Review of Penile Prosthetic Reservoir: Complications and Presentation of a Modified Reservoir Placement Technique

IntroductionMultiple modifications have been made to the inflatable penile prosthesis (IPP) since its inception in the 1970s. These modifications have made reservoir‐related mechanical malfunctions highly unlikely in current IPP models. Although these complications are rare, it would be incumbent upon the implanting surgeon to be aware of these potential complications, how they present, how they are best treated, and how to prevent them from occurring.AimsThe aim of this article was to present our experience with complications associated with penile prosthesis reservoirs, perform a review of the literature regarding reservoir‐related complications, and present our modified technique to place the reservoir into the space of Retzius.Main Outcome MeasuresReservoir‐related complications including inguinal herniation, erosion into bladder or bowel, intraperitoneal reservoir placement with subsequent visceral injury, vascular injury, autoinflation, and infection.MethodsWe retrospectively reviewed our experience with penile prosthesis reservoir complications or procedures requiring an alternative implantation approach at our center over the past 10 years where over 400 devices were implanted. We also review reservoir‐related complications published in the English literature since the 1980s.ResultsWhile exceedingly rare, reservoir complications do occur. Six cases from our institution are presented including one reservoir herniation, one postoperative direct inguinal hernia, one bladder laceration during revision surgery, one ectopic reservoir placement due to morbid obesity, one iliac vein compression syndrome, and one vascular laceration during reservoir revision. Reported reservoir complications include inguinal herniation, erosion into the bladder or bowel, intraperitoneal reservoir placement with subsequent injury to the ureter or bowel, vascular injury, autoinflation, and infection.ConclusionPenile prosthesis reservoirs rarely fail mechanically but are associated with a variety of complications or may require alternate implantation technique. In our experience, the Jorgensen scissors technique allows safe entry into the space of Retzius with diminished risk of hernia as well as vascular, bladder, or bowel injury. Levine LA and Hoeh MP. Review of penile prosthetic reservoir: Complications and presentation of a modified reservoir Placement technique. J Sex Med 2012;9:2759–2769.     

In Vitro Th17-Polarized Human CD4+ T Cells Exacerbate Xenogeneic Graft-versus-Host Disease

Acute graft-versus-host disease (aGVHD) is a severe complication of allogeneic hematopoietic stem cell transplantation. The role of Th17 cells in its pathophysiology remains a matter of debate. In this study, we assessed whether enrichment of human peripheral blood mononuclear cells (PBMCs) with in vitro Th17-polarized CD4⁺ T cells would exacerbate xenogeneic GVHD (xGVHD) into NOD-scid IL-2Rγ null (NSG) mice. Naive human CD4⁺ T cells were stimulated under Th17-skewing conditions for 8 to 10 days and then coinjected in NSG mice with fresh PBMCs from the same donor. We observed that Th17-polarized cells engrafted and migrated toward xGVHD target organs. They also acquired a double-expressing IL-17A⁺IFNγ⁺ profile in vivo. Importantly, cotransfer of Th17-polarized cells (1?×?10⁶) with PBMCs (1?×?10⁶) exacerbated xGVHD compared with transplantation of PBMCs alone (2?×?10⁶). Furthermore, PBMC cotransfer with Th17-polarized cells was more potent for xGVHD induction than cotransfer with naive CD4⁺ T cells stimulated in nonpolarizing conditions (Th0 cells, 1?×?10⁶?+?1?×?10⁶ PBMCs) or with Th1-polarized cells (1?×?10⁶?+?1?×?10⁶ PBMCs). In summary, our results suggest that human Th17-polarized cells can cooperate with PBMCs and be pathogenic in the NSG xGVHD model.     

Hearing impairment as an early sign of alpha‐mannosidosis in children with a mild phenotype: Report of seven new cases

Alpha‐mannosidosis (AM) is a very rare (prevalence: 1/500000 births) autosomal recessive lysosomal storage disorder. It is characterized by multi‐systemic involvement associated with progressive intellectual disability, hearing loss, skeletal anomalies, and coarse facial features. The spectrum is wide, from very severe and lethal to a milder phenotype that usually progresses slowly. AM is caused by a deficiency of lysosomal alpha‐mannosidase. A diagnosis can be established by measuring the activity of lysosomal alpha‐mannosidase in leucocytes and screening for abnormal urinary excretion of mannose‐rich oligosaccharides. Genetic confirmation is obtained with the identification of MAN2B1 mutations. Enzyme replacement therapy (LAMZEDE^R) was approved for use in Europe in August 2018. Here, we describe seven individuals from four families, diagnosed at 3–23 years of age, and who were referred to a clinical geneticist for etiologic exploration of syndromic hearing loss, associated with moderate learning disabilities. Exome sequencing had been used to establish the molecular diagnosis in five cases, including a two‐sibling pair. In the remaining two patients, the diagnosis was obtained with screening of urinary oligosaccharides excretion and the association of deafness and hypotonia. These observations emphasize that the clinical diagnosis of AM can be challenging, and that it is likely an underdiagnosed rare cause of syndromic hearing loss. Exome sequencing can contribute significantly to the early diagnosis of these nonspecific mild phenotypes, with advantages for treatment and management.     

Severe classical congenital muscular dystrophy and merosin expression

Vajsar J, Chitayat D, Becker LE, Ho M, Ben-Zeev B, Jay V. Severe classical congenital muscular dystrophy and merosin expression. Clin Genet 1998: 54: 193–198. 0 Munksgaard, 1998
It has been suggested that patients with autosomal recessive merosin deficient congenital muscular dystrophy (CMD), as opposed to the merosin positive cases form a homogeneous subgroup of a clinically more severe form of CMD. We examined merosin expression in muscle biopsies from five children with the severe classical form of CMD. Merosin deficiency was found only in 1 patient, a 6–year-old female, with abnormal brain myelination. However, her initial biopsy did not reveal the classical picture of dystrophy. The four merosin positive cases exhibited severe muscle weakness but their brain imagings were normal. There were no familial cases, except for the mother of 1 patient who had a milder form of the disease, suggesting an autosomal dominant mode of inheritance.
In contrast to previous reports, the merosin deficient CMD cases were rare in our group. Furthermore, merosin positive cases were also associated with severe phenotype suggesting that a severe phenotype is not exclusive to merosin deficient cases. Finally, the absence of merosin in a neonate with hypotonia and weakness can be helpful in making a definitive diagnosis of CMD, even though the dystrophic process may not be evident yet and histology may be non-specific.