Caudal regression syndrome and a pelvic kidney: case report

Dany Hage,Joe Iwanaga,Aaron S. Dumont,R. Shane Tubbs 대한해부학회 2020 Anatomy & Cell Biology Vol.53 No.4

Caudal regression syndrome (CRS) is a rare congenital variation. A high incidence of renal and genitourinary malformations has been observed in patients with CRS, with the most common being neurogenic bladder and renal agenesis. We report a rare case report documenting both CRS and a pelvic kidney found during a diagnostic magnetic resonance imaging. Although renal anatomy variants are found in patients with CRS, a pelvic kidney is normally not part of the constellation of findings in this malformation. As seen in our patient, a pelvic kidney should be considered in patients suspected of having CRS.

Case report of a vertical straight sinus with hydrocephalus and Chiari I malformation

Dany Hage,Joe Iwanaga,Aaron S. Dumont,R. Shane Tubbs 대한해부학회 2020 Anatomy & Cell Biology Vol.53 No.4

The straight sinus is a division of the dural venous sinuses, found beneath the splenium of the corpus callosum. At the internal occipital protuberance, it comes together with the superior sagittal sinus and transverse sinus to form the torcular Herophili. It functions as a major site of venous drainage for the cerebellum, inferior sagittal sinus, and vein of Galen. Many morphological variations have been reported involving the angulation, positioning, and number of straight sinuses present. Patients with Chiari II and III malformations have been observed to have a high incidence of anatomical variation with their dural venous sinuses, including vertically oriented straight sinuses. Additionally, there is a high rate of hydrocephalus in this patient population. Herein, we report a vertically oriented straight sinus in a child.

Chiari 1.5 malformation, accessory odontoid synchondrosis, and ventral compression: case report

Dany Hage,Joe Iwanaga,CJ Bui,Aaron S. Dumont,R. Shane Tubbs 대한해부학회 2021 Anatomy & Cell Biology Vol.54 No.1

The pathogenesis of Chiari 1 malformations has been explained in several different ways, but extensive evidence suggests a relationship between loss of volume within the posterior cranial fossa and Chiari 1 presentations. It is important to be able to differentiate Chiari 1.5 from Chiari 1 malformations as they have similar clinical presentations, but the latter have progressed further and are characterized by caudal herniations of the brain stem through the foramen magnum. Despite the similarities of presentation, Chiari 1.5 malformations have greater rates of complications following posterior decompression surgeries, which are typically performed to relieve ventral compression. An improved understanding of the odontoid synchondroses could lead to better understanding of Chiari malformations and lead to improved treatment of patients with these presentations. Here we present a rare case of an accessory odontoid synchondrosis in a patient with a Chiari 1.5 malformation and ventral compression.

Plasmacytoid Dendritic Cells in Pityriasis Rubra Pilaris

( Jana Al-hage ),( Randa Akel ),( Mazen Kurban ),( Ossama Abbas ) 대한피부과학회 2019 Annals of Dermatology Vol.31 No.1

기억의 오염 - 호주에서의 이주와 식민의 책임

가산 하지(Ghassan Hage),박전일 (번역자) 문화과학사 2001 흔적 Vol.- No.2

Internal Mammary Artery Perforator Flap for Immediate Volume Replacement Following Wide Local Excision of Breast Cancer

van Huizum, Martine A.,Hage, J. Joris,Oldenburg, Hester A.,Hoornweg, Marije J. Korean Society of Plastic and Reconstructive Surge 2017 Archives of Plastic Surgery Vol.44 No.6

Background Breast-conserving therapy is defined as a breast-conserving wide local excision (WLE) of a mammary tumour combined with postoperative radiotherapy. Immediate restoration of the mammary shape by use of breast reduction techniques (volume displacement) or tissue replacement techniques (volume replacement) is gaining popularity to prevent breast malformation. Methods To date, using the internal mammary artery perforator (IMAP) flap has been suggested for immediate volume replacement after WLE, but has never been evaluated in a published study. Results We applied this flap in 12 women (mean age, 56.1 years) after WLE (mean specimen weight, 46.5 g) of the medial aspect of the breast. Over a median follow-up of 35.3 months (standard deviation, 1.2 months), 4 women needed repeated surgery for dog-ear correction of the donor site. Conclusions In our experience, the use of an IMAP flap was a reliable technique with good cosmetic outcomes after oncoplastic reconstruction. In this series, donor site revision often proved necessary initially, but we showed that this may easily be prevented.

An algorithm for labia minora reduction based on a review of anatomical, configurational, and individual considerations

Lange Maurits DJ,Hage J. Joris,Karim Refaat B,Amant Frederic 대한성형외과학회 2023 Archives of Plastic Surgery Vol.50 No.1

A variety of labiaplasty techniques have been introduced to date, but no single technique will offer the optimal solution for every patient. Rather, the technique should be chosen based on anatomical, configurational, and technical considerations, as well as on patients’ personal preferences regarded maintenance of the labial rim, maintenance of labial sensitivity, and prevention of iatrogenic thickening of the labium. We reviewed, defined, and assessed labial configurational variety, neurovascular supply, reduction techniques, and patient’s preferences as the considerations relevant to the choice of labiaplasty technique. Based on this review, an algorithm was constructed that leads to a choice of reduction technique through five decisions to be made regarding (1) resection or (partial) retention of the labial free rim, (2) the measure of required labial width reduction, (3) labial vascular status, (4) prevention of iatrogenic labial thickening, and (5) preservation of labial sensibility. The choice of techniques includes edge trimming, central spindle form de-epithelialization or full thickness resection, and three modifications of the wedge resection or de-epithelialization technique. These three modifications comprised a modified anterior resection or de-epithelialization combined with posterior flap transposition, a custom flask resection or de-epithelialization, and a modified posterior wedge resection or de-epithelialization combined with anterior flap transposition. Use of the five decisional steps and the inclusion of modifications of all three conventional reduction techniques offer an improved algorithm for the choice of labioplasty technique.

Internal Mammary Artery Perforator Flap for Immediate Volume Replacement Following Wide Local Excision of Breast Cancer

Martine A. van Huizum,J. Joris Hage,Hester A. Oldenburg,Marije J. Hoornweg 대한성형외과학회 2017 Archives of Plastic Surgery Vol.44 No.6

Background Breast-conserving therapy is defined as a breast-conserving wide local excision (WLE) of a mammary tumour combined with postoperative radiotherapy. Immediate restoration of the mammary shape by use of breast reduction techniques (volume displacement) or tissue replacement techniques (volume replacement) is gaining popularity to prevent breast malformation. Methods To date, using the internal mammary artery perforator (IMAP) flap has been suggested for immediate volume replacement after WLE, but has never been evaluated in a published study. Results We applied this flap in 12 women (mean age, 56.1 years) after WLE (mean specimen weight, 46.5 g) of the medial aspect of the breast. Over a median follow-up of 35.3 months (standard deviation, 1.2 months), 4 women needed repeated surgery for dog-ear correction of the donor site. Conclusions In our experience, the use of an IMAP flap was a reliable technique with good cosmetic outcomes after oncoplastic reconstruction. In this series, donor site revision often proved necessary initially, but we showed that this may easily be prevented.

Short-Term Surgical Complications of Skin-Sparing Mastectomy and Direct-to-Implant Immediate Breast Reconstruction in Women Concurrently Treated with Adjuvant Radiotherapy for Breast Cancer

Merel M. L. Kooijman,J. Joris Hage,Astrid N. Scholten,Marie-Jeanne T. F. D. Vrancken Peeters,Leonie A. E. Woerdeman 대한성형외과학회 2022 Archives of Plastic Surgery Vol.49 No.3

Background Postmastectomy radiotherapy (PMRT) is allegedly associated with a higher risk of complications of combined nipple-sparing or skin-sparing mastectomy and subpectoral direct-to-implant immediate breast reconstruction ([N]SSM/SDTI-IBR). For this reason, this combination is usually advised against or, even, refused in women who need to undergo PMRT. Because this advice has never been justified, we assessed the short-term complications that may potentially be associated with PMRT after [N]SSM/SDTI-IBR. Methods We compared the complications requiring reintervention and implant loss occurring after 273 [N]SSM/SDTI-IBR that were exposed to PMRT within the first 16 postoperative weeks (interventional group) to those occurring in 739 similarly operated breasts that were not (control group). Additionally, we compared the fraction of complications requiring reintervention occurring after the onset of radiotherapy in the interventional group to that occurring after a comparable postoperative period in the control group. Results The fraction of breasts requiring unscheduled surgical reinterventions for complications and the loss of implants did not differ significantly between both groups but significantly more reinterventions were needed among the controls (p¼0.00). The fraction of events after the onset of radiotherapy in the interventional group was higher than the fraction of events after 6.2 weeks in the control group, but not significantly so. Conclusion We found no prove for the alleged increase of short-term complications of adjuvant radiotherapy. Therefore, we advise that these should not be considered valid arguments to advice against [N]SSM/SDTI-IBR.

Direct and Indirect Targeting of PP2A by Conserved Bacterial Type-III Effector Proteins

Jin, Lin,Ham, Jong Hyun,Hage, Rosemary,Zhao, Wanying,Soto-Herná,ndez, Jaricelis,Lee, Sang Yeol,Paek, Seung-Mann,Kim, Min Gab,Boone, Charles,Coplin, David L.,Mackey, David Public Library of Science 2016 PLoS pathogens Vol.12 No.5

<▼1><P>Bacterial AvrE-family Type-III effector proteins (T3Es) contribute significantly to the virulence of plant-pathogenic species of <I>Pseudomonas</I>, <I>Pantoea</I>, <I>Ralstonia</I>, <I>Erwinia</I>, <I>Dickeya</I> and <I>Pectobacterium</I>, with hosts ranging from monocots to dicots. However, the mode of action of AvrE-family T3Es remains enigmatic, due in large part to their toxicity when expressed in plant or yeast cells. To search for targets of WtsE, an AvrE-family T3E from the maize pathogen <I>Pantoea stewartii</I> subsp. <I>stewartii</I>, we employed a yeast-two-hybrid screen with non-lethal fragments of WtsE and a synthetic genetic array with full-length WtsE. Together these screens indicate that WtsE targets maize protein phosphatase 2A (PP2A) heterotrimeric enzyme complexes via direct interaction with B’ regulatory subunits. AvrE1, another AvrE-family T3E from <I>Pseudomonas syringae</I> pv. tomato strain DC3000 (<I>Pto</I> DC3000), associates with specific PP2A B’ subunit proteins from its susceptible host Arabidopsis that are homologous to the maize B’ subunits shown to interact with WtsE. Additionally, AvrE1 was observed to associate with the WtsE-interacting maize proteins, indicating that PP2A B’ subunits are likely conserved targets of AvrE-family T3Es. Notably, the ability of AvrE1 to promote bacterial growth and/or suppress callose deposition was compromised in Arabidopsis plants with mutations of PP2A genes. Also, chemical inhibition of PP2A activity blocked the virulence activity of both WtsE and AvrE1 <I>in planta</I>. The function of HopM1, a <I>Pto</I> DC3000 T3E that is functionally redundant to AvrE1, was also impaired in specific PP2A mutant lines, although no direct interaction with B’ subunits was observed. These results indicate that sub-component specific PP2A complexes are targeted by bacterial T3Es, including direct targeting by members of the widely conserved AvrE-family.</P></▼1><▼2><P><B>Author Summary</B></P><P>Gram-negative bacterial pathogens employ type-III effector (T3E) proteins to suppress host immunity and promote disease symptoms. AvrE-family T3Es, which are widely distributed among plant-pathogenic bacteria, suppress host defense responses and also contribute to water-soaking, which is perhaps the most common symptom of bacterial diseases and likely results in the release of nutrients from host cells to promote pathogen growth. Despite the central virulence functions of AvrE-family T3Es, their mode of action remains enigmatic largely due to their cell toxicity. We report here that two AvrE-family T3Es, WtsE from the maize pathogen <I>Pantoea stewartii</I> subsp. <I>stewartii</I> and AvrE1 from the tomato and Arabidopsis pathogen <I>Pseudomonas syringae</I> pv. tomato, each target protein phosphatase 2A (PP2A) complexes in susceptible hosts via direct interaction/association with specific B’ regulatory subunits. Chemical inhibitors were used to demonstrate that PP2A activity is required for the virulence functions of WtsE and AvrE1. PP2A isoform specificity was also tested using mutants of Arabidopsis. More generally, PP2A subunits regulate, both positively and negatively, rapid pattern-triggered immune responses in Arabidopsis. Thus, bacterial T3Es target sub-component specific PP2A complexes to manipulate host immunity and cause disease symptoms during infection.</P></▼2>