Intestinal Atresias.

Intestinal atresia is a form of congenital bowel obstruction that requires operative repair in the early neonatal period. Duodenal atresia and jejunoileal (JI) atresia are appropriately seen as distinct entities. Both can be suspected with fetal imaging, which can assist with prenatal counseling of families. Duodenal atresia is more commonly associated with comorbidities, whereas JI atresia is more often an isolated finding. Surgical repair is essential and is typically well tolerated. Although it may take time to achieve intestinal function postoperatively, these infants are usually able to tolerate full feeds after resolution of the ileus. Excellent short- and long-term outcomes for isolated duodenal atresia and JI atresia are expected.

Hypertrophic Pyloric Stenosis.

Hypertrophic pyloric stenosis is a common condition seen in the first 1 to 3 months after birth. Patients typically present with nonbilious projectile emesis after feeds that may result in hypokalemic, hypochloremic metabolic alkalosis. Although inability to tolerate feeds is frequently seen with self-limited conditions such as reflux, a low threshold to obtain an ultrasonographic image is important to prevent a delay in diagnosis. Although operative intervention is the treatment, it is imperative that patients are hydrated and serum electrolyte concentrations normalized before the induction of anesthesia. Laparoscopic pyloromyotomy is safe and effective. Postoperative emesis is normal, and reassurance to parents is appropriate. There is no significant long-term physiologic impairment from pyloric stenosis after successful surgical intervention.

Clarifying Misleading Lumps and Sinuses in the Newborn.

Neonates often have congenital lumps or sinuses. It is expected that pediatricians will distinguish those with important physiologic implications from those without. Accurate understanding of these lesions is important for the practitioner to avoid unnecessary tests and anxiety and to ensure that seemingly benign lesions with important implications are addressed in a timely manner. This review aims to clarify the consequences of some lesions that can easily be misinterpreted, offering guidance in the initial management of patients with congenital lumps or sinuses. We address several lesions that can easily be misconstrued, including wattles, preauricular lesions, sacral sinuses, second branchial cleft anomalies, torticollis, and dermoid cysts.

Necrotizing Enterocolitis.

Necrotizing enterocolitis (NEC) is a frequently encountered condition in the premature neonate, which can have devastating effects. The signs and symptoms of NEC are variable and can be confused with those of sepsis. An abdominal radiograph is often obtained for diagnosis, and findings that indicate NEC include pneumatosis and portal venous gas. The treatment of NEC includes gastrointestinal rest, gastric decompression, broad-spectrum intravenous antibiotics, and systemic support. A finding of pneumoperitoneum signifies intestinal perforation, which requires surgical intervention. Long-term sequelae of NEC include short-gut syndrome, intestinal stricture, and neurodevelopmental delays. The presentation of intestinal stricture can be puzzling. It can appear at presentation as a bowel obstruction or, conversely, as increased stool output or diarrhea. The clinician should have a high level of suspicion for intestinal stricture in a patient with a history of NEC.

Trends in the Diagnosis and Management of Pediatric Appendicitis.

• On the basis of class B evidence and consensus, acute appendicitis in children can often be diagnosed clinically with only selective use of imaging. (13)(14)(15)(16) • On the basis of class B evidence and consensus, ultrasonography is the test of choice when acute appendicitis is suspected but is unclear based on history, physical examination, and laboratory results. (17)(18)(19) • On the basis of class B evidence and consensus, the use of computed tomography scan should be limited to cases of suspected complex appendicitis with abscess or when there is clinical suspicion for acute appendicitis but ultrasonography results are not helpful. (16) • On the basis of class C evidence and consensus, children with possible appendicitis ideally should be treated in medical centers that have skilled sonographic personnel. (21) • On the basis of class B evidence and consensus, simple appendicitis should be treated by appendectomy during normal operating hours. Preoperative treatment with intravenous antibiotics and fluids during the overnight hours halts disease progression and allows for the safest surgery with the benefit of a full and rested staff. (24)(25)(26) • On the basis of class B evidence and consensus, complex appendicitis with a well-defined abscess can be treated nonoperatively initially, with the option of an interval appendectomy after recovery from the acute infection. (29)(30) (31)(32)(33)

Recent trends in the operative experience of junior pediatric surgical attendings: a study of APSA applicant case logs.

PURPOSE: Pediatric surgical education and workforce have changed significantly in the past decade. To document trends in the operative experiences of junior pediatric surgeons, we examined case logs submitted by applicants for membership to APSA. METHODS: Case logs for 164 APSA membership applicants from 2006 to 2013 were reviewed. Total case volume, categories, and specific operations were analyzed. Negative binomial regression assessed for significant associations between the number of cases and the application year, presence of a pediatric surgery training program, region of the country, and years since fellowship completion. RESULTS: Overall case numbers decreased initially after 2006/2007, but have remained stable since. Decreasing trends were seen in a number of specific cases/categories. The number of newborn cases did not change. Significant variations in operative experience were identified depending upon region, presence of a pediatric surgery training program, and years since fellowship completion. Median reported value for several important cases was ≤4 per year, and for some was zero. CONCLUSION: These data describing the experience of young pediatric surgeons supplement recent observations regarding pediatric surgery fellows and general surgery residents. The limited exposure of surgeons to particularly rare conditions appears to be an unresolved problem. This information will be useful in developing future workforce proposals.

Life-threatening postoperative hemorrhage from hepatic artery pseudoaneurysm successfully treated by transcatheter embolization in a 5-year-old.

Upper gastrointestinal bleeding caused by a pseudoaneurysm after hepatobiliary operation is well described in adults. This can be successfully treated with transcatheter embolization under angiographic guidance. We report a case of massive upper gastrointestinal bleeding in a 5-year-old boy secondary to a pseudoaneurysm of the right hepatic artery after choledochal cyst excision. A coil embolization successfully treated this life-threatening event and spared the child, the morbidity of a challenging gastrointestinal and vascular reconstruction. Use of percutaneous interventional technique to treat this rare complication of choledochal cyst excision has not been previously described in the pediatric surgical literature. Transcatheter embolization of a pseudoaneurysm may be a safe and less morbid treatment alternative for this surgical complication even in the pediatric population.

A comparison of the cleft lift procedure vs wide excision and packing for the treatment of pilonidal disease in adolescents.

BACKGROUND/PURPOSE: The cleft lift for pilonidal disease is a flap procedure designed to counteract suspected causes of closed-technique failure. This study compares cleft lift with wide excision and packing in adolescents with respect to complications, healing, and recurrence. METHODS: Charts of all patients surgically treated for pilonidal disease at our institution from August 2000 to August 2009 were reviewed retrospectively. Wide excision was routinely performed until May 2007 when the cleft lift as described by Bascom was instituted here. Factors examined were postoperative complications, wound healing, and disease recurrence. RESULTS: Seventy patients (49 males, 21 females; mean age, 16 years; mean weight, 170.5 lb) with pilonidal disease underwent a total of 39 cleft lift procedures and 34 wide excision procedures. All but 1 cleft lift patient (97.4%) healed completely, whereas 25 (73.5%) of 34 patients in the excision group healed (P < .001). The remaining 9 excision patients had chronic wounds, 3 of whom have undergone cleft lift with full healing. One cleft lift patient had recurrent disease (2.5%) compared with 7 (20.6%) of 34 excision patients (P < .02). CONCLUSIONS: The cleft lift procedure is a superior treatment method of pilonidal disease in adolescents, resulting in primary healing, lower likelihood of recurrent disease, and simplified wound care.

Nonoperative management of symptomatic urachal anomalies.

INTRODUCTION: Symptomatic urachal anomalies are rare disorders that consist of urachal remnants or fistulas with or without an associated cyst. Traditionally, when a urachal anomaly was recognized, operative excision was performed. There has been a shift toward the nonoperative management of urachal anomalies at many centers, although there is little in the literature to support this practice. METHODS: A retrospective chart review of patients with urachal anomalies was performed from January 2002 to March 2008. Children with a draining umbilicus and no radiographic or surgical confirmation of a urachal anomaly were excluded. RESULTS: Fifteen patients with symptomatic urachal anomalies were identified. The average age was 3.5 years (4 weeks to 14 years). Symptoms included umbilical drainage (n = 10), abdominal pain (n = 6), omphalitis (n = 4), intraabdominal mass (n = 3), dysuria (n = 1), recurrent urinary tract infections (n = 1), and fever (n = 4). The diagnosis was confirmed by ultrasound (n = 13) and/or computed tomographic scan (n = 4). The surgically treated cases included 7 urachal cysts (5 uninfected, 2 infected) and 1 patent urachal fistula. Mean follow-up is 37 months, and there have been no reported recurrences. Those treated without surgical excision included 4 patent urachal fistulas (mean follow-up, 20 months-no recurrences) and 3 infected urachal cysts (percutaneous drainage [n = 2] and laparoscopic drainage [n = 1]-no recurrences on ultrasound at 26 months). CONCLUSION: Nonoperative management of urachal anomalies is a reasonable approach and may be extended to infected urachal cysts after initial drainage. Infected cysts that are adequately drained seem to obliterate with time. Modern ultrasonography facilitates thorough follow-up. We propose a treatment algorithm for the management of suspected urachal anomalies.

Surgical management of upper gastrointestinal and small bowel Crohn's disease.

Burill Crohn's convincing description of the disease that now carries his name conceived of the illness as arising exclusively from the terminal ileum, involving other sites only secondarily. As a result, he took the condition to be curable by an adequate operative resection. The current concept is that Crohn's disease may affect any segment of the gastrointestinal tract. The practical implication of this change in thinking is the need to conserve bowel when weighing medical and surgical options for each child. Operations should be used to treat complications of the disease. Absolute indications for the surgery are uncommon and include perforation, bleeding, and refractory obstruction. The margins of resection need only include a short amount of grossly normal intestine. Strictureplasty to relieve obstruction without resection should be done when applicable. Maintenance medication after an operation to limit recurrence or recrudescence is frequently advocated.

Postoperative retrograde intussusception in an infant: a rare occurrence.

Retrograde intussusception is a rare postoperative complication, most commonly after gastric resection or gastrojejunostomy. This report describes a case of retrograde jejunojejunal intussusception after duodenal atresia repair.

The incidence of complete androgen insensitivity in girls with inguinal hernias and assessment of screening by vaginal length measurement.

BACKGROUND/PURPOSE: Complete androgen insensitivity syndrome (CAIS) is a rare disorder; however, surgeons have noted a higher rate in girls with inguinal hernias. A few retrospective studies have estimated the incidence of CAIS to be 0.8% to 2.4% in girls with inguinal hernias. An inexpensive, quick screening method for this population has not been established. Because CAIS is associated with a short vagina, measuring vaginal length could serve this purpose if normal standards were known. The authors endeavored to (1) prospectively confirm the incidence of CAIS, (2) identify normal standards of vaginal length, and (3) assess the usefulness of measuring vaginal length to screen for CAIS in girls with inguinal hernias. METHODS: Vaginal lengths were measured in 270 girls with inguinal hernias at a university hospital from 1991 to 2003. A fallopian tube was identified to exclude CAIS. If CAIS was suspected, gonadal tissue was sampled and karyotyping was performed. Linear regression analysis was performed, and 95% confidence intervals were calculated for individual values. RESULTS: Normal vaginal length for age was established. Three patients were found to have significantly short vaginas: 2 were confirmed to have CAIS, 1 did not (false-positive). One other infant was proved to have CAIS despite having a normal vaginal length (false-negative). The incidence of CAIS in our study was 1.1% (3/270). CONCLUSIONS: This is the largest prospective study of the incidence of CAIS in girls with hernias. The authors found that 1.1% of premenstrual girls with inguinal hernias have this syndrome. The authors also provide standards for normal vaginal length in the pediatric population. Vaginal length increases predictably with age, and some patients with CAIS have significantly shorter vaginas. Vaginal length is not a perfect predictor of this disorder, but can be a useful adjunctive screening tool.

Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia.

Almost a half-century since Kasai described the portoentersotomy for extrahepatic biliary atresia (EHBA), some questions about the management of this condition have been resolved and many are unanswered. The most useful diagnostic steps to aid in the diagnosis are debated. Sonography can be helpful but its sensitivity and selectivity are arguable with strong advocates for its effectiveness. Likewise, the magnetic resonance imaging has forceful advocates but also has not been universalized. The liver biopsy, done commonly before an operation for cholestasis, is often not discriminating. The radionuclide scan hepatobiliary iminodiacetic acid (HIDA) scan after phenobarbital stimulation is helpful if negative, but false positive results are common. It is agreed to proceed expeditiously to the operation in the cholestatic infant after a prompt investigation. The proposal to avoid this step and provide liver transplantation as initial management for EHBA has been suppressed by several clinical findings. The Kasai procedure has not worsened the outcome of eventual liver transplantation. The Kasai, even if it eventually fails, will often buy time and allow the child to grow before transplantation is needed. Multiple reoperations prior to the transplant are discouraged. Revisions to improve bile flow have not gained wide popularity. Use of a stoma to divert the bile has been largely abandoned. The need for frozen section examination of the liver at the site chosen for the portoenterostomy is no longer demanded. The preferred type of intestinal conduit is argued. Unanswered questions about the post-operative management include the role of steroids and of prophylactic antibiotics. The Biliary Atresia Research Consortium, a multi-institutional National Institutes of Health (NIH)-supported project, will address many of the unanswered issues.

A case confirming the progressive stages of pyomyositis.

A teenage boy presented in the early stage of pyomyositis. He had neck pain, tenderness, and fever. A computed tomography scan showed inflammation in the sternocleidomastoid muscle with no fluid collection. This progressed to a pus-filled drainable mass caused by Stapylococcus aureus. The authors describe this case to highlight the predictable stages and increase the index of suspicion to enhance its early recognition.