Left Ureterocalycostomy With Ileal Interposition for Retroperitoneal Fibrosis in Patient With Erdheim-Chester Disease.

BACKGROUND: Erdheim-Chester disease (ECD) is a rare progressive non-Langerhans' cell histiocytic multisystem disorder with a broad spectrum of clinical manifestations, including infiltrative perinephric with ureteral involvement resulting in hydronephrosis, renal atrophy, and eventual renal failure. OBJECTIVE: To present a patient with ECD with bilateral renal/ureteral involvement managed with bilateral percutaneous nephrostomy tubes (PCNT) and trametinib who underwent bilateral robotic upper tract reconstruction, the first such published report. The video demonstrates only the left-sided repair, which posed specific challenges and demonstrates reconstructive techniques useful in complex upper tract repairs with limited tissue availability. MATERIALS AND METHODS: A 35-year-old male initially presented with baseline creatinine of 1.62 and split renal function; 30% right and 70% left by Lasix renogram. Extra-genitourinary manifestations of disease included cardiac hypertrophy and skin ulcers/lesions. Bilateral retrograde pyeloureterography showed proximal ureteral obliteration ∼4 cm bilaterally. Multiple management options were discussed including PCNTs, but patient elected for definitive repair. He was seen by Cardiology and Anesthesia and deemed to be optimized. He held his trametinib for 1week before surgery. We demonstrate a difficult ureteral dissection with fibrotic hilum preventing separation. Simultaneous ureteroscopy identified the distal extent of stricture which was excised, leaving a ∼15 cm gap. Downward nephropexy was performed with ultrasound guidance to identify an inferior calyx. Partial nephrectomy was then performed without vascular control due to hilar fibrosis. Ileal interposition was chosen to bridge the remaining ∼8 cm gap. Proximal ileo-calyceal and distal ileo-ureteral anastomoses were performed. We then placed a 30 cm × 7 Fr double-J ureteral stent in standard fashion. The ileum was secured to the renal pelvis to maintain a straight lie and an omental flap was secured in place. RESULTS: Immediate postoperative course was complicated by partial small bowel obstruction leading to a negative exploratory laparotomy and a subsequent episode of urosepsis. The patient is now voiding well without stents or PCNTs, without infections and with improving renal function, now with GFR (glomerular filtration rate) of 62 from 43 preoperatively. With aggressive hydration, patient has had no obstruction of the distal ureter with mucus. MRI Abdomen/Pelvis 6months later showed irregularity of the calyces with stable mild hydronephrosis. The patient continues to be medically managed on trametinib for his underlying disease, with surveillance for recurrent fibrosis and obstruction which has not yet occurred. CONCLUSION: Robotic ureterolysis and ureterocalycostomy with possible bowel interposition is a reasonable option for upper tract reconstruction in select patients with ECD.

The Promise of Neoadjuvant and Adjuvant Therapies for Renal Cancer.

Because metachronous metastatic disease will develop in 20% to 40% of patients with presumed localized renal cell carcinoma (RCC) treated surgically, research is focused on neoadjuvant and adjuvant systemic therapy, to improve disease-free and overall survival. Neoadjuvant therapies trialed include anti-vascular endothelial growth factor (VEGF) tyrosine kinase inhibitor (TKI) agents, or combination therapies (immunotherapy with TKI), and aim to improve resectability of locoregional RCC. Adjuvant therapies trialed include cytokines, anti-VEGF TKI agents, or immunotherapy. These therapeutics can facilitate the surgical extirpation of the primary kidney tumor in the neoadjuvant setting and improve disease-free survival in the adjuvant setting.

Integrating Surgery in the Multidisciplinary Care of Advanced Renal Cell Carcinoma.

The role of surgery for patients with locally advanced and metastatic renal cell carcinoma (RCC) is not precisely defined in our contemporary era of systemic therapies. Research in this field is focused on the role of regional lymphadenectomy, along with indications and timing of cytoreductive nephrectomy and metastasectomy. As our understanding of the molecular and immunological basis of RCC continues to develop along with the advent of novel systemic therapies, prospective clinical trials will be critical in defining how surgery should be integrated into the treatment paradigm of advanced RCC.

Local and Regional Recurrences of Clinically Localized Renal Cell Carcinoma after Nephrectomy: A 15 Year Institutional Experience with Prognostic Features and Oncologic Outcomes.

OBJECTIVE: To evaluate outcomes for patients with local recurrence (LR) of clinically localized renal cell carcinoma (RCC) without concurrent systemic metastasis from our institution, an event that occurs rarely (1%-3%) after surgery. LR may be a harbinger of poor outcomes, and the best management of these patients is unclear. MATERIALS/METHODS: We retrospectively reviewed patients surgically treated for clinically localized RCC (cT1-2N0M0) with subsequent LR (in the partial or radical nephrectomy bed) and/or regional recurrence (RR; in the abdomen distant from the direct site of surgery) without concurrent metastasis from our institutional database (2004-2018). Comparative and survival analyses were performed. RESULTS: Out of 3038 total patients, 1895 had clinically localized RCC, with 30 patients (1.6%) having isolated LR/RR. Median time to recurrence was 26.5 months (IQR:16-35). Of 26 patients treated with local therapy, 14 (53.8%) recurred over a median follow-up time of 29.5 months (IQR:12-45). The 1-year and 2-year secondary recurrence-free survival rates are 60.7% and 49.7%, respectively. Two or more sites of locoregional recurrence significantly predicted secondary recurrence/metastasis after local therapy for local recurrence (hazard ratio: 2.22, P= .04). CONCLUSION: Our results suggest local therapy is appropriate for select patients with LR/RR, with almost 50% of patients undergoing a second local therapy remaining alive with "local cure" and no secondary recurrence. The number of sites of recurrence can be used to better select patients that will benefit from local therapy or systemic/combination therapy. This work provides a framework onto which further studies regarding local therapy and locoregional recurrence of RCC can be performed.

Inaccuracies in News Media Reporting About the 2019 US Food and Drug Administration Ban on Transvaginal Mesh for Pelvic Organ Prolapse Repair.

OBJECTIVE: To analyze inaccuracies in the news media reporting of the 2019 US Food and Drug Administration (FDA) ban on surgical mesh for transvaginal repair of pelvic organ prolapse (POP). METHODS: We queried the NexisUni media database for English-language news articles about "transvaginal mesh" or "FDA" published before and after the ban. Content analysis was based on discussion of the ban, indications for surgical mesh, and assessment of bias. We characterized public interest in transvaginal mesh using Google Trends. RESULTS: Of 290 news articles reviewed, 42 articles were included for analysis. Public interest in transvaginal mesh increased 4-fold after the FDA announcement. While 15 articles (38%) accurately reported that mesh was used in both POP and incontinence repairs, a plurality (18, 43%) only described using mesh for POP. The majority (30, 71%) of articles did not specify that the FDA ban applied to only transvaginal repair of POP and not to incontinence. Despite multiple professional societies affirming the use of mesh for incontinence, only 2 (5%) articles cited these evidence-based recommendations. About half of the articles had an overtly biased tone; articles with an anti-mesh bias were significantly less likely to identify the mesh indications relevant to the ban (P <.01). CONCLUSION: Seventy percent of news reports about the 2019 FDA ban on transvaginal mesh for POP failed to distinguish between the clinical indications for mesh impacted by the ban. The findings raise concern about patient perceptions of and future access to mesh surgery, regardless of indication.

Active Surveillance for Small Renal Masses.

Active surveillance (AS) is a safe and reasonable management strategy for many patients with small renal masses (SRM) suspicious for a clinical T1a renal cell carcinoma based on excellent metastasis-free and cancer-specific survival. However, the expansion of robotic extirpation of SRM has outpaced the adoption of AS, resulting in the possibility of overtreatment for select patients with SRM, especially the elderly and comorbid. In this review of AS for SRM, with a focus on the Delayed Intervention and Surveillance for Small Renal Masses (DISSRM) Registry, we detail the rationale behind AS, review lessons learned from the past decades of literature, and offer suggestions for appropriate patient selection and follow-up. An improved understanding of the data supporting AS will empower physicians and patients to more comfortably pursue AS to avoid over-treatment and provide individualized care to patients with SRM.

Phosphodiesterase type 5 inhibitor therapy provides sustained relief of symptoms among patients with chronic pelvic pain syndrome.

BACKGROUND: Chronic prostatitis/chronic pelvic pain syndrome type III (CP/CPPS) is associated with pelvic pain, sexual dysfunction and irritative voiding. Sustained symptom relief has proven difficult with alpha blockers, antibiotics, and nonsteroidal anti-inflammatory drugs (NSAIDs). Phosphodiesterase type 5 (PDE5) inhibitors (PDE5is) have the potential to alleviate bladder urgency, relax the pelvic floor, and correct underlying erectile dysfunction; however, few studies have investigated the application of PDE5i's to CP/CPPS. The purpose of this study was to assess the effect of long-term PDE5i therapy on symptoms among patients with diagnosed CP/CPPS. METHODS: A group of patients older than 18 years diagnosed with CP/CPPS presenting from 2009 to 2018 were followed prospectively while they were being prescribed off-label PDE5i therapy for symptoms. National Institute of Health chronic prostatitis symptom index (CPSI) scores before PDE5i therapy initiation and after at least 3 months were utilized to assess impact on symptoms. RESULTS: A total of 25 patients (mean age 44.4±12.9 years) met study criteria. The mean duration of PDE5i therapy was 1.3±1.6 years. Continued use of daily PDE5is was associated with significant decreases in total CPSI, pain, urinary symptom and quality of life scores [total CPSI: -12.8, standard deviation (SD) 9.5; pain: -6.1, SD 4.1; urinary symptoms: -2.4, SD 2.1; quality of life: -4.5, SD 3.9; P<0.001]. CONCLUSIONS: This prospective data suggests that PDE5i therapy is associated with durable decreases in CP/CPPS symptoms past 3 months.

Primary and post-chemotherapy robotic retroperitoneal lymph node dissection for testicular cancer: a review.

Open retroperitoneal lymph node dissection (RPLND) is the gold standard for surgical management of the retroperitoneum in patients with testicular cancer, and is associated with excellent oncologic outcomes and significant morbidity including length of stay. Minimally invasive RPLND, starting with laparoscopic retroperitoneal lymph node dissection in 1992 and now robotic retroperitoneal lymph node dissection in 2006, endeavor to decrease the morbidity of open RPLND while maintaining excellent oncologic outcomes. This review surveys the literature regarding both primary and post-chemotherapy robotic RPLND, emphasizing that while early outcomes are promising, much work needs to be done before widespread use of this technique is implemented.

Speaking in Tongues: What Parents Really Think About Tongue-Tie Surgery for Their Infants.

We evaluate maternal perspectives of frenotomy for ankyloglossia in newborns. We searched highly frequented forums like babycenter.com for comments related to frenotomy posted from 2012 to 2017. We applied modified grounded theory, reviewed posts for thematic synthesis, and calculated frequencies for each theme. High decisional conflict, low discussion of surgical complications, and an overall moderate positive experience were noted. Mothers posted about breastfeeding issues (n = 227, 74.7%) and overall experience (n = 229, 75.3%). Posts related to breastfeeding include discomfort ("I can no longer physically take the pain"; n = 93, 30.6%). Parents posted frequently about a satisfactory outcome ("It was so worth it to improve our nursing relationship"; n = 133, 43.8%), but dissatisfactory outcome ("I'm still having problems latching and am pumping. It's brutal"; n = 31) was present in 10.2%. These results aid in understanding parental thoughts of frenotomy. Physicians can use these self-reported maternal perspectives on frenotomy to guide counseling and improve shared decision making for parents.

Anatomic Distribution of the Morphologic Variation of the Upper Lip Frenulum Among Healthy Newborns.

IMPORTANCE: The maxillary labial frenulum and its potential contribution to breastfeeding difficulty may substantially affect public health. However, objective studies of the frenulum are limited. OBJECTIVE: To measure the variations in length, thickness, and attachments of the maxillary labial frenulum in healthy newborns and to identify which anatomic measurements could be used in further research investigating the maxillary labial frenulum. DESIGN, SETTING, AND PARTICIPANTS: This prospective cross-sectional study conducted measurements on images of maxillary labial frenula captured by digital photography from 150 healthy newborns admitted to the newborn nursery at a tertiary care children's hospital in Maryland between September 1, 2017, and April 1, 2018. MAIN OUTCOMES AND MEASURES: The primary outcome was the measurement of numerous frenulum morphologic components. RESULTS: Of 150 newborns enrolled, 77 were female, the mean (SD) gestational age was 38.60 (1.72) weeks, and the mean (SD) birth weight was 3180 (570) g. The means and SDs of the morphologic components of the frenulum with the broadest distributions, which were most helpful in differentiating degrees of lip tethering, included the following: alveolar edge to frenulum gingival attachment, 1.53 (0.85) mm; frenulum length on stretch, 5.19 (1.68) mm; frenulum gingival attachment thickness, 0.84 (0.36) mm; frenulum labial attachment thickness, 2.83 (1.33) mm; and the percentage of free lip to total lip length, 87.38% (7.67%). Gingival attachment mean (SD) thickness differed between late-preterm (0.69 [0.24] mm) and term (0.88 [0.37] mm) infants (Cohen d, -0.52; 95% CI, -0.94 to -0.10). CONCLUSIONS AND RELEVANCE: To our knowledge, this cross-sectional study was the first to objectively measure the numerous morphologic components of the upper lip anatomy in healthy newborns. Variations in maxillary labial frenulum morphology were identified, and some combination of the stated measurements may be used to create a more robust classification system to advance quality research in the association of lip-tie with breastfeeding difficulty.

CRISPR/Cas9 Mutagenesis Reveals Versatile Roles of Hox Genes in Crustacean Limb Specification and Evolution.

Crustaceans possess a diverse array of specialized limbs. Although shifts in Hox gene expression domains have been postulated to play a role in generating this limb diversity, little functional data have been provided to understand the precise roles of Hox genes during crustacean development. We used a combination of CRISPR/Cas9-targeted mutagenesis and RNAi knockdown to decipher the function of the six Hox genes expressed in the developing mouth and trunk of the amphipod Parhyale hawaiensis. These experimentally manipulated animals display specific and striking homeotic transformations. We found that abdominal-A (abd-A) and Abdominal-B (Abd-B) are required for proper posterior patterning, with knockout of Abd-B resulting in an animal with thoracic type legs along what would have been an abdomen, and abd-A disruption generating a simplified body plan characterized by a loss of specialization in both abdominal and thoracic appendages. In the thorax, Ubx is necessary for gill development and for repression of gnathal fate, and Antp dictates claw morphology. In the mouth, Scr and Antp confer the part-gnathal, part-thoracic hybrid identity of the maxilliped, and Scr and Dfd prevent antennal identity in posterior head segments. Our results allow us to define the role Hox genes play in specifying each appendage type in Parhyale, including the modular nature by which some appendages are patterned by Hox gene inputs. In addition, we define how changes in Hox gene expression have generated morphological differences between crustacean species. Finally, we also highlight the utility of CRISPR/Cas9-based somatic mutagenesis in emerging model organisms.