Purpose Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. Methods Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question. Results 1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops. Conclusions Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.

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.

To the Editor, In March 2005 we submitted a case describing an 11year-old African-American boy with the first reported spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid found to have micrometastasis at initial presentation. As we previously described [1], SETTLE tumors arise from branchial pouch remnants or foci of ectopic thymic gland, and they primarily affect children and young adults. Originally believed to follow an indolent course, long-term follow-up has revealed that many of these patients develop late metastatic disease. Seven of 20 reported patients with SETTLE tumor developed metastasis to locations including lymph nodes, lung, vertebrae, and kidney. Based on the presentation of this 11-year-old boy with early metastatic disease, an aggressive behavior was predicted, and the patient was treated accordingly with postoperative chemotherapy. As a result of the rarity of this lesion, the uniqueness of this particular case, and the difficulty in treating such patients, we here describe his clinical progression since his initial presentation in June 2004. After surgical resection in October 2004, the patient was treated with 4 cycles of etoposide and cisplatin between December 2004 and February 2005, which he tolerated well. In September 2005, computed tomographic (CT) scan of the chest with intravenous contrast revealed a small 5-mm nodule within the left upper lobe. The lesion was followed by repeat CT in January 2006, at which time the lesion was noted to have increased in size to 1.2 cm. Elective lung wedge resection and pathologic evaluation confirmed metastatic SETTLE tumor. The now 13-year-old patient was placed on a chemotherapy regimen including etoposide, carboplatin, and ifosfamide, which he received from February until May 2006. Computed tomographic exam in August 2006 revealed more than 20 new pulmonary nodules, the largest of which measured 6 mm in diameter. On follow-up CT exam in October 2006, additional innumerable nodules were identified, and the previously noted nodules had increased in size. The lesions were considered to be consistent with metastatic disease. Additionally, a rounded soft tissue density is now present in the left paratracheal region adjacent to the original tumor site, and this lesion is also considered suspicious for recurrent SETTLE tumor. Unfortunately, this patient originally presented with micrometastatic disease and, as predicted, has rapidly progressed within 2 years time. Unlike a previously reported 12-year-old girl with metastatic disease after 1 year of delayed treatment [2,3], our patient displayed diffuse thyroid involvement by SETTLE tumor (as opposed to a nodular, circumscribed mass) and has not responded to chemotherapy treatment. In spite of postoperative chemotherapy and a 2nd round of chemotherapy after resection of a single pulmonary SETTLE lesion, this 13-year-old has developed multiple pulmonary nodules. In view of the potential for rapid progression in patients with SETTLE tumor, we recommend extensive pathologic sampling, and in cases presenting with lymphovascular invasion, lymph node metastasis, extrathyroidal extension, or infiltrative borders, adjuvant therapy as well as close long-term follow-up should be strongly considered.

PURPOSE:Radiographic reduction (hydrostatic or pneumatic) of intussusception has become the standard of care in the pediatric population with success rates of more than 80%. Identification of those patients who are likely to fail nonoperative management could lead to earlier operation, a reduction in radiation exposure, and a decreased risk for complications after repeated attempts at enema reduction. During successful radiographic reduction, the small bowel is almost always visualized before the appendix. Visualization of the appendix before visualization of the small bowel during a successful reduction of an intussusception is a rare event. We report a new radiographic sign that we have termed the appendix sign (radiographic visualization of the appendix without reflux of air or contrast into the small intestine), which we hypothesize may have association with failure of nonoperative management.METHOD:We performed a retrospective review of the last 12 years of irreducible intussusception. The associated studies were then reviewed to examine the incidence, sensitivity, and specificity of this radiographic finding.RESULTS:Ninety-one cases of intussusception were identified and had films available for review. Seventy-seven (76%) of the studies included the appropriate image. The appendix sign was visualized in 14 studies for an incidence of 18%. Of 14 patients, 10 failed enema reduction (positive predictive value, 71%). The sensitivity of the appendix sign is 43%. The specificity of the sign is 93%.CONCLUSIONS:Our experience suggests that the presence of an appendix sign is associated with failing enema reduction of an intussusception and may be useful as a marker for determining the end point for further attempts at radiographic reduction.

Advances in neonatal care have resulted in the survival of smaller infants with more complicated medical problems. From a surgical standpoint this has required novel approaches to patient care. Surgical care has evolved in many respects. Procedures performed on premature infants range from elective, minor procedures to major, emergent lifesaving interventions. The emergent nature of these surgical interventions has led to controversies in management. Certain conditions require surgical procedures that are commonly performed at the bedside by pediatric surgical specialists. Under other circumstances, the specific details of management are less uniform with wide variability in approach by different practitioners. The rationale in these cases is primarily driven by personal preference with a paucity of supportive data in the published literature to either support or contradict individual opinion. Nevertheless, the role of bedside procedures appears to be expanding. If these procedures are to be undertaken, significant planning is required to ensure a good outcome for the patient. Prospective data are needed determine which patients may benefit from this approach.

Purpose: The efficacy and safety of the new immunosuppressant agent, FK506 (tacrolimus), was assessed in pediatric renal transplant recipients over a mean 12-month follow-up period. Methods: Twenty pediatric renal transplant recipients received oral FK506 therapy (0.3 mg/kg/d) in combination with azathioprine (1 to 2 mg/kg/d) and lowdose prednisone as primary therapy (n = 11) or were converted from cyclosporine-based therapy (n = 9) for complications including cyclosporine toxicity (n = 2), acute refractory rejection (n = 4), and chronic rejection (n = 3). Patients were then followed-up prospectively to evaluate effectiveness of therapy and complications. Results: In the primary treatment group, 45% of patients had one or more rejection episodes. Two required OKT3 therapy (18%) for persistent rejection, with one (9%) graft loss at 3 months. All other episodes were treated effectively with FK506 dose adjustment and steroid pulses. Patient and graft survival was 100% and 91%, respectively, at 12 months mean follow-up. In the FK506 conversion group, two teenage girls with intractable acne and hirsutism were converted with complete resolution and no change in renal function. Four patients were converted for acute rejection: two who did not respond to steroid pulse and two who did not respond to both steroids and OKT3. All four grafts were salvaged (mean follow-up, 12 months; mean Creatinine [Cr], 1.1). Three patients were converted for biopsy-proven chronic rejection at 3, 10, and 12 years after transplant (mean Cr, 2.4) with two of three of patients stable with functioning grafts at 1 year after conversion. Complications of FK506 therapy included temporary insulin-dependent diabetes mellitus (10%), neurological complications (25%), renal toxicity (15%), and hypertension (85%). There were no cases of gastrointestinal toxicity, hepatic dysfunction, lymphoproliferative disorders, or life threatening viral infection. All symptoms of toxicity responded to dose adjustment. No patient required conversion from FK506 to other agents. Conclusion: This early experience indicates that FK506 in combination with low-dose steroids and azathioprine appears to provide safe and effective immunosuppression in the pediatric age group as a primary agent and may salvage grafts in patients with refractory steroid and OKT3 resistant rejection. Graft and patient survival is comparable to that seen with conventional cyclosporine-based immunosuppression.