A Rare Case of an Inguinal Hernia-Containing (Extraperitoneal) Ureter.

BACKGROUND Ureteroinguinal hernias are exceptionally rare and are seldom diagnosed in the preoperative setting. There are 2 classifications of this type of hernia: paraperitoneal and extraperitoneal. CASE REPORT We report a case of a 67-year-old man who presented with urinary symptoms and a reducible right inguinal hernia. A computed tomography (CT) scan of the abdomen and pelvis suggested an ureteroinguinal hernia. Further diagnostics and treatment via cystoscopy, retrograde pyelogram, and right ureteral stent placement were performed, confirming the diagnosis and providing relief of the obstructive uropathy. The patient underwent an attempted elective transabdominal preperitoneal repair that was converted to an open Lichtenstein repair. Intraoperatively, an extraperitoneal ureteroinguinal hernia was identified. The patient did well postoperatively, and the stent was removed 1 month later. CONCLUSIONS Only 20% of the ureteroinguinal hernias described in the literature are extraperitoneal. In our case presentation, we demonstrated successful identification and treatment of an extraperitoneal ureteroinguinal hernia. The diagnosis was made using a combination of the clinical presentation, CT of the abdomen and pelvis, and cystoscopy with retrograde pyelogram. The extraperitoneal classification was an intraoperative diagnosis. The treatment consisted of a temporizing ureter stent and definitive management with an open Lichtenstein repair. We recommend obtaining a CT scan when a patient presents with a combination of urinary symptoms and an inguinal hernia because this process was invaluable in our preoperative diagnosis. Stent placement at the time of diagnosis permitted an elective repair and aided in the identification of the ureter during the hernia repair.

Surgical management of rare benign tumors of the sternum.

Primary benign tumors of the sternum are an exceedingly rare entity. Surgical techniques regarding intervention for these lesions are not clearly defined in the literature given their scarcity. Operative techniques include en-bloc resection of the tumor, and this has proven to be successful in preventing local recurrence despite benign nature of the lesion. Given the often extensive defect created by the excision, reconstruction is frequently necessary; depending on the size of the defect, either autologous bone grafting or the use of synthetic materials may be indicated. This study serves to present two cases of rare primary benign tumors of the sternum, giant cell tumors and osteoma spongiosum and to summarize the available literature. We present a review of the literature of 17sternal giant cell tumor cases reported so far including our patient and unique case of osteoma spongiosum of the sternum, that discusses their surgical management, as well as reconstructive techniques that provided an excellent clinical result and a lack of recurrence on long term follow-up.

Currarino-Silverman syndrome: diagnosis and treatment of rare chest wall deformity, a case series.

BACKGROUND: Currarino-Silverman (CS) syndrome is an extremely rare congenital deformity of the anterior chest wall. The syndrome is often combined with congenital heart defects and spinal abnormalities. As of currently, there is a lack of definite description in the literature about this type of pectus deformity. Typically, patients do not require surgical intervention for medical reasons, and the correction is usually only for cosmetic purposes. The purpose of this study was to demonstrate surgical intervention for CS syndrome at a tertiary care facility, and to summarize the available literature. METHODS: Patients with CS syndrome were retrospectively reviewed from a period of June 2012 to August 2019. An extensive literature search for "Currarino-Silverman syndrome," "pouter pigeon chest," "chondromanubrial deformity," "type 2 pectus carinatum" and "pectus arcuatum" was performed. RESULTS: Four clinical cases of CS syndrome are presented, two of which were symptomatic and corrected. The procedure of choice was the modified Ravitch-type thoracoplasty with double osteotomy and implantation of support plates. CONCLUSIONS: There is no clear definition of CS syndrome in the literature. Correct and uniform classification plays a crucial role in the surgical treatment of this pathology. Due to the extreme rarity of the disease, challenging deformity, and variable anatomy of the fused sternum, there are no clear guidelines in treatment approaches. The correction is mostly pursued only for cosmetic results, and the best surgical option for CS syndrome remains the relatively aggressive Ravitch-type procedure with multi-level wedge osteotomy.

Intense and specialized dendritic localization of the fragile X mental retardation protein in binaural brainstem neurons: a comparative study in the alligator, chicken, gerbil, and human.

Neuronal dendrites are structurally and functionally dynamic in response to changes in afferent activity. The fragile X mental retardation protein (FMRP) is an mRNA binding protein that regulates activity-dependent protein synthesis and morphological dynamics of dendrites. Loss and abnormal expression of FMRP occur in fragile X syndrome (FXS) and some forms of autism spectrum disorders. To provide further understanding of how FMRP signaling regulates dendritic dynamics, we examined dendritic expression and localization of FMRP in the reptilian and avian nucleus laminaris (NL) and its mammalian analogue, the medial superior olive (MSO), in rodents and humans. NL/MSO neurons are specialized for temporal processing of low-frequency sounds for binaural hearing, which is impaired in FXS. Protein BLAST analyses first demonstrate that the FMRP amino acid sequences in the alligator and chicken are highly similar to human FMRP with identical mRNA-binding and phosphorylation sites, suggesting that FMRP functions similarly across vertebrates. Immunocytochemistry further reveals that NL/MSO neurons have very high levels of dendritic FMRP in low-frequency hearing vertebrates including alligator, chicken, gerbil, and human. Remarkably, dendritic FMRP in NL/MSO neurons often accumulates at branch points and enlarged distal tips, loci known to be critical for branch-specific dendritic arbor dynamics. These observations support an important role for FMRP in regulating dendritic properties of binaural neurons that are essential for low-frequency sound localization and auditory scene segregation, and support the relevance of studying this regulation in nonhuman vertebrates that use low frequencies in order to further understand human auditory processing disorders.