Abdominal Wall Defects.

The 2 most common congenital abdominal wall defects are gastroschisis and omphalocele. Both are usually diagnosed prenatally with fetal ultrasonography, and affected patients are treated at a center with access to high-risk obstetric services, neonatology, and pediatric surgery. The main distinguishing features between the 2 are that gastroschisis has no sac and the defect is to the right of the umbilicus, whereas an omphalocele typically has a sac and the defect is at the umbilicus. In addition, patients with an omphalocele have a high prevalence of associated anomalies, whereas those with gastroschisis have a higher likelihood of abnormalities related to the gastrointestinal tract, with the most common being intestinal atresia. As such, the prognosis in patients with omphalocele is primarily affected by the severity and number of other anomalies and the prognosis for gastroschisis is correlated with the amount and function of the bowel. Because of these distinctions, these defects have different management strategies and outcomes. The goal of surgical treatment for both conditions consists of reduction of the abdominal viscera and closure of the abdominal wall defect; primary closure or a variety of staged approaches can be used without injury to the intra-abdominal contents through direct injury or increased intra-abdominal pressure, or abdominal compartment syndrome. Overall, the long-term outcome is generally good. The ability to stratify patients, particularly those with gastroschisis, based on risk factors for higher morbidity would potentially improve counseling and outcomes.

Ultrasonography and X-Ray guided drain placement to evacuate a pneumopericardium/pneumomediastinum in a 1-day-old infant.

Ultrasonographic (US) guided procedures have wide range of application in the abdomen and pelvis, however their role is somewhat limited in the chest due to complete reflection of the ultrasound beam by the air in the lungs, preventing the direct imaging of the tissues deep to the air-sound interface. Most of the chest procedures, other than the exception of thoracentesis, rely on the use of CT (computed tomography) scan. The disadvantages of using CT scan is the cost, lack of portability, and most importantly the radiation involved, particularly in case of infants and children, whose tissues are more radiosensitive than the adults. Identification of air by Ultrasonography can help direct needles and wires, to accomplish procedures which may otherwise need CT. A 1-day-old infant with respiratory distress syndrome (RDS) on a ventilator, developed an expanding symptomatic pneumopericardium/pneumomediastinum. The patient was too unstable to leave the neonatal intensive care unit (NICU), so a pericardial/mediastinal drain was placed under ultrasonographic and radiographic guidance. This case, highlights a method for bedside treatment of pneumopericardium/pneumomediastinum in an unstable neonate. This procedure may be equally effective in older children and adults.

Subcutaneoscopic Excision of External Angular Dermoid Cysts: Our Covert Scar Approach in 11 Cases.

INTRODUCTION: External angular dermoid cysts, or epidermoid inclusion cysts, are a common subcutaneous tumor of the head and neck. For the majority of these lesions, excision is relatively simple and performed through an incision immediately overlying the mass. Facial lesions in pediatric patients present a unique challenge in that a direct approach carries the potential for visible scar formation. OBJECTIVE: This article aims to detail our experience with subcutaneoscopic excision of external angular dermoid cysts in pediatric patients using endoscopic instrumentation. MATERIALS AND METHODS: Retrospectively, we reviewed 11 cases, between the ages of 4 months and 3 years with external angular dermoid cysts. An incision is made on the scalp above the hairline, then a tunneled working space is created underneath the skin. 3 mm laparoscopy instruments were then used for providing excellent visualization and precise subcutaneoscopic dissection. RESULTS: There were no complications apparent in any of the 11 cases (except cyst rupture in one case), with mean procedure duration at 61 minutes. Final results at follow-up revealed aesthetically pleasing and well-healed skin incisions, hidden from view behind the hairline. CONCLUSION: The subcutaneoscopic technique utilizing endoscopic instrumentation has the advantage of improved visualization of the cyst, greater precision of dissection, and excellent cosmesis. One disadvantage of this procedure is that this involves learning a new technique while the majority of surgeons are already comfortable with the open approach. Given the safety and efficacy observed for this subcutaneoscopic procedure, this technique can have tremendous possibilities.

Transumbilical repair of umbilical hernia in children: The covert scar approach.

BACKGROUND: Umbilical hernia repairs are one of the most commonly performed operations in children. The traditional repair involves an infraumbilical incision, which produces a visible scar. We report a novel technique of umbilical hernia repair through a transumbilical incision, which eliminates the scar by hiding it within the umbilicus. METHODS: We performed a retrospective chart review of 134 patients who had undergone a transumbilical hernia repair at a single institution between 2008 and 2016. Satisfaction with cosmesis and the presence of complications were assessed through parental interviews during follow up visit or by telephone survey. These data were compared to a large volume retrospective analysis of the standard infraumbilical approach. RESULTS: 121 of the 134 patients were evaluated in the clinic or by telephone interview. The overall complication rate was 7.44%. Parents of 118 patients reported satisfaction with the cosmetic result (97.52%). In comparison to the largest study of pediatric infraumbilical repair, there was an improvement in subjective cosmesis without a significant increase in complications. CONCLUSION: Transumbilical hernia repair is a safe and cosmetically appealing technique for umbilical hernia repair in children. LEVEL OF EVIDENCE: Treatment study, level III.

Esophageal stents in children: Bridge to surgical repair.

Management of complex esophageal problems in children is challenging. We report our experience with the use of esophageal stents in three children with esophageal strictures, leaks, or airway-esophageal fistulae refractory to conventional treatment. The stent played a key role in allowing extubation of a child with a large tracheo-esophageal-pleural fistula and in the resolution of pulmonary infection in a child with esophago-bronchial fistula, both followed by surgery. In the third child, with stricture, stents were complicated with migration, esophageal erosion, and esophago-bronchial fistula. In our experience, esophageal stents were useful mainly as a bridge to definitive surgical repair.

Innovative Use of Denver Shunt in a Child with Generalized Lymphatic Anomaly.

BACKGROUND: Denver® shunts have traditionally been used for palliation of refractory malignant and chylous peritoneal and pleural collections. We describe an innovative use of the Denver shunt in a child with generalized lymphatic anomaly. MATERIALS AND METHODS: Retrospective chart review of a 6-year-old girl with generalized lymphatic anomaly, who presented with refractory lymphorrhea from the labium majus, was performed. This was managed with innovative placement of the Denver shunt between a large abdominal wall cyst and the peritoneal cavity. RESULTS: There was progressive reduction in lymphorrhea with complete cessation at 11 months post shunt placement. At 33-month follow-up, the shunt remains patent with no further lymphorrhea. CONCLUSION: Denver shunts can be utilized for the creation of internal drainage pathways besides its traditional uses for draining peritoneal and pleural fluids.

Papillary adnexal neoplasm (aggressive digital papillary adenocarcinoma) on the ankle of a 15-year-old girl: case report and review of literature from a pediatric perspective.

Digital papillary adenocarcinoma is a rare malignant tumor of the sweat glands, most commonly encountered in adult males. Only few pediatric cases have been reported, with an apparent predominance of females and none on the ankle. We report a case of a 15-year-old girl with a slowly growing, non-tender mass on the right ankle presenting with difficulty in walking; clinically believed to be a keloid. Excision of the mass revealed a glandular neoplasm with largely eccrine and focally apocrine differentiation. Histologic features do not reliably predict biologic behavior. Because of the existing confusion over the nomenclature, we propose that such tumors be called 'papillary adnexal neoplasms of distal extremities'.

Multimodality Image-Guided Sclerotherapy of Low-Flow Orbital Vascular Malformations: Report of Single-Center Experience.

PURPOSE: To evaluate the role of multimodality imaging tools for intraprocedural guidance and outcome evaluation during sclerotherapy of low-flow orbital vascular malformations. MATERIALS AND METHODS: A retrospective review was performed of 17 consecutive patients with low-flow orbital malformations (14 lymphatic, two venous, and one venolymphatic) who underwent multimodality image-guided sclerotherapy between November 2012 and May 2015. Sclerotherapy technique, image guidance tools, and complications were recorded. Sclerotherapy outcome was evaluated using clinical response, magnetic resonance (MR) image-based lesion volumetry, and proptosis quantification. RESULTS: There were 22 sclerotherapy sessions performed. Intraprocedural ultrasound (US), fluoroscopy, cone-beam computed tomography (CT) and MR image fusion were used for image guidance with 100% technical success. Resolution of presenting symptoms was observed in all patients at 1-month follow-up. Four major sclerotherapy complications were successfully managed. Statistically significant reduction in lesion volume (P = .001) and proptosis (P = .0117) by MR image analysis was achieved in all patients in whom 3-month follow-up MR imaging was available (n = 13/17). There was no lesion recurrence at a median follow-up of 18 months (range, 8-38 mo). CONCLUSIONS: Multimodality imaging tools, including US, fluoroscopy, cone-beam CT, and MR fusion, during sclerotherapy of low-flow orbital malformations provide intraprocedural guidance and quantitative image-based evaluation of treatment outcome.

Outcomes after peritoneal dialysis catheter placement.

BACKGROUND: The purpose of this study was to review surgical outcomes after elective placement of peritoneal dialysis (PD) catheters in children with end-stage renal disease. METHODS: Children with PD catheters placed between February 2002 and July 2014 were retrospectively reviewed. Outcomes were catheter life, late (>30days post-op) complications (catheter malfunction, catheter malposition, infection), and re-operation rates. Comparison groups included laparoscopic versus open placement, age<2, and weight<10kg. Univariate and multivariate analysis were performed. RESULTS: One hundred sixteen patients had 173 catheters placed (122 open, 51 laparoscopic) with an average patient age of 9.7±6.3years. Mean catheter life was similar in the laparoscopic and open groups (581±539days versus 574±487days, p=0.938). The late complication rate was higher for open procedures (57% versus 37%, p=0.013). Children age<2 or weight<10kg had higher re-operation rates (64% versus 42%, p=0.014 and 73% versus 40%, p=0.001, respectively). Adjusted for age and weight, open technique remained a risk factor for late complications (OR 2.44, 95% CI 1.20-4.95) but not re-operation. DISCUSSION: Laparoscopic placement appears to reduce the rate of late complications in children who require PD dialysis catheters. Children <2years age or <10kg remain at risk for complications regardless of technique.

Transaxillary Subcutaneouscopic Excision of Fibroadenoma of the Breast in Children: The Covert Scar Approach.

INTRODUCTION: Fibroadenoma is a common condition in adolescent girls. Most tumors are excised either through a periareolar approach or the inframammary approach. Both approaches produce visible scars in adolescent girls. We propose a new cosmetic approach to this lesion and report our experience with the transaxillary subcutaneouscopic approach for excision of the fibroadenoma of the breast. The purpose of this case report is to delineate an innovative surgical approach to resection of a breast fibroadenoma that yields an adequate resection without possible damage to the ductal system while optimizing cosmetic results by avoiding scars. MATERIALS AND METHODS: We retrospectively reviewed the medical records of four adolescent girls who underwent the above approach for excision of fibroadenoma of the breast. The age range was 14-16 years. RESULTS: There were no complications in all 4 patients. The final result at the 3-month follow-up revealed an esthetically pleasing skin incision that healed well and was hidden by the natural skin fold of the axilla. CONCLUSIONS: Transaxillary subcutaneouscopic excision of fibroadenoma of the breast in children is a safe and effective technique and should be considered for excision of benign breast lesions in children.

Routine gastrostomy tube placement in children: Does preoperative screening upper gastrointestinal contrast study alter the operative plan?

BACKGROUND: Upper GI (UGI) studies are routinely ordered to screen for malrotation before routine placement of gastrostomy (G) tubes. However, the usefulness of this study is unknown. METHODS: A retrospective review of children with surgically placed G-tubes over a 2 year period (2011-2013) was performed. Patients with concomitant fundoplications were excluded. RESULTS: Three hundred ninety-three patients underwent G-tube placement. Of these, 299 patients (76%) had preoperative UGI, and 11 patients (3.7%) were identified with malrotation on UGI. Five (1.7%) patients underwent a Ladd's procedure. The remaining 6 either had malrotation associated with gastroschisis (n=5) or were lost to follow-up (n=1). Children <1 year did not have different rates of malrotation compared to older children (4.3% vs. 3.2%, p=0.617). Likewise, children with neurologic impairment (NI) had similar rates of malrotation compared to neurologically normal (NN) children (2.6% vs. 3.8%, p=0.692). The only significant difference in malrotation rate was between those with congenital gastrointestinal anomalies (24%) and those without (1.5%) (p<0.001). CONCLUSION: Preoperative screening UGI before routine G-tube placement led to an unexpected diagnosis of malrotation in only 1.7%. Given the added radiation risk associated with an UGI, our data suggest that an UGI is unnecessary prior to routine G-tube placement. A larger prospective study is warranted to validate these results.

Laparoscopic gastroscopic transgastric cystogastrostomy and cholecystectomy for pseudopancreatic cyst after gallstone pancreatitis in children.

A 15-year-old girl presented with gallstone pancreatitis. Subsequently, a pseudopancreatic cyst developed that was diagnosed on computed tomographic scan. She underwent a laparoscopic and gastroscopic transgastric cystogastrostomy. In the following report, we describe our novel approach and technique for the above condition.

"The umbilical fat sign": an important and consistent landmark during single port/incision laparoscopic surgery and standard laparoscopy.

INTRODUCTION: During single port laparoscopic surgery (SPLS), access is obtained through the umbilicus and the scar is hidden within the scar of the umbilicus for providing good cosmesis. It is essential that the incision be well planned so as to get the maximum exposure through the umbilical incision. The umbilical fat sign is an important landmark to achieve this. PURPOSE: The aim of this study is to retrospectively review importance of the umbilical fat sign as a landmark for peritoneal access during SPLS in children. METHOD: A retrospective chart review of 57 children (33 males and 24 females) who underwent single port access surgery at Texas Children's Hospital from April 2009 to December 2010 was conducted. The median age of the patients was 10.8 years, ranging from 4 to 17 years. TECHNIQUE: The limits of the umbilicus were marked using a marking pen. A vertical incision is made through the center of the umbilical scar. It is of vital importance to maintain the incision in the exact center of the scar tissue. Skiving away from the center makes the entry in the peritoneal cavity harder and prolongs peritoneal access time. During all the single port cases, we have done so far we have noted that if we are in the center of the scar then we always see a blob of fat ("umbilical fat" sign) in the center. If we use a probe or grooved director through this fat direct access is obtained in the peritoneal cavity. Incision can then be extended on both sides and be kept to the limits of the umbilical ring. Peritoneal access can be obtained in 1 to 2 minutes using this approach. Umbilical reconstruction is the best performed with this approach. RESULTS: One child developed signs of wound infection and was treated successfully with antibiotics for 5 days. Scars healed well in all cases with no wound dehiscence. No umbilical scars were visible at follow-up (3 to 4 weeks postoperatively). CONCLUSION: Umbilical fat sign is an important landmark for surgeons during SPLS for direct and quick peritoneal access and better reconstruction of the umbilicus.

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.

Immediate laparoscopic repair of a traumatic abdominal wall hernia in a 2-year-old child.

Traumatic abdominal wall hernia in children with intact skin is rare. This type of hernia is caused by blunt trauma abdomen that does not penetrate the skin. These hernias have been repaired in adults by open repair. We report the first laparoscopic repair of this condition in a 2-year-old child with blunt abdominal trauma in emergent settings.