Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee.

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.

Damage control surgery in a <1 kg neonate: a brief report.

Damage control surgery is a feasible and successful approach for the management of unstable neonates with intra-abdominal catastrophes, including liver injuries. We report the case of a premature infant with a liver injury secondary to the placement of an umbilical vein catheter who was successfully managed using damage control surgery techniques.

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.

Operating on critically ill neonates: the OR or the NICU.

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.

Modified laparoscopic gastrostomy technique reduces gastrostomy tract dehiscence.

PURPOSE: To describe a modification in the Georgeson technique for laparoscopic gastrostomy tube (LGT) placement and compare the rate of reoperation for disruption of the gastrostomy tract after LGT by our modified technique to our own series of percutaneous endoscopic gastrostomy (PEG) tubes as well as the published rates of reoperation for LGTs and PEGs. METHODS: In 2003, we modified our technique for LGT to include laparoscopically placed sutures to secure the stomach to the abdominal wall. A retrospective review was performed on all children undergoing LGT placement and PEGs from March 2003 to October 2009. In addition, a review of the literature was performed to identify the published rates of complications for these procedures. RESULTS: During this time period, we have performed 85 LGT using this modified technique. In that same period, there have been 34 PEGs placed. The modification in our technique was instituted after a patient required reoperation for dislodgement in a laparoscopic U-stitch gastrostomy. To date, in the modified LGT group, there have been no disruptions of the gastrostomy tract in either the early or late periods, <90 days or >90 days, respectively. There have been 5 (5.9%) early dislodgements of the gastrostomy tube. All of these were before postoperative day 14 (at postoperative days 1, 2, 6, and 12), and all were replaced with placement verified by contrast study. None required reoperation. In the PEG group, there was 1 (2.9%) early and 1 (2.9%) late dislodgements and tract disruption that required reoperation. The published rate of dislodgement requiring reoperation in the Georgeson series of LGTs is 2.6%. The published rate of reoperation for dislodgement in PEGs is 4%-6%. CONCLUSION: This modification of the Georgeson technique has been successful in reducing the need for reoperation associated with gastrostomy replacement after dislodgement.

The appendix sign: a radiographic marker for irreducible intussusception.

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.

Early metastasizing spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid.

Spindle epithelial tumor with thymus-like elements is a rare thyroid lesion of children and young adults thought to be derived from branchial pouch remnants or foci of ectopic thymus. The lesion is poorly understood, and although it was originally believed to follow an indolent clinical course, its potential for late metastasis is becoming generally acknowledged. We have recently seen a unique case of this rare tumor in an 11-year-old boy, in which an unexpected and salient feature is the presence of a micrometastasis in a single lymph node at presentation. With the exception of 1 case with extensively infiltrative tumor and metastatic disease at the time of onset, in all other cases dissemination occurred years after surgical resection of the primary lesion. We review all previously reported cases and provide a detailed study of the histologic and ultrastructural appearances of this lesion.