Tim Jancelewicz

Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution

BACKGROUND/PURPOSE Surgical complications are common in survivors of congenital diaphragmatic hernia (CDH), but little is known about long-term incidence patterns and associated predictors. METHODS A cohort of 99 CDH survivors was prospectively followed at a single-institution multidisciplinary clinic. Data were gathered regarding the adverse surgical outcomes of hernia recurrence, chest and spinal deformity, and operative small bowel obstruction (SBO), and then were retrospectively analyzed in relation to perinatal and perioperative markers of disease severity to determine significant predictors. Statistical methods used included univariate and multivariate regression analysis, hazard modeling, and Kaplan-Meier analysis. RESULTS At a median cohort age of 4.7 (range, 0.2-10.6) years, 46% of patients with patch repairs and 10% of those with primary repairs had a hernia recurrence at a median time of 0.9 (range, 0.1-7.3) years after repair. Chest deformity was detected in 47%. Small bowel obstruction and scoliosis occurred in 13%. Recurrence and chest deformity were significantly more common with patch repair, liver herniation, age at neonatal extubation greater than 16 days, oxygen requirement at discharge, and prematurity. The strongest predictor of SBO was patch repair. Multivariate analysis showed that patch repair was independently predictive of recurrence and early chest deformity (odds ratios of 5.0 and 4.8, confidence intervals of 1-24 and 1-21, P < .05). Use of an absorbable patch was associated with the highest risk of surgical complications. CONCLUSIONS For long-term survivors of CDH, specific perinatal and operative variables, particularly patch repair, are associated with subsequent adverse surgical outcomes.

Predicting Strangulated Small Bowel Obstruction: An Old Problem Revisited

BackgroundDiagnosing intestinal strangulation complicating a small bowel obstruction (SBO) remains a considerable challenge. Despite decades of experience and numerous studies, no clinical indicators have been identified that reliably predict this life-threatening condition. Our goal was to determine which clinical indicators in patients with SBO can be used to independently predict the presence of strangulated intestine.MethodsMedical records were reviewed for 192 adult patients operated on for acute SBO over an 11-year period (1996–2006). Seventy-two preoperative clinical, laboratory, and radiologic findings at admission were examined. Data from patients with strangulated intestine were compared to data from patients without bowel compromise. Likelihood ratios were generated for each significant parameter in a multivariate logistic regression analysis.ResultsForty-four patients had bowel strangulation requiring bowel resection, and 148 had no strangulation. The most significant independent predictor of bowel strangulation was the computed tomography (CT) finding of reduced wall enhancement, with a sensitivity and specificity of 56% and 94% [likelihood ratio (LR) 9.3]. Elevated white blood cell (WBC) count and guarding were moderately predictive (LR 1.7 and 2.8).ConclusionRegression analysis of multiple preoperative criteria demonstrates that reduced wall enhancement on CT, peritoneal signs, and elevated WBC are the only variables independently predictive of bowel strangulation in patients with SBO.

Late surgical outcomes among congenital diaphragmatic hernia (CDH) patients: Why long-term follow-up with surgeons is recommended

BACKGROUND CDH patients experience multi-system morbidity. Despite apparent health, late childhood complications do occur. We reviewed the long-term surgical morbidity of our CDH patients to determine whether protracted clinical surveillance is warranted. METHODS A single-institution retrospective chart review of all CDH survivors treated from 1999 to 2011 who are followed at our CDH multidisciplinary clinic was performed. Descriptive and statistical analyses were performed to show risk of surgical complications over time. RESULTS A total of 187 CDH patients were treated with 160 surviving to discharge (86%). Primary repair was performed in 115 (73%), and 42 (27%) underwent patch repair. CDH recurrence occurred in 23 (15%) at a median time of 0.7 (range 0-8.5) years (65% asymptomatic). Seventy percent of recurrences occurred before 2 years and 17% after 4 years. Bowel obstruction occurred in 12 (8%) at a median time of 0.7 (range 0.2-7.2) years post-repair, and chest deformity occurred in 13 (8%) at a median of 5 (range 1.1-6.8) years. For patch repairs, scoliosis occurred in 4 (10%) patients at a median age of 3 (range 0.6-5) years. CONCLUSION Surgical complications in CDH survivors are common, can occur many years later, and are frequently asymptomatic. Long-term surveillance of CDH patients is recommended for early identification and treatment of complications.

Neonatal appendicitis: a new look at an old zebra

Acute neonatal appendicitis is a rare condition associated with significant morbidity and mortality. The severity of this disease is caused by its tendency to occur more frequently in premature infants, an increased perforation rate with rapid progression to peritonitis, and delay in diagnosis and intervention. Although appendicitis in the perinatal period may occur as an isolated event, in many cases it occurs in association with other pathologic states, including prematurity, inguinal hernia, and others. The presentation of neonatal appendicitis can be identical to necrotizing enterocolitis, leading to misdiagnosis. Here we report a case of perforated appendicitis in a 9-day-old boy with tetralogy of Fallot and esophageal atresia with tracheoesophageal fistula. In addition, we present a review of the clinical features of neonatal appendicitis and a discussion of previously described, associated conditions.

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

OBJECTIVE Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations. METHODS Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included. RESULTS Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material. CONCLUSIONS Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.

Laser microdissection allows detection of abnormal gene expression in cystic adenomatoid malformation of the lung

BACKGROUND/PURPOSE Congenital cystic adenomatoid malformation (CCAM) of the lung may result from a localized aberrant epithelial-mesenchymal interaction during lung development. We used laser microdissection (LMD) to isolate the epithelium and mesenchyme of CCAM, and studied candidate gene expression in these pure cell populations. METHODS Congenital cystic adenomatoid malformation tissue was obtained from fetal (n = 5) and postnatal (n = 5) surgical specimens. Normal fetal lung (n = 10) was obtained from abortus material, and normal postnatal lung (n = 5) was identified from surgical specimens. Whole tissue was analyzed using immunohistochemistry and reverse transcriptase polymerase chain reaction (RT-PCR). Using LMD, columnar bronchiolar type epithelium and underlying mesenchyme were isolated. Multiplex nested RT-PCR was then used to detect message levels of candidate genes. RESULTS Reverse transcriptase polymerase chain reaction performed on LMD-isolated tissue, but not whole tissue homogenate, revealed differences between CCAM and normal lung. In this report, we focus on the fibroblast growth factor (FGF) family. By RT-PCR, there was 4-fold more epithelial expression of FGF9 in fetal CCAM vs normal fetal lung (P < .07). This was qualitatively confirmed by immunohistochemistry. We also detected decreased FGF7 expression in CCAM mesenchyme (P < .05) but no significant differences in FGF10 or FGFR2. CONCLUSIONS LMD may be used to overcome the limitations of tissue heterogeneity in the study of CCAM. Abnormal growth factor expression may play a role in the etiology of this lesion.

A History of Fetal Surgery

Over the past 3 decades, fetal surgery for congenital disease has evolved from merely a fanciful concept to a medical field in its own right. Techniques for open hysterotomy, minimal-access hysteroscopy, and image-guided percutaneous fetal access have become well established, first in animal models and subsequently in humans. At the same time, major advances in fetal imaging and diagnosis, anesthesia, and tocolysis have allowed fetal intervention to become a vital tool for subsets of patients who would otherwise endure significant morbidity and mortality. This article offers a concise overview of the history of fetal surgery, from its tumultuous early days to its current status as an important means for the early treatment of potentially devastating congenital anomalies.

Delayed primary serial transverse enteroplasty as a novel management strategy for infants with congenital ultra-short bowel syndrome.

BACKGROUND Congenital ultra-short bowel syndrome (USBS) is a challenging problem with a poor outcome. We report a new management approach for USBS infants that attempts to optimize gut growth potential. METHODS We report five neonates with USBS in whom no correction was performed at primary surgery except placement of a gastrostomy (G) tube. Sham feeds were started with intermittent G-tube clamping to induce bowel dilatation/growth. Serial fluoroscopy was done until bowel caliber reached 5 cm. STEP was performed and continuity established to the colonic remnant. Small bowel length (SBL) and enteral caloric intake were tabulated. RESULTS Patients were born with a mean residual SBL of 19 ± 7.6 cm (14.8% of expected). Median duration of sham feeds prior to STEP was 108 (range 27-232)days. Mean SBL at STEP was 47 ± 12.1cm, which increased post-STEP to 70 ± 12.7 cm (a mean increase of 296% from birth, representing 36.4% ± 13.1% of expected gut length). With a median follow-up time of 20 months (range 8-28), 4/5 achieved >50% enteral calories and have normal liver function. One has undergone liver transplantation. CONCLUSIONS In USBS patients, delayed surgical correction with sham feeds accelerates gut growth, optimizing potential for autologous reconstruction. This approach may offer greater opportunity for intestinal adaptation than traditional options.

Thoracoscopic repair of neonatal congenital diaphragmatic hernia (CDH): outcomes after a systematic quality improvement process.

BACKGROUND Higher recurrence rates have been reported for thoracoscopic repair (TR) of neonatal congenital diaphragmatic hernia (CDH) compared to open repair. Our centre initiated changes in surgical management following a quality review in order to improve outcome. METHODS A retrospective review of TR patients from 2000 to 2011 at a single institution was performed. A review was done in 2007, and changes were implemented to decrease recurrence rates. These included use of pledgets, an extracorporeal corner stitch, liberal prosthetic patch use, lower insufflation pressures, and TR was limited to two experienced surgeons. Outcome data before and after this quality improvement process were compared. Non-TR patients from the same time period served as controls. Data are quoted as median (range) and non-parametric tests used to compare. P<0.05 was regarded as significant. RESULTS There were 23 neonatal TR patients with median follow-up of 3.6 (range 0.4-7) years. Median age at repair was 2 (range 0-21) days. There were 5 patch repairs (22%), all after 2008. There were 9 recurrences (39%) at a median time of 162 days after TR, compared to 13 (10%) recurrences in the control cohort. For primary TR, there was a trend towards a decreased recurrence rate from 50% prior to 2008 to 25% after 2008 (P=0.26). CONCLUSIONS Systematic quality review was modestly effective in decreasing the recurrence rate for neonatal TR, but further outcome data are required.

A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice

BACKGROUND Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice. METHODS A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed. RESULTS There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin <2.5mg/dL, gamma-glutamyl transpeptidase <150U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%. CONCLUSION We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.