Extrahepatic portal vein morphology in children with extrahepatic portal hypertension assessed by 3-dimensional computed tomographic portography: a new etiology of extrahepatic portal hypertension

Background/Purpose

The cause of extrahepatic portal hypertension in children has not been clarified. Our aim was to determine the morphological features of the extrahepatic portal vein in children with extrahepatic portal hypertension by 3-dimensional computed tomographic portography and to clarify the etiology of this disorder.

Materials and Methods

Six patients ranging in age from 10 to 18 years (median age, 12.8 years) who had portal hypertension presented with hematemesis. They underwent intravenous computed tomographic portography using a helical computed tomography scanner and 3-dimensional image reconstruction.

Results

The extrahepatic portal vein was visualized in all patients by 3-dimensional computed tomographic portography. None of the patients showed extrahepatic portal vein obstruction or cavernous transformation. All patients had a tortuous η-shaped extrahepatic portal vein, and a line could be drawn through the flexures of the portal vein to the hepatic hilum.

Conclusion

In children, extrahepatic portal hypertension is not caused by extrahepatic portal vein obstruction and may be of embryological origin.     

Pediatric thoracic outlet syndrome: a disorder with serious vascular complications

Background

Thoracic outlet syndrome (TOS), caused by compression of the neurovascular structures between the clavicle and scalene muscles, typically presents with neurologic symptoms in adults. We reviewed our experience with 25 adolescents and propose a diagnostic/treatment algorithm for pediatric TOS.

Methods

From 1993 to 2005, 25 patients were treated with TOS. A retrospective chart review was performed with institutional review board approval. Demographics, clinical presentation, diagnostic studies, and treatment were evaluated.

Results

Seven male (28%) and 18 female (72%) patients presented between the ages of 12 to 18 years. Thirteen (52%) had vascular TOS (11 venous, 2 arterial), 11 (44%) had neurologic TOS, and 1 had both. Vascular TOS included subclavian vein thrombosis (7), venous impingement (4), and arterial impingement (2). Three patients had hypercoagulable disorders, and 6 had effort thrombosis. Venography was diagnostic in 10 cases. Neurogenic TOS was diagnosed by clinical symptoms. Five patients with subclavian vein thrombosis underwent thrombolysis, with 3 maintaining long-term patency. Of 25 patients, 24 underwent transaxillary first rib resection.

Conclusion

Vascular complications are more common in adolescents with TOS than in adults. A diagnostic/treatment algorithm includes urgent venography and thrombolysis for venous TOS and a workup for hypercoagulability. Neurogenic TOS is diagnosed clinically, whereas other studies are rarely beneficial.     

Intravenous landiolol, a novel β(1)-adrenergic blocker, reduces the minimum alveolar concentration of sevoflurane in women

Study Objective

To investigate the effect of intravenous (IV) landiolol, a novel β₁-adrenergic blocker, on the minimum alveolar concentration (MAC) of sevoflurane in adult women.

Design

Prospective, randomized study.

Setting

University hospital.

Patients

42 ASA physical status 1 and 2 women, aged 24-57 years, who were scheduled to undergo elective abdominal surgery.

Interventions

Anesthesia was induced in all patients by vital capacity rapid inhalation induction of sevoflurane. In the landiolol group, administration of landiolol began when patients took a vital-capacity breath: 0.125 mg/kg/min for one minute and then 0.04 mg/kg/min. Normal saline was administered in the control group.

Measurements

MAC was determined by a technique adapted from the conventional up-down method.

Main Results

The MAC of sevoflurane was 2.2% ± 0.2% in the control group and 1.7% ± 0.2% in the landiolol group, a statistically significant difference (P = 0.0005).

Conclusions

IV landiolol reduces the MAC of sevoflurane in women by approximately 20%.     

Stapler vs suture closure of pancreatic remnant after distal pancreatectomy: a meta-analysis

Background

Suture closure and stapler closure of the pancreatic remnant after distal pancreatectomy are the techniques used most often. The ideal choice remains a matter of debate.

Methods

Five bibliographic databases covering 1970 to July 2009 were searched.

Results

Sixteen articles met the inclusion criteria. Stapler closure was performed in 671 patients, while suture closure was conducted in 1,615 patients. The pancreatic fistula rate ranged from 0% to 40.0% for stapler closure of the pancreatic stump and from 9.3% to 45.7% for the suture closure technique. There were no significant difference between the stapler and suture closure groups with respect to the pancreatic fistula formation rate (22.1% vs 31.2%; odds ratio, .85; 95% confidence interval, .66-1.08), although there was a trend toward favoring stapler closure. In 4 studies including 437 patients, stapler closure was associated with a trend (not statistically significant) toward a reduction in intra-abdominal abscess (odds ratio, .53; 95% confidence interval, .24-1.15).

Conclusions

No significant differences occur between suture and stapler closure with respect to the pancreatic fistula or intra-abdominal abscess after distal pancreatectomy, though there is a trend favoring stapler closure.     

Closure of giant omphaloceles by the abdominal wall component separation technique in infants

Background/Purpose

Several techniques have been described to repair giant omphaloceles. There is no procedure considered to be the criterion standard worldwide. The aim of the present prospective study was to analyze the early and late results of secondary closure of giant omphaloceles using the component separation technique (CST) in infants.

Methods

From January 2004 to January 2007, 10 consecutive pediatric patients with a giant omphalocele were treated at our department. Initially, patients were treated conservatively. After epithelialization of the omphalocele, the abdominal wall was reconstructed using CST. Patients were monitored for complications during admission, and all patients were seen for follow-up.

Results

Component separation technique was performed at median age of 6.5 months (range, 5-69 months). The median diameter of the hernia was 8 cm (range, 6-9 cm). There was no mortality. The postoperative course was uneventful in 7 patients. Complications were seen in 3 patients (infection, skin necrosis, and hematoma). Median hospital stay was 7 days. After median follow-up of 23.5 months (range, 3-39 month), no reherniations were found.

Conclusions

The CST is a safe 1-stage procedure for secondary closure in children with a giant omphalocele without the need for prosthetic material and with good clinical outcome.     

Caecal volvulus

Background

Caecal volvulus accounts for 30% of all cases of volvulus of the colon.

Methods

We recorded clinical data and accurate images of volvulus of the cecum.

Results

A detorsion with resection and primary anastomosis was performed. We reviewed the management and surgical strategies for cecal volvulus.

Conclusions

Caecal volvulus is an uncommon entity with potentially severe outcomes and requires surgical management.     

Impact of omphalocele size on associated conditions

Purpose

Omphalocele is often associated with the presence of other congenital anomalies. Case reports have demonstrated nonclassical associations occurring in smaller omphaloceles. The aim of this study was to determine if omphalocele defect size correlates with the type of anomalies found.

Methods

Patient records at a pediatric hospital were retrospectively reviewed for an 8-year period. Data were collected on patient demographics, omphalocele size, and congenital anomalies identified. Size of the abdominal wall defect was determined by either physical examination or operative record of repair. Patient cohorts were designated as those with small (4 cm and less) or large (greater than 4 cm) omphaloceles.

Results

Fifty-three cases of omphalocele were observed. Twenty-seven cases were classified as small, with 26 classified as large. A predominance of males was noted in the small omphalocele group (78% vs 42%; P = .01). Intestinal anomalies, including Meckel's diverticulum and intestinal atresia, were only seen in patients with small omphaloceles. Most cardiac anomalies were associated with large omphaloceles (34.6% vs 3.7%; P = .01).

Conclusion

Small omphalocele size correlates with an increased prevalence of associated gastrointestinal anomalies, a lower prevalence of cardiac anomalies, and a higher predominance of male sex.     

Compartment syndrome in children and adolescents

Background

The authors performed a computerized search of the National Pediatric Trauma Registry for all patients who had compartment syndrome during a 51-month period.

Methods

One hundred thirty-three cases were identified. Boys outnumbered girls 4 to 1. The median age was 12 years and the peak incidence was in the 10- to 14-year-old age group.

Results

The most common mechanisms of injury were pedestrians struck by motor vehicles, falls, sports, and occupants in motor vehicle crashes. Eighty-five percent of the cases were the sequelae of fractures. Forearm fractures were the most common cause in the upper extremity, and tibia and/or fibula fractures were most common in the lower extremity.

Conclusions

Open fractures significantly increased the risk of developing a compartment syndrome for both forearm and leg fractures. Sixty percent of the patients went directly from the emergency room to the operating room, suggesting that the others developed the compartment syndrome after admission, or had delayed diagnosis.     

Demographic and environmental risk factors for gastroschisis and omphalocele in the National Birth Defects Prevention Study

Background

Primary prevention efforts for both gastroschisis and omphalocele are limited by the lack of known risk factors. Our objective was to investigate associations between potential maternal risk factors and gastroschisis and omphalocele within a large population-based sample of participants enrolled in the National Birth Defects Prevention Study (NBDPS).

Methods

Demographic, health-related, and environmental exposure data from the NBDPS were collected from women with expected delivery dates between October 1997 and December 2003. Data were collected on 485 cases of gastroschisis, 168 cases of omphalocele, and 4967 controls.

Results

Women who had offspring with gastroschisis were younger (adjusted odds ratio [AOR], 0.84; 95% confidence interval [CI], 0.81-0.86) and less likely to be black (AOR, 0.54; 95% CI, 0.34-0.85) than controls. They also were more likely to have smoked (AOR, 1.51; 95% CI, 1.12-2.03), taken ibuprofen (AOR, 1.61; 95% CI, 1.23-2.10), and consumed alcohol (AOR, 1.38; 95% CI, 1.06-1.79) than controls. Women who had offspring with omphaloceles were more likely to have consumed alcohol (AOR, 1.53; 95% CI, 1.04-2.25) and be heavy smokers (AOR, 4.26; 95% CI, 1.58-11.52) than controls.

Conclusions

Our results suggest a moderately increased risk of gastroschisis among women who used tobacco, alcohol, and ibuprofen during early pregnancy. A modestly elevated risk was observed for omphaloceles among women who used alcohol during the first trimester and among women who were heavy smokers.     

Reversal of Graft Steatosis After Liver Transplantation: Prospective Study

Objective

To determine the risk factors for reversal of liver graft steatosis.

Patients and Methods

This prospective study included 70 patients (47 men and 23 women) who received steatotic liver grafts between July 2003 and February 2008. No grafts from prisoners were used in the study. Patients were divided into 3 groups according to degree of liver steatosis, as follows: mild (n = 29, group 1), moderate (n = 23, group 2), and severe (n = 18, group 3).

Results

The median (SD) degree of steatosis in liver grafts at transplantation was 15.7% (7.3%) in group 1, 26.3% (10.5%) in group 2, and 45.1% (8.3%) in group 3. Postoperative histologic analysis demonstrated dramatically decreased steatosis in all graft recipients.

Conclusion

Graft steatosis can be decreased substantially after liver transplantation. Factors for reversibility of steatosis include donor age, degree of macrovesicular steatosis, and cold ischemia time.     

Histologic oddities at the porta hepatis in biliary atresia

Purpose

Highly unusual histologic findings at the porta hepatis in 3 infants who underwent Kasai portoenterostomy for biliary atresia are reported.

Methods

Portoenterostomy was performed using a standard operative technique. Serial transverse sections of the excised portal plate were examined by light microscopy along with sections from the distal extrahepatic biliary remnants, gallbladder, and liver biopsy.

Results

Of 61 consecutive infants who underwent Kasai portoenterostomy for biliary atresia, 3 were found to have highly unusual histologic features at the porta hepatis. All had type 3 biliary atresia. Two had hilar biliary ductules lined in part by squamous epithelium, and the third had a focus of mature hyaline cartilage surrounded by perichondrium adjacent to biliary ductules. In each case, these unusual histologic features were localized to the porta hepatis in the region of the transected portal plate.

Conclusions

The presence of hyaline cartilage at the portal plate is likely to be an expression of defective morphogenesis, thus supporting the concept of disordered embryogenesis in the etiology of biliary atresia. Squamous epithelium within biliary ductules might also reflect a similar mechanism but could alternatively be an unusual metaplastic response to inflammation at this site.     

Laparoscopic unroofing of splenic cysts results in a high rate of recurrences

Purpose

Laparoscopic unroofing is described as an appropriate treatment modality of nonparasitic splenic cysts. However, we repeatedly encountered recurrences with this technique. Because splenic cysts are rare, we analyzed the combined experience of 3 German pediatric surgical departments.

Materials and Methods

Between 1995 and 2005, primary and secondary nonparasitic splenic cysts were unroofed laparoscopically in 14 children (aged 5-12 years; median, 8.5 years). In 3 patients, the inner surface was coagulated with the argon beamer. In most children, the cavity was surfaced with omentum. In addition, in 4 patients the omentum was sutured to the splenic parenchyma.

Results

No intraoperative complications occurred, and no inadvertent splenectomy or blood transfusions were necessary. However, in 9 children (64%) the cysts recurred at intervals ranging from 6 to 12 months (median, 12 months). Also, argon laser treatment of the surface resulted in recurrence.

Conclusion

Laparoscopic unroofing of true splenic cysts alone proved inadequate in this series. Either removal of the inner layer or partial splenectomy appears to be necessary to prevent recurrences.     

The use of human acellular dermis in the operative management of giant omphalocele

Purpose

The management of giant omphalocele presents a major challenge to pediatric surgeons. Current treatment modalities often result in wound infection, fascial separation, and abdominal domain loss. Human acellular dermis (AlloDerm), as a primary abdominal fascial substitute, may prevent these complications. We present our experience with its application in neonates with giant omphalocele.

Methods

Charts of patients with giant omphalocele from January 2003 to September 2004 were reviewed and data collected regarding wound healing, rate of infection, ventilatory support, and outcome.

Results

Three neonates underwent abdominal wall closure with AlloDerm (gestational ages: 38, 37, and 28 weeks; birthweights 2880, 2640, and 1160 g, respectively). All had cardiac anomalies; 1 required cardiac surgery and 1 was ventilator-dependent, secondary to pulmonary hypoplasia. Omphalocele repair was performed on day-of-life 9, 2, and 87. No fascial dehiscence or infection was encountered. Neovascularization was noted by day 7. Two died of cardiopulmonary disease (6 months and 1 year). The third exhibited normal growth and development without complication.

Conclusions

AlloDerm provides visceral coverage without compromising cardiopulmonary function, diminishing abdominal domain, or requiring multiple operations, allowing prompt treatment of associated anomalies. AlloDerm represents an exciting alternative in the treatment of giant omphalocele. Further study is required to determine long-term benefits.     

Establishment of an Ex Vivo Lung Perfusion Model Using Non-Heart-Beating Large Pigs

Background

Functional evaluation of potentially damaged lungs donated after cardiac death is crucial for widespread clinical transplantation. To date, the mean weight of animals used in studies of ex vivo lung perfusion (EVLP) has been 60 kg; however, in the clinical setting, donor weight may be greater.

Objective

To investigate EVLP using lungs from large pigs (mean weight, 115 kg) to simulate human adult lungs donated after cardiac death.

Materials and Methods

Five heart-lung blocks were obtained at 20 minutes after death at the slaughterhouse. The lungs were flushed and preserved on ice for 6 hours before being connected to an ex vivo lung circuit, and were perfused for at least 2 hours.

Results

In all cases, perfusion was sustained for at least 2 hours. Mean (SEM) final flow rate was 4.9 (0.1) L/min, pulmonary artery pressure was 14.8 (1.7) mm Hg, and oxygen tension/fraction of inspired oxygen was 518.0 (18.0) mm Hg. The shunt fraction was 20.5% (4.0%). Histologic analysis demonstrated no significant pulmonary edema at the end of perfusion.

Conclusion

We successfully completed EVLP using lungs from large pigs.     

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

Background

The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM.

Method

We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period.

Results

Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%.

Conclusion

Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.     

Ipsilateral talar and calcaneal fractures: A retrospective review of complications and sequelae

Background

Ipsilateral talar and calcaneal fractures represent a rare combination injury that has only recently been reported in the literature with small case series.

Objective

To identify the commonly observed fracture patterns, complications, and outcomes of a consecutive series of patients with ipsilateral talar and calcaneal fractures.

Methods

Forty-five cases of ipsilateral talus and calcanal fractures were identified from an orthopaedic trauma registry at a University-based, level I trauma center for retrospective review.

Main outcome measurements

Post-operative complications, the need for secondary surgery, and the visual analogus pain score.

Results

Five patients were treated with an early below knee amputation (BKA). Five patients were treated with a primary subtalar arthrodesis. Twenty-eight of the 35 patients who did not undergo early BKA or primary subtalar arthrodesis developed subtalar arthritis. Five patients had deep wound complications. Four patients had talar body collapse from avascular necrosis. There were 13 open fractures of which 8 resulted in an eventual BKA. The mean visual analogus pain score for the patient population was 4.0.

Conclusion

The combination of ipsilateral talar and calcaneal fractures represents a severe injury pattern that is associated with significant morbidity. Subtalar arthritis was a common finding regardless of treatment. Open fractures frequently resulted in a below knee amputation.     

Conditional mutation of fibroblast growth factor receptors 1 and 2 results in an omphalocele in mice associated with disruptions in ventral body wall muscle formation

Background/Purpose

We observed that fibroblast growth factor receptors 1 and 2 (Fgfr1, Fgfr2) are expressed during abdominal wall development in mice and hypothesized that conditional mutation of these genes would result in abdomial wall defects.

Methods

Section in situ hybridizations were performed for Fgfr1 and Fgfr2 on wild-type embryos at embryonic day (E) 11.5 and E13.5. Conditional mutation of Fgfr1and Fgfr2 was achieved with a tamoxifen inducible Cre at E8.5. Litters were harvested at E17.5, whole mount photographs were taken, and paraffin sections were generated and stained with hematoxylin and eosin.

Results

Fgfr1 was expressed in ectoderm, lateral plate mesoderm, and myoblasts, whereas Fgfr2 was expressed almost exclusively in the early dermis and ectoderm of the abdominal wall. Conditional mutation of both Fgfr2 alleles and one Fgfr1 allele resulted in omphalocele in 38.7% of mutants. Histologic examination in mutants demonstrated disruptions in dermal and muscle development.

Conclusions

Mutant embryos with omphalocele arising from mutation in Fgfr1 and Fgfr2 exhibit disruptions in the development of the secondary abdominal wall structures. These findings are consistent with a model of ventral abdominal wall development in which organization of the muscles and connective tissue (secondary abdominal wall structures) is influenced by positional information emanating from the primary abdominal wall.     

Influence of stapler size used at ileal pouch-anal anastomosis on anastomotic leak, stricture, long-term functional outcomes, and quality of life

Background

The aim of this study was to evaluate whether stapler size used at ileal pouch-anal anastomosis (IPAA) influences outcomes.

Methods

Data of patients undergoing stapled IPAA (1983-2007) were obtained. Differences between groups A (stapler size 28-29 mm) and B (31-33 mm) for pre- and perioperative factors, stricture, leak, quality of life (QOL), and function were compared. Associations between stapler size and stricture or leak were assessed with a multivariable Cox model.

Results

Groups A (n = 1,221) and B (n = 899) had comparable age, diagnosis, body mass index (BMI), and albumin level. Group B had more males (P < .001) but fewer patients with ileostomy (P < .001). There was no significant difference in rates of leak (4.5% vs 6.2%, P = .08) or stricture (1.9% vs 2.7%, P = .1) for groups A and B. On multivariate analysis, female gender was associated with stricture, while greater BMI and male gender were associated with leak. Group A had greater urgency at 1 year and nighttime pad use at 15 years. The other determinants of function and QOL were similar.

Conclusions

There was no significant association between the size of stapler used at IPAA and long-term complications.     

Depression and quality of life in terms of personal resources in heart transplant recipients

Background

Heart transplantation is the first option for treatment of heart failure engendering increased survival and quality of life among recipients. However, this surgical intervention causes many psychological problems such as depression and anxiety. Protective factors and personal recourses are significant forces behind healthy adjustments to life stresses.

Purpose

The aim of this study was to estimate the prevalence of depression among heart transplant recipients.

Procedure

The study consisted of a sample of 46 patients after heart transplantation. Standardized instruments used to measure the key constructs were Beck Depression Inventory Short Form for the prevalence of depression, World Health Organization Quality of Life - BREF for quality of life, Sense of Coherence (SOC-29), and Coping Orientation to Problems Experienced BREF to identify coping strategies. The data were analyzed statistically.

Results

We found that sense of coherence and coping strategies were significant predictors for quality of life and prevalence of depression, which were significantly associated with each others. Strategies focused on the problem are moderate quality of life with an age of recipient. Recipients who have a tendency to use emotion-focused strategies and are older showed a poorer quality of life, were less satisfied with their health, and displayed a prevalence of depression.

Conclusions

These results suggested that assessment of coping strategies and sense of coherence should be explored in heart transplant recipients with skills training in this domain.