Eijck, F.C. van

Strategies and trends in the treatment of (giant) omphalocele (Doctoral Thesis)

2011-04-06T00:00:00Z

The management of giant omphaloceles remains a challenge for pediatric surgeons. Although the mortality rate is still high (up to 20%) in case of multiple congenital anomalies, surviving patients with omphalocele achieve a state of health and quality of life comparable to that of general population peers. Results from our study confirm the hypothesis that neonates with a congenital abdominal wall defect have a high risk for adhesive small bowel obstruction and could benefit from adhesion prevention. Awaiting complete epithelialisation before operation of giant omphalocele might reduce serosal injury and limit adhesiogenic areas. The liver was partly unprotected in all giant omphaloceles evaluated in this thesis. In case of an incisional hernia, the liver was located underneath the abdominal defect. A pre-operative ultrasound study is recommended, therefore. Furthermore, the parents should receive good documentation and information. The question remains whether contact sports and other risk behaviour should be advised against, as there is no indication of more blunt trauma in these patients in the literature. The results of the questionnaire sent to the authors do not show a consensus for a generally accepted treatment method after more than thirty years of innovations in the management of patients with a giant omphalocele. The newly introduced Component Separation Technique seems to have a good outcome. The herniation rate is low, and prosthetic materials are not needed. However, the question remains if delayed closure with this technique is better than immediate staged closure. There is not yet an evidence base; we shall have to await the long-term results of the published techniques. Based on these outcomes, a randomized multicenter trial comparing the staged and delayed techniques is recommended. Until then, we remain dependent on expert opinion.

Past and current surgical treatment of giant omphalocele: Outcome of a questionnaire sent to authors (Article)

2011-03-01T00:00:00Z

Purpose: Operative treatment of giant omphalocele (OC) is still a challenge for pediatric surgeons. We were interested to ascertain whether published operative techniques for giant OC once advocated by their authors were still being used by these authors and whether the techniques had been modified or even abandoned for other techniques. Methods: Relevant studies concerning the treatment of giant OC were identified by an electronic search. Publication date of the articles was from 1967 to 2009. A questionnaire was sent to the first author or coauthor, unless contact details were unavailable. The described surgical techniques were categorized into primary closure, staged closure, and delayed closure. Results: Almost half of the authors (42%), independent of the initial technique used (primary, staged, or delayed closure), changed or stopped using their technique after the publication of the article. The change was not to one particular proven better technique. Herniation rate was lower in delayed closure (9% delayed vs 18% staged vs 58% primary). Conclusions: The results of the questionnaire did not show a generally accepted method of treatment after more than 30 years of innovations in managing patients with a giant OC. There are generally 2 main treatment modalities: staged closure and delayed closure. Because of the lack of large patient numbers and late follow-up, long-term results of the published techniques are needed, and randomized multicenter trials based on these outcomes are recommended. Until then, we remain dependent on expert opinions.

Has the liver and other visceral organs migrated to its normal position in children with giant omphalocele? A follow-up study with ultrasonography (Article)

2010-05-01T00:00:00Z

This study evaluates whether, on the long run, in patients born with a giant omphalocele, the liver and other solid organs reach their normal position, shape, and size. Seventeen former patients with a giant omphalocele, treated between 1970 and 2004, were included. Physical examination was supplemented with ultrasonography for ventral hernia and precise description of the liver, spleen, and kidneys. The findings were compared with 17 controls matched for age, gender, and body mass index. We found an abnormal position of the liver, spleen, left kidney, and right kidney in eight, six, five, and four patients, respectively. An unprotected liver was present in all 17 patients and in 11 controls, the difference being statistically significant (p∈=∈0.04). In ten of the 11 patients with an incisional hernia, the liver was located underneath the abdominal defect. Conclusion: In all former patients with a giant omphalocele, an abnormal position of the liver and in the majority of them, an incisional hernia was also found. The liver and sometimes also the spleen and the kidneys do not migrate to their normal position. Exact documentation and good information are important for both the patient and their caretakers in order to avoid liver trauma.

Minor and giant omphalocele: long-term outcomes and quality of life (Article)

2009-07-01T00:00:00Z

Purpose: Long-term outcome and quality of life in omphalocele (OC) studies are mainly focused on cosmetic disorders with the abdominal scar and gastrointestinal disorders. The aim of this study was to compare long-term mortality, morbidity, and quality of life between patients with minor and giant OCs. Methods: Records of 89 minor and 22 giant OC children were reviewed. A questionnaire on general health was sent to all patients. A second questionnaire concerning quality of life and functional status; Darthmouth COOP Functional Health Assessment Charts/WONCA (COOP/WONCA) was sent to all patients aged 18 years or older and a peer control group. Results: Of the surviving patients (69 minor OC, 20 giant OC), 12 were lost to hospital follow-up. The first questionnaire was returned by 64 (83%) of 77 patients. There were no significant differences in gastrointestinal disorders. Cosmetic problems were experienced significantly more in giant OC. The results of the COOP/WONCA charts indicated a good to very good quality of life in both groups comparable to the control group. Conclusions: Our study indicates that after a high level of medical intervention perinatally, quality of life is good to very good in both groups and comparable to healthy young adults.

Hartmann's gallbladder pouch revisited 60 years later (Article)

2007-07-01T00:00:00Z

Background: Hartmann's gallbladder pouch was the subject of an article in The Lancet 60 years ago. It has regained new interest in view of laparoscopic cholecystectomy. However, different opinions exist with regard to its incidence and nature. To elucidate these discrepancies, a descriptive study was performed with regard to the incidence and morphology of Hartmann's pouch. Methods: Gallbladders were obtained after elective laparoscopic cholecystectomy. In addition, gallbladders were obtained during routine postmortem examination. The gallbladders were divided in two groups: those with Hartmann's pouch and those without Hartmann's pouch. All the gallbladders were examined macroscopically and microscopically. Fisher's Exact Probability Test (p < 0.05, two-tailed) was used to analyze the data. Results: A total of 98 gallbladders were examined: 49 obtained after laparoscopic or open cholecystectomy and 49 obtained after postmortem examination. Among the gallbladders with Hartmann's pouch (n = 51), 65% contained stones and 35% had no stones. Among the gallbladders without Hartmann's pouch, 43% contained stones and 57% had no stones. Macroscopically, in all the gallbladders with Hartmann's pouch, the pouch was observed to result from adhesions between the cystic duct and the neck of the gallbladder. After cleavage of these adhesions, the phenomenon of Hartmann's pouch was abolished in all cases. Conclusions: Hartmann's gallbladder pouch is a frequent but inconstant feature of normal and pathologic human gallbladders. There is a significant association between the presence of Hartmann's pouch and stones (p < 0.05). Adhesions between the cystic duct and the neck of the gallbladder are responsible for Hartmann's pouch. Consequently, Hartmann's gallbladder pouch is a morphologic rather than an anatomic entity.

Endocrine and metabolic responses in children with meningoccocal sepsis: striking differences between survivors and nonsurvivors (Article)

2000-01-01T00:00:00Z

To get insight in the endocrine and metabolic responses in children with meningococcal sepsis 26 children were studied the first 48 h after admission. On admission there was a significant difference in cortisol/ACTH levels between nonsurvivors (n = 8) and survivors (n = 18). Nonsurvivors showed an inadequate cortisol stress response in combination to very high ACTH levels, whereas survivors showed a normal stress response with significantly higher cortisol levels (0.62 vs. 0.89 micromol/L) in combination with moderately increased ACTH levels (1234 vs. 231 ng/L). Furthermore, there was a significant difference between nonsurvivors and survivors regarding pediatric risk of mortality score (31 vs. 17), TSH (0.97 vs. 0.29 mE/L), T3 (0.53 vs. 0.38 nmol/L), reverse T3 (rT3) (0.75 vs. 1.44 nmol/L), C-reactive protein (34 vs. 78 mg/L), nonesterified fatty acids (0.32 vs. 0.95 mmol/L), and lactate (7.3 vs. 3.2 mmol/L). In those who survived, the most important changes within 48 h were seen in a normalization of cortisol and ACTH levels, but without a circadian rhythm; a decrease of rT3 and an increase in the T3/rT3 ratio; and a decrease in the levels of the nonesterified free fatty acids and an unaltered high urinary nitrogen excretion. At this moment, it is yet unknown whether the hormonal abnormalities are determining factors in the outcome of acute meningococcal sepsis or merely represent secondary effects. Understanding the metabolic and endocrine alterations is required to design possible therapeutic approaches. The striking difference between nonsurvivors and survivors calls for reconsideration of corticosteroid treatment in children with meningococcal sepsis.