From the Cecum to the Sigmoid: Twisted Colon in the Pediatric Population.

Colonic volvulus (CV) is the third leading cause of colonic obstruction in adults. In infants and children, this is exceedingly rare, with only sporadic cases reported so far. We present two cases of CV to highlight the differences in etiology, presentation, diagnosis, and treatment of this condition. The first patient is a 12-year-old boy with no previous surgeries who presented with four days of abdominal pain. Imaging showed a sigmoid volvulus that was decompressed endoscopically, and he was discharged. He had a contrast enema showing an abnormal rectosigmoid ratio. At the time of the rectal biopsy four weeks later, he was found to have a recurrence, at which point definitive operative treatment was pursued. The second patient is a 17-year-old boy who presented with five days of abdominal pain and CT findings concerning for ischemic volvulus. This prompted emergent operative intervention, where a cecal volvulus was discovered as the result of a congenital band. The band was divided without complication.  Pediatric CV is a rare condition that might be severe in some cases. High suspicion, prompt diagnosis, and treatment are essential to prevent early and long-term morbidity.

Composite Anterolateral Thigh and Fascia Lata Free Flap for Abdominal and Groin Reconstruction in a Pediatric Patient.

Large abdominal wall and groin defects present complex reconstructive challenges. These defects typically require free flap reconstruction to bring in healthy vascularized tissue and recreate the complex full-thickness defect. A 6-year-old previously healthy girl presented to our trauma center after sustaining a close-range shotgun injury resulting in a full-thickness defect to the inferior hemi-abdomen and groin. A composite anterolateral thigh flap with fascia lata free flap was performed to reconstruct the myofascial, skin, and subcutaneous tissue of the abdomen and groin. We present the first composite anterolateral thigh flap with fascia lata for full-thickness abdominal wall and groin reconstruction in a pediatric patient.

Impact of helmet laws on motorcycle crash mortality rates.

BACKGROUND: Helmets are effective in reducing traumatic brain injury. However, population effects of helmet laws have not been well described. This study assesses the impact of helmet laws on the motorcycle (MC) fatality rate in the United States from 1999 to 2015. METHODS: Fatality Analysis Reporting System MC fatalities (aged ≥16 years), crash characteristics, and MC-related laws were collected by year for all 50 states from 1999 to 2015 to create a pooled time series. Generalized linear autoregressive modeling was applied to assess the relative contribution of helmet laws to the MC fatality rate while controlling for other major driver laws and crash characteristics. RESULTS: Universal helmet laws were associated with a 36% to 45% decline in the motorcycle crash mortality rate during the study period across all age cohorts (unstandardized regression coefficients are reported): 16 to 20 years, B = -0.45 (p < 0.05); 21 to 55 years, B = -0.42 (p < 0.001); 56 to 65 years, B = -0.38 (p < 0.04); and older than 65 years, B = -0.36 (p < 0.02). Partial helmet laws were associated with a 1% to 81% increase in the fatality rate compared with states with no helmet laws and a 22% to 45% increase compared with universal laws. Helmet usage did not attenuate the countervailing effect of weaker partial laws for 16 to 20 years (B = 0.01 [p < 0.001]). Other laws associated with a declining motorcycle crash mortality rate included the following: social host/overservice laws, 21 to 55 years (B = -0.38 [p < 0.001]); 56 to 65 years (B = -0.16 [p < 0.002]), and older than 65 years (B = -0.12 [p < 0.003]); laws reducing allowable blood alcohol content, 21 to 55 years (B = -4.9 [p < 0.02]); and laws limiting passengers for new drivers 16 to 20 years (B = -0.06 [p < 0.01]). CONCLUSION: During the period of the study, universal helmet laws were associated with a declining mortality rate, while partial helmet laws were associated with an increasing mortality rate. Other state driver laws were also associated with a declining rate. In addition to universal helmet laws, advocating for strict alcohol control legislation and reevaluation of licenses in older riders could also result in significant reduction in MC-related mortality. LEVEL OF EVIDENCE: Prognostic and epidemiological, level III.

Gastrointestinal disorders in Curry-Jones syndrome: Clinical and molecular insights from an affected newborn.

Curry-Jones syndrome (CJS) is a pattern of malformation that includes craniosynostosis, pre-axial polysyndactyly, agenesis of the corpus callosum, cutaneous and gastrointestinal abnormalities. A recurrent, mosaic mutation of SMO (c.1234 C>T; p.Leu412Phe) causes CJS. This report describes the gastrointestinal and surgical findings in a baby with CJS who presented with abdominal obstruction and reviews the spectrum of gastrointestinal malformations in this rare disorder. A 41-week, 4,165 g, female presented with craniosynostosis, pre-axial polysyndactyly, and cutaneous findings consistent with a clinical diagnosis of CJS. The infant developed abdominal distension beginning on the second day of life. Surgical exploration revealed an intestinal malrotation for which she underwent a Ladd procedure. Multiple small nodules were found on the surface of the small and large bowel in addition to an apparent intestinal duplication that seemed to originate posterior to the pancreas. Histopathology of serosal nodules revealed bundles of smooth muscle with associated ganglion cells. Molecular analysis demonstrated the SMO c.1234 C>T mutation in varying amounts in affected skin (up to 35%) and intestinal hamartoma (26%). Gastrointestinal features including structural malformations, motility disorders, and upper GI bleeding are major causes of morbidity in CJS. Smooth muscle hamartomas are a recognized feature of children with CJS typically presenting with abdominal obstruction requiring surgical intervention. A somatic mutation in SMO likely accounts for the structural malformations and predisposition to form bowel hamartomas and myofibromas. The mutation burden in the involved tissues likely accounts for the variable manifestations.

Ketorolac does not reduce effectiveness of pleurodesis in pediatric patients with spontaneous pneumothorax.

PURPOSE: Antiinflammatory medications are thought to reduce the effectiveness of pleurodesis performed for the treatment of spontaneous pneumothorax. We reviewed our experience with children undergoing video-assisted thorascopic surgery (VATS) with pleurodesis for pneumothorax to determine if ketorolac administration influences patient outcomes. METHODS: A retrospective review of patients who underwent VATS pleurodesis for spontaneous pneumothorax from 2009 to 2013 at a pediatric hospital was performed. Length of stay, radiographic pneumothorax resolution prior to discharge, and ipsilateral recurrence rates were compared in patients who did and did not receive perioperative ketorolac. RESULTS: Over a 50-month period, 51 patients underwent VATS with mechanical pleurodesis for spontaneous pneumothorax. The average age was 15.5years, and 76% were male. Ketorolac was administered to 26/51 patients. There were no differences in average length of stay (11.3 vs 10.9days, p=0.36), incidence of residual pneumothorax at discharge (22/41 vs 19/41, p=0.48), or ipsilateral recurrence (5/10 vs 5/10, p=1). CONCLUSIONS: Despite the intrinsic antiinflammatory properties of ketorolac, our data suggests that its use for patients undergoing pleurodesis for spontaneous pneumothorax does not detrimentally influence the outcomes of surgery. Therefore, we conclude that ketorolac can be used for pain control in this population. Large-scale studies are warranted to validate these findings.

Breast Imaging-Reporting and Data System (BI-RADS) classification in 51 excised palpable pediatric breast masses.

INTRODUCTION: The American College of Radiology Breast Imaging Reporting and Data System (BI-RADS) classification was developed to risk stratify breast lesions and guide surgical management based on imaging. Previous studies validating BI-RADS for US do not include pediatric patients. Most pediatric breast masses present as palpable lesions and frequently undergo ultrasound, which is often accompanied with a BI-RADS classification. Our study aimed to correlate BI-RADS with pathology findings to assess applicability of the classification system to pediatric patients. METHODS: We performed a retrospective review of all patients who underwent excision of a breast mass at a single center from July 2010 to November 2013. We identified all patients who underwent preoperative ultrasound with BI-RADS classification. Demographic data, imaging results, and surgical pathology were analyzed and correlated. RESULTS: A total of 119 palpable masses were excised from 105 pediatric patients during the study period. Of 119 masses, 81 had preoperative ultrasound, and BI-RADS categories were given to 51 masses. Of these 51, all patients were female and the average age was 15.9 years. BI-RADS 4 was given to 25 of 51 masses (49%), and 100% of these lesions had benign pathology, the most common being fibroadenoma. CONCLUSIONS: Treatment algorithm based on BI-RADS classification may not be valid in pediatric patients. In this study, all patients with a BI-RADS 4 lesion had benign pathology. BI-RADS classification may overstate the risk of malignancy or need for biopsy in this population. Further validation of BI-RADS classification with large scale studies is needed in pediatric and adolescent patients.

Culture-independent evaluation of the appendix and rectum microbiomes in children with and without appendicitis.

PURPOSE: The function of the appendix is largely unknown, but its microbiota likely contributes to function. Alterations in microbiota may contribute to appendicitis, but conventional culture studies have not yielded conclusive information. We conducted a pilot, culture-independent 16S rRNA-based microbiota study of paired appendix and rectal samples. METHODS: We collected appendix and rectal swabs from 21 children undergoing appendectomy, six with normal appendices and fifteen with appendicitis (nine perforated). After DNA extraction, we amplified and sequenced 16S rRNA genes and analyzed sequences using CLoVR. We identified organisms differing in relative abundance using ANOVA (p<0.05) by location (appendix vs. rectum), disease (appendicitis vs. normal), and disease severity (perforated vs. non-perforated). RESULTS: We identified 290 taxa in the study's samples. Three taxa were significantly increased in normal appendices vs. normal rectal samples: Fusibacter (p = 0.009), Selenomonas (p = 0.026), and Peptostreptococcus (p = 0.049). Five taxa were increased in abundance in normal vs. diseased appendices: Paenibacillaceae (p = 0.005), Acidobacteriaceae GP4 (p = 0.019), Pseudonocardinae (p = 0.019), Bergeyella (p = 0.019) and Rhizobium (p = 0.045). Twelve taxa were increased in the appendices of appendicitis patients vs. normal appendix: Peptostreptococcus (p = 0.0003), Bilophila (p = 0.0004), Bulleidia (p = 0.012), Fusobacterium (p = 0.018), Parvimonas (p = 0.003), Mogibacterium (p = 0.012), Aminobacterium (p = 0.019), Proteus (p = 0.028), Actinomycineae (p = 0.028), Anaerovorax (p = 0.041), Anaerofilum (p = 0.045), Porphyromonas (p = 0.010). Five taxa were increased in appendices in patients with perforated vs. nonperforated appendicitis: Bulleidia (p = 0.004), Fusibacter (p = 0.005), Prevotella (p = 0.021), Porphyromonas (p = 0.030), Dialister (p = 0.035). Three taxa were increased in rectum samples of patients with appendicitis compared to the normal patients: Bulleidia (p = 0.034), Dialister (p = 0.003), and Porphyromonas (p = 0.026). CONCLUSION: Specific taxa are more abundant in normal appendices compared to the rectum, suggesting that a distinctive appendix microbiota exists. Taxa with altered abundance in diseased and severely diseased (perforated) samples may contribute to appendicitis pathogenesis, and may provide microbial signatures in the rectum useful for guiding both treatment and diagnosis of appendicitis.

On mixed reality environments for minimally invasive therapy guidance: systems architecture, successes and challenges in their implementation from laboratory to clinic.

Mixed reality environments for medical applications have been explored and developed over the past three decades in an effort to enhance the clinician's view of anatomy and facilitate the performance of minimally invasive procedures. These environments must faithfully represent the real surgical field and require seamless integration of pre- and intra-operative imaging, surgical instrument tracking, and display technology into a common framework centered around and registered to the patient. However, in spite of their reported benefits, few mixed reality environments have been successfully translated into clinical use. Several challenges that contribute to the difficulty in integrating such environments into clinical practice are presented here and discussed in terms of both technical and clinical limitations. This article should raise awareness among both developers and end-users toward facilitating a greater application of such environments in the surgical practice of the future.

Pediatric gastroesophageal reflux disease.

This article reviews the mechanisms responsible for gastroesophageal reflux disease (GERD), available techniques for diagnosis, and current medical management. In addition, it extensively discusses the surgical treatment of GERD, emphasizing the use of minimally invasive techniques.

Pediatric malignancies: neuroblastoma, Wilm's tumor, hepatoblastoma, rhabdomyosarcoma, and sacroccygeal teratoma.

Common pediatric malignancies are reviewed: neuroblastoma, Wilms tumor, hepatoblastoma, rhabdomyosarcoma, and sacrococcygeal teratoma. Elements of presentation, diagnosis, staging, treatment, and longterm prognosis are discussed, with particular attention to surgical management.

Video. Diagnostic laparoscopy and preoperative planning in ischiopagus tripus conjoined twins: a surgical first, with detailed demonstration of the complex anatomical relationships.

BACKGROUND: Diagnosis and management of conjoined twins are constantly evolving. New imaging techniques provide important anatomic details and help in planning the separation procedure. Despite these technological advances, however, the complex arrangement of conjoined organs is somewhat difficult to interpret, leaving unresolved questions at time of surgery. The authors present a video demonstrating laparoscopy as an adjunct in the preoperative planning of separation of ischiopagus tripus conjoined twins as well as illustrating the complex anatomy in a combination of video, drawings, and radiologic imaging. To date, this is the first time that laparoscopy has been used in preoperative assessment of conjoined twins. METHODS: After elective cesarean delivery, ischiopagus tripus conjoined twins were thoroughly evaluated with conventional imaging, including plain radiographs and computed tomography scan with three-dimensional (3-D) reconstruction images. The anatomy of the gastrointestinal and genitourinary tracts was further defined with barium enema, retrograde pyelography, and cystoscopy. In addition to these tests, diagnostic laparoscopy was performed at time of tissue expander placement. An angled scope, introduced through a 5-mm umbilical port, was used to visualize the intraperitoneal organs as well as all accessible retroperitoneal structures. RESULTS: Laparoscopy provided useful information regarding the bowel distribution between the twins. In addition, it helped demonstrate the relationship of shared solid organs with other intra-abdominal structures and identify anatomic landmarks used in the subsequent separation of the twins. Finally, laparoscopy helped confirm the presence, number, and morphology of the internal female genitalia. CONCLUSIONS: Diagnostic laparoscopy is a useful tool in evaluation of ischiopagus tripus conjoined twins. It is an important adjunct to preoperative studies in preparing for an expeditious and safe separation procedure.