1.0–2.7% in patients under 65 years of age, and 3.5–5.2% in patients over 65 years of age.12 Using Medicare data adjusted for high volume surgeons, Birkmeyer et al.13 reported perioperative mortality rates of 4.5% for colectomy, 8.6% for gastrectomy, 8.4% for esophagectomy, 3.8% for pancreatic resection, 4.0% for pulmonary lobectomy, and 10.7% for pneumonectomy. While it is true that all of these patients were over 65 years of age, the fact still remains that these perioperative mortality rates are all substantially greater than that after RYGB (even in Medicare recipients, as recently reported9) and neither the public, the press, the insurance industry, or various state Departments of Health are appalled or alarmed, or calling for a moratorium on those procedures. This is not meant to suggest that every effort should not be made to lessen the risks of bariatric surgery and to improve operative mortality, but rather to inject some proportionality into the discussion. The importance of such careful analysis of bariatric surgical data, including its limitations, and the need to continue to offer bariatric surgery to those patients for whom it constitutes the best available treatment has recently been emphasized.14 In his 2004 PERSPECTIVE, Surgery for Severe Obesity, Steinbrook15 quotes Robert Brolin, MD, cautioning physicians and the public “...to reconcile the fact that the operation has a real mortality and it will continue to have a real mortality under the best of circumstances. Some of these patients are just profound operative risks for any kind of surgical intervention...The sickest ones are the ones who benefit the most, but they are also the highest risk”.
Conclusion
RYGB can be performed with acceptable perioperative morbidity in patients over a wide range of BMIs. Patients with BMI ≥ 50 have a higher morality for both initial operations and after reexploration. Age, coronary artery disease, and obstructive sleep apnea correlate with perioperative mortality. These three comorbidities were more prevalent in these patients and may contribute to this finding.

morbidity
mortality
roux-en-y gastric bypass
obesity
morbid obesity


Tracheobronchomalacia due to amyloidosis in a patient with rheumatoid arthritis

In this case report, we describe a patient with longstanding rheumatoid arthritis who developed tracheobronchomalacia with fatal outcome. Despite negative antemortem biopsies of abdominal fat and tongue, amyloid was found postmortem in the trachea and appeared to be associated with tracheobronchomalacia.
Introduction
Amyloidosis in longstanding chronically active rheumatoid arthritis (RA) is a well-known complication of this disease [1]. We present a RA patient with amyloidosis, albeit with a unique presenting symptom and unexpected organ involvement.
Case report
The patient was a 69-year-old female with seropositive erosive RA, who had not been treated with disease-modifying antirheumatic drugs since 1984. In March 2005, she presented to another hospital because of progressive dyspnea of several hours, due to a bronchopneumonia. After 2 days, she developed a stridor. Otolaryngological examination results showed diffuse swelling of the tongue, pharynx and neck, and an extensive tracheobronchomalacia. Treatment consisted of intubation, antibiotics, diuretic therapy, and corticosteroids. She was admitted to our Intensive Care Unit because of progressive swelling and respiratory insufficiency due to tracheobronchomalacia. Physical examination results revealed extensive swelling and a remarkable protrusion of the tongue. Typical rheumatoid joint deformities and rheumatoid nodules were present but no active arthritis. Laboratory investigations revealed a normocytic anemia and a renal insufficiency. The measured creatinine clearance was 12 ml/min, with a proteinuria of 0.35 g/24 h. Computed tomography scan of thorax and neck provided no explanation for the tracheobronchomalacia. Clinical suspicion of amyloidosis was not confirmed by biopsies of abdominal fat tissue and of the tongue. Ultimately, the patient succumbed to multiple organ failure. Autopsy revealed AA amyloid in the trachea (Fig. 1), spleen, liver, and perivascularly in the kidneys.
Fig. 1Amyloidosis of the trachea in Congo red
Discussion
Amyloidosis of the trachea is a rare but known disorder, even rarer in patients with systemic diseases (such as Sjogren syndrome) but never associated with tracheobronchomalacia [2–4]. We report the first case of an RA patient with tracheobronchomalacia due to AA amyloidosis of the trachea. The diagnosis was only confirmed at postmortem, although several biopsies had been done in life to confirm the clinical suspicion. We refrained from rectal and kidney biopsies because of the relatively high risk of complications associated with these procedures; the absence of diarrhea and the low level of proteinuria suggested a relatively low pretest probability. Therefore, the finding of renal amyloid was surprising. Recently, Uda et al. [5] described a prospective cohort study of patients with RA without clinical signs of kidney disease and amyloidosis of the kidney. At follow-up, the group of patients with amyloid deposition confined to the perivascular region continued to have little proteinuria and a good renal prognosis, compared to those with amyloid deposition in the glomeruli who developed rapidly deteriorating renal function. This patient, however, does show that the disease course in RA patients with perivascular amyloid deposition in the kidney is not benign and may be associated with fatal complications elsewhere.
In conclusion, tracheobronchomalacia can be due to amyloid deposition in patients with RA. Amyloidosis should be considered in RA patients with a small amount of proteinuria, and a renal biopsy should be performed, especially as accumulating evidence shows that patients with amyloidosis have a better prognosis when the ongoing inflammation is effectively suppressed [1].

tracheobronchomalacia
amyloidosis
rheumatoid arthritis
kidney


2005 PRETEXT: a revised staging system for primary malignant liver tumours of childhood developed by the SIOPEL group

Over the last 15 years, various oncology groups throughout the world have used the PRETEXT system for staging malignant primary liver tumours of childhood. This paper, written by members of the radiology and surgery committees of the International Childhood Liver Tumor Strategy Group (SIOPEL), presents various clarifications and revisions to the original PRETEXT system.
Introduction
The PRETEXT system was designed by the International Childhood Liver Tumor Strategy Group (SIOPEL) for staging and risk stratification of liver tumours [1, 2]. PRETEXT is used to describe tumour extent before any therapy, thus allowing more effective comparison between studies conducted by different groups. The system has good interobserver reproducibility [3] and good prognostic value in children with hepatoblastoma [2–5], and is the basis of risk stratification in current SIOPEL hepatoblastoma studies. Most other study groups now use the PRETEXT system to describe imaging findings at diagnosis, even if this is not their main staging system.
Certain limitations of the system have become obvious over the last 15 years. In addition, there have been significant advances in imaging during this period [6]. This paper is the report of a working party that met in June 2005 to update the PRETEXT system.
PRETEXT staging is based on Couinaud’s system of segmentation of the liver (Fig. 1) [7]. The liver segments are grouped into four sections as follows: segments 2 and 3 (left lateral section), segments 4a and 4b (left medial section), segments 5 and 8 (right anterior section) and segments 6 and 7 (right posterior section). The term section is used (where other authors use segment or sector) to avoid terminological confusion.
Fig. 1Schematic representations of the segmental anatomy of the liver. a Frontal view of the liver. The numerals label Couinaud’s segments 2 to 8. b The hepatic veins (black) and the intrahepatic branches of the portal veins (grey) are shown. Segment 1 (equivalent to the caudate lobe) is seen to lie between the portal vein and the inferior vena cava. c Exploded frontal view of the segmental anatomy of the liver. The umbilical portion of the left portal vein (LPV) separates the left medial section from the left lateral section (LLS). Segment 1 is obscured in this view. Note that the term “section” has been used in preference to “segment” or “sector” (see text). d Transverse section of the liver shows the planes of the major venous structures used to determine the PRETEXT number. The hepatic (blue) and portal (purple) veins define the sections of the liver (2–8). This schematic diagram shows how the right hepatic (RHV) and middle hepatic (MHV) veins indicate  the borders of the right anterior section (RAS)  with the right posterior (RPS) and left medial (LMS) sections. Note that the left portal vein (LPV) actually lies caudal to the confluence of the hepatic veins and is not seen in the same transverse image. The left hepatic vein (LHV) runs between segments 2 and 3 and is not used in PRETEXT staging
In the original system, the caudate lobe (segment 1) was ignored. The PRETEXT number was derived by subtracting the highest number of contiguous liver sections that were not involved by tumour from four [1]. This number is, very roughly, an estimate of the difficulty of the expected surgical procedure (Table 1). Pedunculated tumours are considered to be confined to the liver and to occupy only the section(s) from which they originate.
Table 1Definitions of PRETEXT number (see text for PRETEXT number of tumours involving the caudate lobe)PRETEXT numberDefinitionIOne section is involved and three adjoining sections are freeIIOne or two sections are involved, but two adjoining sections are freeIIITwo or three sections are involved, and no two adjoining sections are freeIVAll four sections are involved
In addition to describing the intrahepatic extent of the primary tumour(s), the PRETEXT system includes certain other criteria. These assess involvement of the inferior vena cava (IVC) or hepatic veins (designated V), involvement of the portal veins (P), extrahepatic abdominal disease (E) and distant metastases (M).
The purpose of the 2005 revision was to improve the original definitions of the PRETEXT stages, to clarify the criteria for “extrahepatic” disease, and to add new criteria (Table 2). The term “extrahepatic” disease is confusing, and these categories will in future be called “additional criteria”. There is still much to be learned about prognostic factors in the primary malignant liver tumours of childhood. An important goal of these changes, therefore, is to improve our ability to identify prognostic imaging findings, and thereby refine risk stratification.
Table 22005 PRETEXT staging: additional criteriaCaudate lobe involvementCC1Tumour involving the caudate lobeAll C1 patients are at least PRETEXT IIC0All other patientsExtrahepatic abdominal diseaseEE0 No evidence of tumour spread in the abdomen (except M or N)Add suffix “a” if ascites is present, e.g., E0aE1Direct extension of tumour into adjacent organs or diaphragmE2Peritoneal nodulesTumour focalityFF0Patient with solitary tumourF1Patient with two or more discrete tumoursTumour rupture or intraperitoneal haemorrhageHH1Imaging and clinical findings of intraperitoneal haemorrhageH0All other patientsDistant metastasesMM0No metastasesAdd suffix or suffixes to indicate location (see text)M1Any metastasis (except E and N)Lymph node metastasesNN0No nodal metastasesN1Abdominal lymph node metastases onlyN2Extra-abdominal lymph node metastases (with or without abdominal lymph node metastases)Portal vein involvementPP0No involvement of the portal vein or its left or right branchesSee text for definition of involvement. Add suffix “a” if intravascular tumour is present, e.g., P1aP1Involvement of either the left or the right branch of the portal veinP2Involvement of the main portal veinInvolvement of the IVC and/or hepatic veinsVV0No involvement of the hepatic veins or inferior vena cava (IVC)See text for definition of involvement. Add suffix “a” if intravascular tumour is present, e.g., V3aV1Involvement of one hepatic vein but not the IVCV2Involvement of two hepatic veins but not the IVCV3Involvement of all three hepatic veins and/or the IVC
Although the PRETEXT system is principally used for hepatoblastoma, the 2005 revision is intended to be applicable to all primary malignant liver tumours of childhood, including hepatocellular carcinoma and epithelioid haemangioendothelioma. The original SIOPEL risk stratification system for hepatoblastoma has already been modified in the protocols for current SIOPEL studies (Table 3). Firstly, tumour rupture or intraperitoneal haemorrhage at the time of diagnosis (H1, see below) is now a defining criterion of high risk. Secondly, children with alpha-fetoprotein levels of <100 μg/l are also considered to be high risk. The 2005 revision involves no further change in the SIOPEL risk stratification system for hepatoblastoma.
Table 3Risk stratification in hepatoblastoma for current SIOPEL studiesHigh riskStandard riskPatients with any of the following:Serum alpha-fetoprotein <100 μg/lAll other patientsPRETEXT IVAdditional PRETEXT criteria: E1, E1a, E2, E2a H1 M1 (any site) N1, N2 P2, P2a V3, V3a
PRETEXT grouping
The traditional approach to radiological segmentation of the liver, based on the paths of the hepatic veins, is an oversimplification. This is partly due to the variability of hepatic venous anatomy [8–10]. The main problem, however, is the imperfect correlation with segments defined by the branching pattern of the portal veins [8, 11–13]. Although the plane of the right hepatic vein reliably separates the right posterior and anterior sections [9], the left hepatic vein runs to the left of the boundary between the left lateral and medial sections, which is best defined by the plane of the fissure of the ligamentum teres and the umbilical portion of the left portal vein (Fig. 1) [14].
PRETEXT I
This group includes only a small proportion of primary malignant liver tumours of childhood. From the definition of the PRETEXT number, it can be seen that only tumours localized to either the left lateral section or the right posterior section qualify as PRETEXT I (Fig. 2).
Fig. 2PRETEXT I. a The left lateral section (segments 2 and 3) is involved. b The right posterior section (segments 6 and 7) is involved
PRETEXT II
Most PRETEXT II tumours are limited to either the right lobe or the left lobe of the liver. Tumours of the left medial or right anterior sections are also PRETEXT II. Multifocal tumours involving only the left lateral and right posterior sections are classified as PRETEXT II; this pattern is very rare. Tumours limited to the caudate lobe were not classifiable under the original PRETEXT system [1]. In the 2005 PRETEXT system these tumours are classified as PRETEXT II (but see also C, below). This is the only change in the PRETEXT numbering system in this revision. There is no change in numbering for tumours involving the caudate lobe and any other part of the liver, which are classified as PRETEXT II (if two or three contiguous sections are free), III (if there are no two contiguous sections free) or IV (if all four sections are involved) (Fig. 3).
Fig. 3PRETEXT II. a Tumour involving only the right lobe of the liver. b A transverse T1-weighted MR image of a child with hepatoblastoma shows that the middle hepatic vein (arrow) is displaced but not involved by the tumour. This is the most common type of PRETEXT II tumour. c Tumour involving only the left lobe of the liver. d Tumour involving only the left medial section. e Tumour involving only the right anterior section. f Multifocal tumours involving only the left lateral and right posterior sections. g The tumour is confined to the caudate lobe (PRETEXT II C1, see text; RPV right portal vein)
PRETEXT III
The unifocal tumours in this category spare only the left lateral or right posterior section. These tumours are relatively common. In children with hepatoblastoma, great care must be taken to distinguish between invasion and compression of the apparently uninvolved section of the liver, because risk stratification (and/or the need for liver transplantation) may depend on this point. Anterior central liver tumours involve segment 4 and either or both of segments 5 and 8. Although recent advances in surgical technique permit resection of these tumours without trisectionectomy [15], classification as PRETEXT III reflects the difficulty of these operations (Fig. 4).
Fig. 4PRETEXT III. a Extensive tumour sparing only the left lateral section. b Extensive tumour sparing only the right posterior section. c Anterior central liver tumour involving the left medial and right anterior sections. d Contrast-enhanced CT image shows a central liver tumour lying between the left portal vein (white arrow) and the right hepatic vein (black arrow). e Multifocal PRETEXT III tumour, sparing the right anterior section. f Multifocal tumours sparing only the left lateral and right anterior sections. g Multifocal tumours sparing only the left medial and right posterior sections
Multifocal PRETEXT III tumours may also spare the right anterior or left medial sections, or two non-contiguous sections. These patterns are rare.
PRETEXT IV
PRETEXT IV tumours involve all sections of the liver. These tumours are often multifocal. Alternatively, a very large solitary tumour can involve all four sections (Fig. 5).
Fig. 5PRETEXT IV. a Multifocal PRETEXT IV tumours involve all four sections. b Contrast-enhanced CT image of a patient with PRETEXT IV F1 (see text) hepatoblastoma. c Unifocal PRETEXT IV tumours often have a diffuse growth pattern. d Contrast-enhanced CT image of a patient with diffuse PRETEXT IV hepatoblastoma
C: caudate lobe tumours
The caudate lobe and caudate process (segment 1 or segments 1 and 9, depending on the system of nomenclature) can be resected with either the left or right lobe of the liver [7]. For this reason, segment 1 was not considered in the PRETEXT classification in the original system [2]. Modern surgical techniques have made resection of segment 1 safer, but these operations remain difficult. Involvement of the caudate lobe is, therefore, a potential predictor of poor outcome. If any tumour is present in segment 1 on imaging at diagnosis (Fig. 3g), the patient will be coded as C1, irrespective of the PRETEXT group (see above). All other patients should be coded as C0.
E: extrahepatic abdominal disease
The assessment of extrahepatic abdominal disease was one of the most confusing aspects of the original PRETEXT system, and clearly needed revision. Originally, there was a requirement for all extrahepatic abdominal spread of tumour (E+) to be proved by biopsy. Modern imaging techniques are capable, in principle, of identifying extrahepatic abdominal tumour extension in many forms. The frequency and significance of these imaging findings is different for different tumour types, and not all patterns are easily biopsied.
In hepatoblastoma, for example, direct extension of tumour into other abdominal organs is unusual. Tumour extension through the diaphragm (Fig. 6) is uncommon, but can be shown quite convincingly by MRI or CT, and biopsy proof may be impractical. In the 2005 revision, patients with direct extension of tumour through the diaphragm or into other organs can be coded as E1 without biopsy proof.
Fig. 6Extrahepatic abdominal tumour extension. This composite of contrast-enhanced CT images in a patient with hepatoblastoma shows growth of the primary tumour through the diaphragm into the thorax (E1). The 2005 PRETEXT system no longer requires biopsy proof for this form of tumour spread
Pedunculated tumours are considered to be confined to the sections from which they arise, and are not extrahepatic disease.
Peritoneal tumour seeding was originally not included in this category [2]. It probably indicates more advanced abdominal disease than direct extension of the primary tumour. Imaging techniques, especially ultrasonography, can often show even small peritoneal nodules clearly, and the differential diagnosis is very limited. In the 2005 revision, peritoneal nodules will be assumed to be metastases, and will be coded as E2. All other patients should be coded as E0.
Ascites is an unusual finding at presentation in hepatoblastoma, but is more common in hepatocellular carcinoma, where it may be an independent predictor of poor prognosis. For this reason, patients with ascites will be coded as E0a, E1a or E2a as appropriate.
Abdominal lymph node metastases, which were previously recorded as E+, are now coded as N (see below).
F: tumour focality
In SIOPEL 1, multifocal tumours were identified at the time of diagnosis in 18% of the patients with hepatoblastoma where this information was available [4]. Univariate analysis showed that the 5-year event-free survival was significantly worse for patients with multifocal tumour (40%) than for those with unifocal tumour (72%) [4]. The independent significance of this finding is unclear, as there is clearly an association between multifocality and advanced PRETEXT number. The German Society of Pediatric Oncology and Hematology reported slightly different results [16]. In its HB89 study, 21% of patients had multiple well-defined tumours, and these children had a similar disease-free survival (DFS; 87%) to those with a single tumour (86%). However, in 20% of children the tumour exhibited a diffuse growth pattern (Fig. 5), and these had a significantly worse DFS (21%) [16]. Unfortunately, a diffuse growth pattern is difficult to define, and despite the promise that this finding shows as a potential risk factor, it was decided not to incorporate it in the 2005 PRETEXT revision.
Patients with one hepatic tumour should be coded as F0. All those with more than one tumour nodule (Figs. 3, 4 and 5), regardless of nodule size or PRETEXT stage, should be coded as F1.
H: tumour rupture or intraperitoneal haemorrhage
It is not uncommon for hepatoblastoma and hepatocellular carcinoma to present with tumour rupture [17, 18]. Originally, these patients were not automatically included as high risk in SIOPEL studies, because of the requirement that extrahepatic disease (E) be proved by biopsy. Although the data to prove this are not currently available, it seems intuitively likely that tumour rupture (usually manifesting as intraperitoneal haemorrhage) is a risk factor, and these patients should be coded as H1. Laparotomy or aspiration of peritoneal blood is not necessary for diagnostic purposes if characteristic imaging and clinical findings (such as hypotension and low haematocrit or haemoglobin level) are present. The presence of peritoneal fluid on imaging alone does not imply tumour rupture (but see E above).
Since the opening of the SIOPEL 4 study in September 2004, tumour rupture has become a defining feature of high-risk hepatoblastoma in SIOPEL studies. Patients with no evidence of tumour rupture or haemorrhage, and those with only subcapsular or biopsy-related intraperitoneal bleeding, are coded as H0.
M: distant metastases
Patients with distant metastases at diagnosis are coded as M1. In hepatoblastoma, these metastases are predominantly found in the lungs. Although the best imaging modality for the identification of lung metastases is currently CT, the defining characteristics of lung metastases in this context have not been specifically studied. It is believed, however, that factors favouring a diagnosis of metastasis include multiple lesions, a rounded, well-defined contour and a subpleural location. In most parts of the world, a single rounded lung lesion with a diameter of >5 mm in a child with a primary liver tumour is very likely to be a metastasis. Patients with these findings on chest CT scans should be classified as M1. Biopsy is not required for staging purposes, because it is uncommon for other lesions to mimic metastases in this clinical context. The protocols of the SIOPEL studies recommend central radiological review if there is any doubt about the presence of lung metastases.
Other metastases are infrequently found at diagnosis in hepatoblastoma, but are more common in hepatocellular carcinoma. The imaging findings of brain metastases are usually characteristic, and biopsy is not required.
Bone scintigraphy is recommended for staging in children with hepatocellular carcinoma, but not hepatoblastoma. Abnormal calcium metabolism is common in children with hepatoblastoma, and may cause abnormal uptake on bone scintigraphy, especially in the ribs [19], whereas bone metastases are rare [20]. Biopsy proof is therefore mandatory for suspected bone metastases in hepatoblastoma, unless the findings of cross-sectional imaging are characteristic and the patient is already in the high-risk category for some other reason, such as the presence of lung metastases.
Bone marrow biopsy is not recommended in children with hepatoblastoma, because bone marrow spread is rare [20]. It is not known whether metastases at different sites have different prognostic implications. For statistical purposes, it is therefore recommended that one or more suffixes be added to M1 to indicate the major sites of metastasis: pulmonary (p), skeletal (s), central nervous system (c), bone marrow (m), and other sites (x). A child with lung, brain, and adrenal metastases would therefore be coded as M1cpx. Patients with no evidence of haematogenous metastatic spread of tumour should be coded as M0.
N: lymph node metastases
Because porta hepatis (and other abdominal) lymph node metastases are quite unusual in hepatoblastoma, SIOPEL trials have always required this form of tumour spread to be proved by biopsy. In fact, benign enlargement of lymph nodes is probably not uncommon, and the accuracy of positron emission tomography is not known in this context. Because biopsy of equivocal lymph nodes inevitably carries some risk, the SIOPEL committee actively discourages this. Biopsy may, however, be required if there is significant nodal enlargement (for example short axis >15 mm) in a child with no other criteria for high-risk hepatoblastoma.
Lymph node metastases are quite common in hepatocellular carcinoma and fibrolamellar carcinoma, and biopsy proof is not required if the imaging abnormality is unequivocal. An arbitrary threshold short axis diameter of 15 mm is suggested for this purpose.
Children with no lymph node metastases by these criteria are coded as N0, those with nodal metastases limited to the abdomen (i.e. caudal to the diaphragm and cranial to the inguinal ligament) as N1, and those with extra-abdominal nodal metastases as N2.
P: portal vein involvement
Involvement of the main portal vein and/or both major branches has been considered a risk factor in hepatoblastoma, because this has obvious implications for the resectability of the tumour. It is also possible that portal vein invasion detected by imaging is an independent risk factor for tumour recurrence [21]. The original PRETEXT criteria, however, did not specifically define the word “involvement”.
It is well recognized that a tumour that abuts or displaces a major portal venous branch at imaging performed at diagnosis (Fig. 7) may shrink away from the vein following preoperative chemotherapy. Imaging evidence of complete obstruction or circumferential encasement (Fig. 7) is therefore required to qualify as portal vein involvement. Failure to identify the portal vein or one of its major branches in either its normal position or its expected displaced location, on good quality images, is strong evidence of obstruction. The other form of involvement, portal vein invasion, is not uncommon, and is often best detected by ultrasound (Fig. 7). Various US signs may be present [22, 23], and analogous findings can be seen on CT and MR imaging.
Fig. 7Involvement of the portal and hepatic venous systems. a When the tumour (grey) approaches or abuts the vein (black), there is no venous involvement, even if the vein is partly encased. b Complete obstruction or encasement of the vein is one form of involvement. Obstruction of the inferior vena cava by extrinsic compression, however, does not count as involvement (see text). c Intravascular tumour growth in the portal and/or hepatic venous systems is not uncommon in children with hepatoblastoma or hepatocellular carcinoma. d Transverse ultrasound image of the right lobe of the liver in a patient with hepatoblastoma. The tumour (white circles) has grown into the right branch of the portal vein (P1a), disrupting the normal “white line” of the vein wall (arrows)
Patients with no imaging evidence of involvement of the main portal vein, its bifurcation, or either of its main branches will be coded as P0. Those who fulfil the original PRETEXT definition of P+ (involvement of the main portal vein, its bifurcation, or both of its main branches), as well as those with “cavernous transformation” of the portal vein will be coded as P2. P2, however, represents very advanced disease. For this reason, the category P1 has been created for patients with evidence of involvement of one major branch of the portal vein. In addition, the detection of portal vein invasion should be marked by the suffix “a” (e.g., P2a).
V: involvement of the IVC and/or hepatic veins
The same definitions of involvement (venous obstruction, encasement and/or invasion) used for the portal veins apply to the hepatic veins (Fig. 7). A hepatic vein can be assumed to be involved if it cannot be identified at all, and its expected course runs through a large tumour mass. It is important to look carefully for the hepatic veins, preferably with ultrasonography as well as CT and/or MRI, as they may be displaced from their expected position by the tumour. Complete obstruction of the IVC can occur with mass effect alone, without any tumour extension to the vein itself. Inability to visualize the IVC, and the presence of an enlarged azygos vein, are not, therefore, sufficient criteria for involvement. Patients with no imaging evidence of involvement of the hepatic veins or IVC will be coded as V0.
As for the portal vein, the original classification of involvement (V+) indicated a very advanced level of disease. Intermediate categories have therefore been created. V1 and V2 indicate involvement of one or two main hepatic veins respectively. V3 indicates involvement of either the IVC or all three of the hepatic veins. In addition, the detection of hepatic vein or IVC invasion should be marked by the suffix “a” (e.g., V2a). The presence of tumour in the right atrium automatically makes a patient V3a.
SIOPEL risk stratification for patients with hepatoblastoma
The SIOPEL risk stratification for children with hepatoblastoma is essentially unchanged by this revision. Patients with any one or more of certain criteria (Table 3) are high risk. All other SIOPEL patients are standard risk.
Presurgical re-evaluation
Although the timing of surgery will depend on the treatment protocol and the patient’s response to therapy, preoperative reimaging is almost always necessary. All of the PRETEXT categories should be reassessed after preoperative chemotherapy, as near as possible to the time of surgery, and recorded as POSTEXT (post-treatment extent of disease). Comparison of surgical findings with POSTEXT will allow prospective assessment of the accuracy of imaging techniques.

staging liver tumour children hepatoblastoma