Robert A. Cowles

Solitary hepatic cysts.

The treatment of liver cysts continues to evolve in terms of diagnosis and surgical therapy. In the past, hepatic cysts were usually discovered at laparotomy, but they have become an increasingly common incidental finding on radiographic studies such as ultrasonography and CT scan. Occasionally liver cysts present as symptomatic abdominal masses. Hepatic cysts can be divided into two general categories: congenital and acquired (Table 1). Because the cause and treatment of cystic lesions of the liver differ, depending on the lesion and on the presence or absence of symptoms, it is important to be familiar with the appropriate evaluation and management of these masses. This article reviews common presentations, diagnostic methods, and treatment of congenital and acquired liver cysts. Discussion of infectious cysts, including amoebic, pyogenic, and hydatid cysts, and complex multicystic liver disease, has been excluded from this review.

Parenteral nutrition–associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review

OBJECTIVE The aim of this study was to review evidence-based data addressing key clinical questions regarding parenteral nutrition-associated cholestasis (PNAC) and parenteral nutrition-associated liver disease (PNALD) in children. DATA SOURCE Data were obtained from PubMed, Medicine databases of the English literature (up to October 2010), and the Cochrane Database of Systematic Reviews. STUDY SELECTION The review of PNAC/PNALD has been divided into 4 areas to simplify ones understanding of the current knowledge regarding the pathogenesis and treatment of this disease: (1) nonnutrient risk factors associated with PNAC, (2) PNAC and lipid emulsions, (3) nutritional (nonlipid) considerations in the prevention of PNAC, and (4) supplemental medications in the prevention and treatment of PNAC. RESULTS The data for each topic area relevant to the clinical practice of pediatric surgery were reviewed, evaluated, graded, and summarized. CONCLUSIONS Although the conditions of PNAC and PNALD have been well recognized for more than 30 years, only a few concrete associations and treatment protocols have been established.

Malrotation and midgut volvulus: a historical review and current controversies in diagnosis and management

The complex and sometimes controversial subject of malrotation and midgut volvulus is reviewed commencing with the 19th-century Bohemian anatomist, Václav Treitz, who described the suspensory muscle anchoring of the duodenal-jejunal junction in the left upper quadrant, and continuing with William Ladd, the 20th-century American “father of pediatric surgery” who pioneered the surgical treatment of midgut volvulus. In this review, we present the interesting history of malrotation and discuss the current radiologic and surgical controversies surrounding its diagnosis and treatment. In the symptomatic patient with malrotation and possible midgut volvulus, prompt diagnosis is critical. The clinical examination and plain film are often confusing, and delayed diagnosis can lead to significant morbidity and death. Despite recent intense interest in the position of the mesenteric vessels on US and CT scans, the upper gastrointestinal series remains the fastest and most accurate method of demonstrating duodenal obstruction, the position of the ligament of Treitz, and, if the contrast agent is followed distally, cecal malposition. Controversy exists over the management of asymptomatic patients with malrotation in whom the diagnosis is made incidentally during evaluation for nonspecific complaints, prior to reflux surgery, and in those with heterotaxy syndromes.

Reversal of intestinal failure–associated liver disease in infants and children on parenteral nutrition: experience with 93 patients at a referral center for intestinal rehabilitation

PURPOSE Intestinal failure (IF)-associated liver disease (IFALD) complicates the treatment of children with IF receiving parenteral nutrition (PN). We hypothesized that prevention or resolution of IFALD was possible in most children and that this would result in improved outcomes. METHODS We reviewed prospectively gathered data on all children referred to the intestinal rehabilitation and transplantation center at our institution. Total bilirubin level (TB) was used as the marker for IFALD. Patients were grouped based on TB at referral and at subsequent inpatient stays and outpatient visits. Standard treatment consisted of cycling of PN, limiting lipid infusion, enteral stimulation, use of ursodeoxycholic acid, and surgical intervention when necessary. Outcomes such as mortality, dependence on PN, and need for transplantation were assessed. Statistical analyses were performed using Fishers exact, Mann-Whitney U, and Wilcoxon signed rank tests. RESULTS Ninety-three patients with intestinal failure and on PN were treated at our center from 2003 to 2009. Median age at referral was 5 months (0.5-264 months). Prematurity was a complicating factor in 63 patients and necrotizing enterocolitis was the most common diagnosis. Eighty-two children had short bowel syndrome, whereas the remaining 11 had extensive motility disorders. 97% of children required significant alteration of their PN administration. At referral, 76 of 93 children had TB 2.0 mg/dL or higher, and 17 had TB below 2.0 mg/dL. TB normalized in 57 of 76 children with elevated TB at referral, and TB remained elevated in 19. Normalization of TB was associated with a mortality of 5.2%, and transplantation was needed in 5.2%. Conversely, when TB remained elevated, mortality was 58% (P = .0002 vs TB normalized), and transplantation occurred in 58% owing to failure of surgical and medical rehabilitation. CONCLUSIONS Most children referred for treatment of IF have IFALD. A dedicated IF rehabilitation program can reverse IFALD in many children, and this is associated with improved outcome.

Neuronal Serotonin Regulates Growth of the Intestinal Mucosa in Mice

BACKGROUND & AIMS The enteric abundance of serotonin (5-HT), its ability to promote proliferation of neural precursors, and reports that 5-HT antagonists affect crypt epithelial proliferation led us to investigate whether 5-HT affects growth and maintenance of the intestinal mucosa in mice. METHODS cMice that lack the serotonin re-uptake transporter (SERTKO mice) and wild-type mice were given injections of selective serotonin re-uptake inhibitors (gain-of-function models). We also analyzed mice that lack tryptophan hydroxylase-1 (TPH1KO mice, which lack mucosal but not neuronal 5-HT) and mice deficient in tryptophan hydroxylase-2 (TPH2KO mice, which lack neuronal but not mucosal 5-HT) (loss-of-function models). Wild-type and SERTKO mice were given ketanserin (an antagonist of the 5-HT receptor, 5-HT(2A)) or scopolamine (an antagonist of the muscarinic receptor). 5-HT(2A) receptors and choline acetyltransferase were localized by immunocytochemical analysis. RESULTS Growth of the mucosa and proliferation of mucosal cells were significantly greater in SERTKO mice and in mice given selective serotonin re-uptake inhibitors than in wild-type mice, but were diminished in TPH2KO (but not in TPH1KO) mice. Ketanserin and scopolamine each prevented the ability of SERT knockout or inhibition to increase mucosal growth and proliferation. Cholinergic submucosal neurons reacted with antibodies against 5-HT(2A). CONCLUSIONS 5-HT promotes growth and turnover of the intestinal mucosal epithelium. Surprisingly, these processes appear to be mediated by neuronal, rather than mucosal, 5-HT. The 5-HT(2A) receptor activates cholinergic neurons, which provide a muscarinic innervation to epithelial effectors.

Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study.

INTRODUCTION Experience in thoracoscopic congenital diaphragmatic hernia (CDH) repair has expanded, yet efficacy equal to that of open repair has not been demonstrated. In spite of reports suggesting higher recurrent hernia rates after thoracoscopic repair, this approach has widely been adopted into practice. We report a large, single institutional experience with thoracoscopic CDH repair with special attention to recurrent hernia rates. METHODS We reviewed the records of neonates with unilateral CDH repaired between January 2006 and February 2010 at Morgan Stanley Childrens Hospital. Completely thoracoscopic repairs were compared to open repairs of the same period. In addition, successful thoracoscopic repairs were compared with thoracoscopic repairs that developed recurrence. Data were analyzed by Mann-Whitney U and Fisher exact tests. RESULTS Thirty-five neonates underwent attempted thoracoscopic repair, with 26 completed. Concurrently, 19 initially open CDH repairs were performed. Preoperatively, patients in the open repair group required more ventilatory support than the thoracoscopic group. Recurrence was higher after thoracoscopic repair (23% vs 0%; P = .032). In comparing successful thoracoscopic repairs to those with recurrence, none of the factors analyzed were predictive of recurrence. CONCLUSIONS Early recurrence of hernia is higher in thoracoscopic CDH repairs than in open repairs. Technical factors and a steep learning curve for thoracoscopy may account for the higher recurrence rates, but not patient severity of illness. In an already-tenuous patient population, performing the repair thoracoscopically with a higher risk of recurrence may not be advantageous.

Strategies for the prevention of central venous catheter infections: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review

PURPOSE The aim of this study is to review the current evidence-based data regarding strategies for prevention of central venous catheter (CVC) infections at the time of catheter insertion and as a part of routine care. METHODS We conducted a PubMed search from January 1990 to November 2010 using the following keywords: central venous catheter, clinical trials, pediatric, infection, prevention, antibiotic, chlorhexidine, dressing, antiseptic impregnated catheters, ethanol lock, impregnated cuff, insertion site infection, and Cochrane systematic review. Seven questions, selected by the American Pediatric Surgical Association Outcomes and Clinical Trials Committee, were addressed. RESULTS Thirty-six studies were selected for detailed review based on the strength of their study design and relevance to our 7 questions. These studies provide evidence that (1) chlorhexidine skin prep and chlorhexidine-impregnated dressing can decrease CVC colonization and bloodstream infection, (2) use of heparin and antibiotic-impregnated CVCs can decrease CVC colonization and bloodstream infection, and (3) ethanol and vancomycin lock therapy can reduce the incidence of catheter-associated bloodstream infections. CONCLUSION Grade A and B recommendations can be made based on available evidence in adult and limited pediatric studies for multiple components of proper CVC insertion practices and subsequent management. These strategies can minimize the risk of CVC infections in pediatric patients.

Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review

The landmark 1933 case report from Massachusetts General Hospital by Bland, White and Garland (Am Heart J 8:787–801) described a 3-month-old child with progressive feeding problems, cardiomegaly on chest radiography, and EKG evidence of left ventricular damage. Of interest was the fact that the vigilant father of the infant was Aubrey Hampton, a radiologist and future chairman of radiology at Massachusetts General Hospital. At autopsy, the left coronary artery originated from the pulmonary artery rather than from the aorta. Effective treatment for this condition was not available until 1960 when Sabiston, Neill and Taussig showed that the blood flowed from the left coronary artery toward the pulmonary artery. The anomalous left coronary artery was ligated at its junction with the pulmonary artery and the child survived. This historical review of Bland-White-Garland syndrome, now known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA), stresses the continued diagnostic significance of cardiomegaly on chest radiography and EKG changes suggesting left ventricular damage in 2- to 3-month-old infants with feeding intolerance or irritability. With a high index of suspicion, an echocardiogram can be obtained to confirm the diagnosis. Modern surgical methods involve left coronary artery translocation and afford excellent outcomes.

Epidermoid cyst of the spleen

the remaining 10%. 1 Patients with true splenic cysts are usually young and present, as our patient did, with an abdominal mass associated with mild symptoms related to cyst enlargement. Often, these cysts are completely asymptomatic and are discovered incidentally during imaging studies or at autopsy. The differential diagnosis may include epidermoid/dermoid cyst, parasitic cyst, splenic infarction, and splenic abscess. A preoperative ultrasound scan or, preferably, abdominal CT scan is helpful in determining both the cyst’s site of origin and its relationship to surrounding structures. Furthermore, these imaging studies can help plan the optimal operative approach, especially if a laparoscopy is being considered. In patients with asymptomatic true splenic cysts, a course of conservative management is reasonable. In a recent collective review of 191 cases of these cysts reported in the literature, only 10 (5.2%) instances of cyst-related complications were reported. 1 Of these 10 complications, 6 involved cyst rupture and 4 involved cyst infection. There were no cases of cyst hemorrhage in this series. When a splenic cyst is symptomatic or if the diagnosis is in question, operative therapy is warranted. Total splenectomy, partial splenectomy, and cystectomy have been reported to be adequate treatments for these cysts. Limited treatments, such as catheter drainage or sclerosis, are associated with high rates of recurrence or infection and have largely been abandoned. After immunization against pneumococci, Haemophilus influenzae B, and meningococci infection, the patient underwent an exploratory laparotomy where a large cystic mass of the spleen was found, displacing the stomach and falciform ligament to the right (Figure 2). A splenectomy was performed without complication. Gross pathologic examination revealed a cystic spleen measuring 21 cm in greatest dimen- sion and weighing 2,100 g. The histologic findings were consistent with an epidermoid cyst. The patient had an uneventful recovery and was discharged home on postoperative day 4. She has remained well for more than 30 months.

Iatrogenic esophageal perforation in children

Esophageal perforation is most commonly iatrogenic in origin with nasogastric tube insertion, stricture dilation, and endotracheal intubation, being the most frequent sources of the injury in infants and children. Clinical presentation depends on whether the cervical, thoracic, or abdominal esophagus is injured. Any patient complaining of chest pain after an upper endoscopy has esophageal perforation until proven otherwise. In infants and children, plain chest films and esophagography may assist in making the diagnosis. Hemodynamically stable patients with a contained perforation may be managed medically. Free perforation and hemodynamic lability mandates a more aggressive surgical approach for wide drainage of the mediastinum and pleural spaces. Exploration of the chest for attempted direct repair of the injury is now only rarely indicated. Mortality rates have been reported between 20 and 28% with delays in diagnosis and treatment appearing to be most strongly correlated with poor outcomes.