Saif F. Hassan

Impact of prenatal evaluation and protocol-based perinatal management on congenital diaphragmatic hernia outcomes

BACKGROUND/PURPOSE Although intuitive, the benefit of prenatal evaluation and multidisciplinary perinatal management for fetuses with congenital diaphragmatic hernia (CDH) is unproven. We compared the outcome of prenatally diagnosed patients with CDH whose perinatal management was by a predefined protocol with those who were diagnosed postnatally and managed by the same team. We hypothesized that patients with CDH undergoing prenatal evaluation with perinatal planning would demonstrate improved outcome. METHODS Retrospective chart review of all patients with Bochdalek-type CDH at a single institution between 2004 and 2009 was performed. Patients were stratified by history of perinatal management, and data were analyzed by Fishers Exact test and Students t test. RESULTS Of 116 patients, 71 fetuses presented in the prenatal period and delivered at our facility (PRE), whereas 45 infants were either outborn or postnatally diagnosed (POST). There were more high-risk patients in the PRE group compared with the POST group as indicated by higher rates of liver herniation (63% vs 36%, P = .03), need for patch repair (57% vs 27%, P = .004), and extracorporeal membrane oxygenation use (35% vs 18%, P = .05). Despite differences in risk, there was no difference in 6-month survival between groups (73% vs 73%). CONCLUSIONS Patients with CDH diagnosed prenatally are a higher risk group. Prenatal evaluation and multidisciplinary perinatal management allows for improved outcome in these patients.

Modification of an evidence-based protocol for advanced appendicitis in children

INTRODUCTION We previously developed an evidence-based clinical pathway for children with advanced appendicitis. The pathway standardized the choice and duration of antibiotic therapy and established discharge criteria. Initially, the pathway led to a 50% decrease in the rate of superficial and deep surgical site infections and a significant decrease in hospital length of stay. Four years after implementation, we noted an increase in the infectious complication rate and the emergence of resistant bacteria to commonly used antibiotics. In this study, we prospectively collected peritoneal fluid cultures at the time of appendectomy in an effort to optimize our antibiotic therapy and decrease complication rates. METHODS Microbiology analysis of peritoneal fluid cultures obtained at the time of appendectomy was performed in patients with an intraoperative diagnosis of advanced appendicitis. Clinical information, including demographics, laboratory data, and postoperative outcomes were collected and compared to the historic cohort. X(2), Students t-test, and Fisher exact test were used where appropriate. RESULTS The historic and prospective cohorts were similar with respect to clinical and demographic data. The postoperative intra-abdominal abscess rate remained unchanged (28% from 24%, P = 0.603). Escherichia coli and Pseudomonas aeruginosa were the most commonly isolated aerobic bacteria from peritoneal fluid in the prospective cohort. Thirty-two percent of these patients had Pseudomonas spp., and 12% had Enterococcus spp. or Escherichia coli resistant to cefoxitin in their peritoneal fluid cultures. DISCUSSION A significant proportion (40%) of children with advanced appendicitis had organisms either not susceptible or resistant to our first line antibiotic in their peritoneal fluid cultures. Our clinical pathway now recommends piperacillin-tazobactam as the most effective empiric therapy for advanced appendicitis in children. Microbiologic analysis of peritoneal fluid at appendectomy may be used to tailor antibiotic therapy in advanced appendicitis.

Needle core vs open biopsy for diagnosis of intermediate- and high-risk neuroblastoma in children

BACKGROUND Open biopsy has been the mainstay for definitive diagnosis of neuroblastoma in pediatric patients. However, needle core biopsy may represent a faster, less invasive, and safer alternative to open biopsy in children. The purpose of this study was to compare safety and efficacy between needle core and open biopsy in the diagnosis of patients with intermediate- and high-risk neuroblastoma at our institution. METHODS We retrospectively reviewed the medical records of children with intermediate- and high-risk neuroblastoma who underwent open or needle core biopsies from 2002 to 2010. Data collected included patient demographics, tumor size, sample adequacy for diagnosis and risk stratification (histology and cytogenetics), length of hospital stay, time to initiate chemotherapy after biopsy, need for repeat biopsy, and both intraoperative and postoperative complications. Mann-Whitney U and Fishers exact tests were used for statistical analysis. RESULTS During the study period, 7 patients underwent needle core primary biopsies (5 intermediate-risk primary tumors and 2 high-risk primary tumors), and 4 patients underwent needle core biopsy for metastatic tumors, whereas 21 patients had open biopsies (10, intermediate risk; 11, high risk). Median age at biopsy and median tumor size were similar in both groups. There was no significant difference in adequacy of biopsy, need for repeat biopsy, time to initiate chemotherapy, length of stay, or minor complications. The rate of major complications differed significantly between the 2 groups with 0% after needle core biopsy vs 48% after open biopsy (P = .027). CONCLUSIONS In children, needle core biopsy is comparable in efficacy with open biopsy in the diagnosis of intermediate- and high-risk neuroblastoma with significantly lower rates of major postoperative complications. These findings warrant a larger scale evaluation of diagnostic needle core biopsies in pediatric patients with solid tumor.

Characterizing pilomatricomas in children: a single institution experience

BACKGROUND/PURPOSE Pilomatricomas, or calcifying epitheliomas of Malherbe, are among the most common superficial cutaneous soft tissue lesions in children. Familiarity with the presenting signs and symptoms allows for the diagnosis to be made on physical examination alone in most patients, avoiding expensive and unnecessary diagnostic imaging. METHODS A retrospective IRB-approved review of surgical pathology archives and medical records of all patients undergoing excision of pilomatricomas between 1982 and 2010 was performed to determine the characteristics of the pilomatricoma tumors. Data regarding gender, age, location, size of tumor, and histopathology were collected. RESULTS There were 916 pilomatricomas resected in 802 patients. Fifty-five percent of the patients were girls (441 patients). The median age at the time of resection was 6 years (range 5 months to 18 years). Multiple lesions were found in 43 patients (5%). The most common location was head and neck (n = 529, 58%), followed by upper limbs (n = 214, 23%), trunk (n = 130, 14%), and lower limbs (n = 43, 5%). Information on size was available for 674 lesions; mean lesion diameter was 14.0 ± 7.4 mm. Twenty-eight patients (3%) had either recurrent (n = 11) or metachronous (n = 17) lesions resected at our institution, with a median interval of 12 months after initial resection (range 5 weeks to 5 years). No cases of pilomatrix carcinoma were observed. CONCLUSION The majority of pilomatricomas occur in the head and neck, although they can present in any location. Approximately 5% of children have multiple lesions. Pilomatricomas occur slightly more commonly in girls, and 66% of lesions occur in children < 10 years of age. Complete surgical excision is necessary to prevent recurrence. Recurrences and pilomatrix carcinoma are very rare if complete excision is achieved.

Primary Suture-less Closure of Gastroschisis Using Negative Pressure Dressing (Wound Vacuum)

INTRODUCTION Primary suture-less closure of gastroschisis using negative pressure dressing (wound vacuum) involves the application of an initial preformed Silo with subsequent bedside suture-less closure of the defect using negative pressure dressing. The advantages of this gentle approach are the simple bedside closure without intubation, paralysis or ventilation as well as reduced risk of barotrauma, abdominal compartment syndrome, acidosis, bowel infarction and necrotizing enterocolitis. This study is a report of the technique we used for gastroschisis closure at our institute. METHODS The medical records of 15 newborns who underwent primary suture-less closure of gastroschisis using a negative pressure dressing (wound vacuum) technique between March 2008 and February 2010 were retrospectively reviewed. Outcome criteria such as time on ventilation, time to initiating feeds, time to full feeds, time to discharge from NICU and complications were recorded. RESULTS The median follow-up was 234 days (range: 13-528 days). The time on ventilation was (n = 8: no ventilation; n = 3: 1 day; n = 2: 2-5 days; n = 2: > 5 days). The median time to initiating feeds was 13 days (range: 6-61 days), the median time to full feeds was 20 days (range: 12-91 days) and the median time to discharge was 24 days (range: 21-131 days). 2 patients had a tiny umbilical hernia at last follow-up. CONCLUSION Primary suture-less closure of gastroschisis using a negative pressure dressing (wound vacuum) technique is easily reversible, does not need intubation/ventilation, avoids a trip to the operating room (OR), has minimal to no complications and provides good cosmetic results.

Novel use of glycopyrrolate (Robinul) in the treatment of anastomotic leak after repair of esophageal atresia and tracheoesophageal fistula

Anastomotic leak after tracheoesophageal fistula repair is a well-known complication and can represent a challenging clinical scenario. We present the case of an infant girl with VACTERL syndrome who underwent repair of a type C esophageal atresia and tracheoesophageal fistula repair, which was complicated by an anastomotic leak. Glycopyrrolate (Robinul), an anticholinergic agent, was successfully used to decrease copious salivary secretion and promote spontaneous closure of the leak. This report represents the first description in the medical literature of the use of glycopyrrolate in the treatment of an esophageal anastomotic leak. Glycopyrrolate may be a useful adjunct in the management of anastomotic leak after tracheoesophageal repair.

Esophageal bronchus in an infant—a rare cause of recurrent pneumonia

A 4-month-old female infant presented to our institute with recurrent pneumonia and was diagnosed with a right lower lobe esophageal bronchus. This congenital anomaly is extremely rare with very few cases reported in the literature. We describe the diagnostic workup and management that led to a successful outcome.