University of Pennsylvania Health System

Clinical Briefings™: Clinical Reports from Penn Medicine

Monday, April 20, 2015

Peroral Endoscopic Myotomy (POEM) for Esophageal Achalasia

Gastroenterological endoscopists at Penn Medicine are now performing incisionless Peroral Endoscopic Myotomy (POEM) to treat esophageal achalasia.

Achalasia is a rare idiopathic motility disorder that manifests as hypertension and incomplete relaxation of the lower esophageal sphincter (LES) and aperistalsis of the esophageal body. The disorder is a result of impairment of the smooth muscle fibers, leading to failure of bolus transit through the esophagus. Symptoms include dysphagia, regurgitation, heartburn and chronic chest pain, with the consequent potential for weight loss, malnutrition and pulmonary sequelae.

Following diagnosis of esophageal achalasia by esophageal manometry and barium swallow esophagram, the standard surgical treatment is the Heller myotomy. First performed in 1914, Heller myotomy involves cutting the muscles of the LES to open the valve and permit food and liquids to pass into the stomach. Laparoscopic multi-port Heller myotomies are now the preferred approach. Post-operative complications may include infection, bleeding and rarely, esophageal or gastric perforation. The Heller procedure is often combined with fundoplication to prevent gastroesophageal reflux.

Alternatives to surgery for the treatment of achalasia include balloon dilation to expand the constricted sphincter and injections of botulinum directly into the esophagus to relax spastic muscle contractions. Both treatments are effective in the short term, but may require repeated administration to improve the symptoms of achalasia.

A more recent innovation, Peroral Endoscopic Myotomy (POEM) has been developed in Japan by Haruhiro Inoue, MD, PhD, who guided the introduction of the procedure at Penn Medicine. POEM involves the use of endoscopic tools to perform an intramural myotomy (as opposed to the extramural Heller procedure). A full description of the procedure can be found in the Case Study below.

In Dr. Inoue’s original series of 70 cases at Showa University Hospital, Yokohama, Japan, [1] POEM resulted in significant reductions in LES pressure (elevated in most patients with achalasia) and subjective symptom score. Marked improvement was noted in endoscopic appearance and esophageal emptying on barium swallow. Symptomatic post-POEM gastroesophageal reflux disease was observed in 11.4% of patients, but all were successfully treated with standard proton pump inhibitors.

1. Minami H, Inoue H, Haji A, et al. Per-oral endoscopic myotomy: Emerging indications and evolving techniques. Digestive Endoscopy 2015; 27: 175–181.

Case Study

Mr. Y, age 43 years, was referred to Penn Gastroenterology eight months after a botulinum toxin injection procedure for diagnosed achalasia at a hospital near his home in New Jersey.

Mr. Y’s symptoms at this time included protracted post-prandial pain, dysphagia to both solids and liquids and occasional vomiting.

The botulinum toxin injection procedure provided some immediate relief, but within three to six weeks, his symptoms began a slow and progressive return. Mr. Y was in otherwise good health.

At Penn, a barium esophagram revealed esophageal dilatation and stenosis of the cardiac region of the esophagus with delayed evacuation of the barium meal (Fig. 1) . After a consultation to discuss his options, it was agreed that Mr. Y would have a POEM procedure. Mr. Y’s POEM was initiated by creating a 2 cm entry site into the mid-esophageal wall and then a tunnel in the submucosal space extending immediately beyond the esophagogastric junction to the lesser curve of the gastric cardia (Fig. 2).

Next, an inner circular muscle myotomy was performed by grasping and dividing the fibers. Following the myotomy, the endoscope was withdrawn from the submucosal tunnel and reinserted into the lumen to inspect the mucosa, ensure mucosal integrity and confirm easy passage of the endoscope through the LES consistent with an adequate myotomy. The mucosal entry site was then closed with endoscopic clips (Fig. 3).

Mr. Y was observed overnight in the Second-Stage Recovery Unit following his procedure. He received IV-hydration until post-operative day 1, when a barium esophagram was obtained. Noting no loss of mucosal integrity or leakage, Mr. Y was started on a liquid diet and discharged home. Two days later, he began a solid diet, and his recovery thereafter was unremarkable. At his six-month follow-up,
he reported a complete resolution of symptoms.

Faculty Team
The Division of Gastroenterology at Penn Medicine is comprised of a multidisciplinary team of clinician specialists who treat a variety of digestive, liver and pancreatic disorders. Many Penn gastroenterologists are actively involved in clinical research, as well, pioneering advances within their fields to bring more options to the detection and management of inflammatory bowel disease, Crohn’s disease, celiac disease and gastroesophageal reflux disease and other gastrointestinal disorders. The genetics of gastroenterological disease are a particular focus of research at Penn, as are the effects of comorbid disease and other risk factors.

Performing Peroral Endoscopic Myotomy at Penn Medicine

Gregory G. Ginsberg, MD
Director of Endoscopic Services
Professor of Medicine
Professor of Surgery in Medicine

Vinay Chandrasekhara, MD
Assistant Professor of Medicine

Other collaborators include:

Daniel T. Dempsey, MD
Professor of Surgery

Kristoffel R. Dumon, MD
Assistant Professor of Surgery

Gary W. Falk, MD, MS
Professor of Medicine

David Metz, MD
Clinical Chief of Gastroenterology
Professor of Medicine

Michael L. Kochman, MD
Wilmott Family Professor of Medicine
Professor of Surgery in Medicine

Noel N. Williams, MD
Professor of Clinical Surgery

Penn Gastroenterology
Perelman Center for Advanced Medicine
South Pavilion, 4th Floor
3400 Civic Center Boulevard
Philadelphia, PA 19104

Enrolling Clinical Trials at Penn Gastroenterology

Expanding the Clinical Applications of Functional Luminal Imaging (EndoFLIP) in Esophageal Stenoses

The purpose of this study is to investigate the use of a functional luminal imaging probe to characterize benign esophageal luminal strictures before and after dilation and identify predictors of response to therapy. Patients will be evaluated during endoscopy using functional luminal imaging (EndoFLIP; Crospon Medical Devices, Galway, Ireland) to characterize the geometry of benign luminal esophageal narrowing before and after dilation. Contact Maureen DeMarshall, BSN, RN, at

Preliminary Evaluation of Septin9 in Patients With Hereditary Colon Cancer Syndromes
This is an observational, case-control study evaluating the quantitative level of Septin9 in plasma pre- and post-colectomy in patients with hereditary colorectal cancer (CRC) syndrome, Familial Adenomatous Polyposis (FAP), Lynch syndrome (also known as HNPCC), and Multiple Adenomatous Polyposis (MAP, also known as MYK/MYH), with genetically related FAP-family members as controls and references. Contact Julie Starr at (215) 349 – 8527, or

Familial Barrett’s Esophagus (FBE)
This is a multi-center study whose aim is to define the epidemiology and genetics of Barrett’s esophagus and adenocarcinoma. The researchers have studied families affected with Barrett’s esophagus and esophageal adenocarcinoma. They have found that Barrett’s and esophageal cancer occur at a younger age in these families, suggesting that familial Barrett’s esophagus is a genetically inherited disease. Contact Maureen Demarshall, RN, at

Thursday, April 16, 2015

Surgical Management of Drug-Resistant Epilepsy

Epileptologists from the departments of Neurosurgery and Neurology at Penn Medicine have recently introduced a collection of innovative technologies to better treat patients with drug-resistant epilepsy (defined as having seizures refractory to two or more seizure medications).

Medications can control seizures in about two-thirds of persons with epilepsy. The approximate one-third of patients with drug-resistant epilepsy experience a significant impact on quality of life, elevated risk of injury and increased risk of sudden unexpected death.

For patients with drug-resistant epilepsy, the options available at Penn Medicine include resective surgery and vagus nerve stimulation (VNS), as well as newer approaches. Surgery has the potential to cure or decrease seizure frequency when an epileptogenic focus can be identified. Intracranial EEG evaluations are often performed to identify seizure foci prior to resection. Vagus nerve stimulation is an option for patients who are not resective surgical candidates. VNS reduces the frequency and intensity of seizures, but is not curative.

The new technologies available to treat patients with refractory epilepsy at Penn Medicine include Visualase® MRI-Guided Laser Ablation and the NeuroPace RNS® System. Penn neurosurgeons and neurologists have collaborated to introduce these advanced modalities, which can better identify the source of seizures and treat or prevent seizures in patients with drug-resistant epilepsy.

Visualase MRI-Guided Laser Ablation Technology

Visualase laser ablation is a technology that combines a saline-cooled 15 watt, 980-diode laser probe (less than 2 mm diameter) with real-time MRI-guidance to induce interstitial thermal ablation of targeted lesions in the brain (Fig. 1).

Pre-treatment images are acquired for target planning and an intraoperative temperature map is used to minimize damage to healthy tissue. Open surgery is not required. Patients may be awake during therapy and are usually discharged the next day.

NeuroPace RNS System

The NeuroPace RNS System is a programmable responsive neurostimulation system designed to detect and treat abnormal electrical activity in the brain. The System employs brief bursts of electrostimulation from an RNS neurostimulator implanted in the cranium to abort pre-seizure EEG patterns programmed by physicians.

Intracranial EEG (iEEG)
Structural MRI, fdg-PET and ictal scalp EEG recordings cannot identify the epileptic network in many refractory epilepsy patients having pre-surgical evaluation. For these patients, intracranial EEG (iEEG) hybrid depth and subdural grid and strip electrodes (Fig. 3) are required for long-term, high-resolution monitoring and mapping of the cortical surface. iEEG allows Penn clinicians to map the epileptic network and cortical function, making safe resective surgery with a goal of cure possible for many drug-resistant epilepsy patients.

Visualase®; 2015 Medtronic, Inc. Minneapolis, Minnesota.
NeuroPace RNS® System; 2015 NeuroPace, Inc. Mountain View, CA.

Faculty Team

The Penn Epilepsy Center (PEC) is comprised of an interdisciplinary team of clinicians dedicated to advancing the fields of invasive neurophysiology, neuroimaging and neurosurgery for patients with epilepsy in all of its forms. The PEC offers state-of-the-art diagnostic techniques, medical treatments, surgery and support to patients
with epilepsy.




Treating Epilepsy at Penn Medicine

Brian Litt, MD
Director, Penn Epilepsy Center
Professor of Neurology, Bioengineering and Neurosurgery

Gordon H. Baltuch, MD, PhD
Director, Center for Functional and Restorative Neurosurgery
Professor of Neurosurgery

Kathryn Davis, MD, MTR
Medical Director, Epilepsy Monitoring Unit
Assistant Professor of Neurology

Timothy H. Lucas II, MD, PhD
Director, Translational Neuromodulation Laboratory
Assistant Professor of Neurosurgery

John R. Pollard, MD
Associate Professor of Clinical Neurology

Sarah Schmitt, MD
Director, EEG Laboratory
Assistant Professor of Clinical Neurology

Danielle Becker, MD, MSc
Assistant Professor of Clinical Neurology

Doug Maus, MD, PhD
Assistant Professor of Clinical Neurology

The Penn Epilepsy Center

The Penn Epilepsy Center offers a comprehensive, individualized evaluation and a wide variety of surgical treatments for patients whose epilepsy is difficult to manage. As a Level 4 epilepsy center, we have the expertise and facilities to provide the highest-level of medical and surgical evaluation and treatment for patients with epilepsy.

The Center offers comprehensive evaluation for people who have experienced:
  • Long-standing seizures that are not adequately controlled
  • One or more seizures
  • Unacceptable side effects from epilepsy treatments
  • Unusual events that a physician believes may have been seizures

Epilepsy Monitoring Unit

The Epilepsy Monitoring Unit features a modern eight-bed unit with video EEG for the evaluation of individuals who are candidates for surgery and for differential diagnosis of “spells.” Epilepsy patients are admitted for long-term monitoring (anywhere from 3 to 7 days) and are typically weaned from medications to determine the cause and origin of seizures. Some patients undergoing this treatment require intracranial electrode monitoring. A number of other diagnostic tools may also be used to locate the origin of the seizures, including MRI, MEG, EEG, SPECT and PET.


Penn Neuroscience Center
Perelman Center for Advanced Medicine
South Pavilion, 2nd Floor
3400 Civic Center Boulevard
Philadelphia, PA 19104

Pennsylvania Hospital
330 South 9th Street
Philadelphia, PA 19107

Penn Medicine Bucks County
777 Township Line Road
Yardley, PA 19067

Tuesday, April 7, 2015

Diagnosis and Treatment of Dry Eye Syndrome

Penn Dry Eye & Ocular Surface Center

Ophthalmologists at the Penn Dry Eye & Ocular Surface Center are applying recent advances in diagnostic technology to diagnose the primary causes of keratoconjunctivitis sicca (or dry eye syndrome) in order to optimize treatment for the condition.

Traditionally, dry eye syndrome (DES) has been thought of as a deficiency of tears at the ocular surface. Symptoms include blurred vision, scratchiness, irritation, redness or tiredness of the eyes. Recent investigations have shown, however, that DES is much more complex than previously thought, and that “tear film dysfunction syndrome” might more accurately describe the condition.

Tear film dysfunction can be broken down into two basic etiologic classifications: insufficient tear production or increased evaporation of tears from the eye surface. The tear film is made up of lipid, aqueous and mucin components. Individuals with dry eye syndrome can be deficient in any of these basic factors.

Lipid tear deficiency is most commonly caused by blepharitis or meibomian (oil producing) gland dysfunction. This leads to abnormally increased evaporation of the tears from the surface of the eye.
Mucin deficiency can be caused by conditions such as vitamin A deficiency, chemical injury, and Stevens-Johnson syndrome. Mucin is produced by goblet cells, and promotes even distribution of the aqueous tears over the surface of the eye.

Aqueous tear deficiency is associated with insufficient tear production. Congenital causes include conditions such as Riley-Day syndrome or familial dysautonomia. Acquired causes of aqueous tear deficiency include contact lens wear, increasing age, hormonal changes, medications, and Sjogren’s Syndrome and other autoimmune diseases.

DES is often misdiagnosed, and accurate assessment of the underlying causes of a patient’s ocular surface disease is critical. Misdiagnosis and the resulting delay in appropriate treatment can permit the continuation of destructive disease processes and can lead to eventual permanent scarring of the ocular surface.

The Penn Dry Eye & Ocular Surface Center has developed a multidisciplinary approach to identify the cause of a patient’s tear film dysfunction or ocular surface disease. Ophthalmologists collaborate with specialists in other departments to provide care for any medical problems or conditions that may be contributing to the patient’s eye problems.

In the office, specially-trained ophthalmologists perform thorough evaluations of the ocular surface. These assessments may include analysis of the tear film for specific proteins, cytokines and osmolarity. Schirmer testing is used to measure tear production, while optical coherence tomography (OCT) allows quantification of tear meniscus height.

Special stains and impression cytology are used to evaluate the cornea and conjunctiva. Keratographs utilizing trans-illumination and infrared light capture detailed images of the meibomian glands in the upper and lower eyelid and lid margin. The oil or lipid layer is also measured with sophisticated surface interferometers.

Treatment Options
Management of tear film dysfunction and ocular surface disease at the Penn Dry Eye & Ocular Surface Center is tailored to the individual patient and has the objective of promoting the health of the ocular surface. Lifestyle changes, artificial tears and topical eye ointments may help patients with mild DES.

Patients with moderate to severe DES may benefit from medical treatment with immunomodulators, anti-inflammatory agents, omega-3 fatty acid supplements, autologous serum, mucolytic agents or surgical interventions such as punctal occlusion, cautery or various lid surgeries.

The specialists at the Penn Dry Eye & Ocular Surface Center also perform amniotic membrane transplantation, artificial cornea transplants (keratoprosthesis surgery) and other advanced ocular reconstructive surgeries. Therapeutic options for eyelid diseases include intense pulsed light (IPL) therapy (Fig. 2), Lipiflow, Blephex, lid debridement and meibomian gland probing (Fig. 3).

Patients may also be fitted for specialized contact lenses including various types of scleral lenses. In addition, supplemental treatments including drops, gels, ointments, vitamins, lid scrubs, warming and cooling gel packs, goggles, specialized sunglasses, etc. are offered on site for patients to purchase.

Research at the Dry Eye & Ocular Surface Center

Dry Eye Assessment and Management Study (DREAM)

The objective of the DREAM study is to evaluate the effectiveness and safety of supplementation with omega-3 fatty acids in relieving the symptoms of moderate to severe dry eye disease. The study is designed to test the hypothesis that omega-3 supplementation is an effective treatment for dry eye disease (DED). Please see for inclusion criteria.

Primary investigator: Vatinee Bunya, MD
Contact: 215.662.8191

Evaluation of Efficacy of 20 µg/ml rhNGF New Formulation (With Anti-oxidant) in Patients With Stage 2 and 3 Neurotrophic Keratitis

The primary objective of this study is to evaluate the efficacy of 20 µg/ml 6 times a day of recombinant nerve-growth factor (rhNGF) eye drop solution containing anti-oxidant compared to vehicle (formulation containing anti-oxidant) given 6 times a day. The evaluation of efficacy is intended as complete healing of stage 2 (persistent epithelial defect) and 3 (corneal ulcer) neurotrophic keratitis (NK) as measured by the central reading center using corneal fluorescein staining; assessing the duration of complete healing; improvement in visual acuity and improvement in corneal sensitivity. Please see for additional information.

Primary investigator: Mina Massaro Giordano, MD
Contact: 215.662.8100

Faculty Team
Tear film dysfunction and other ocular surface diseases are treated at the Penn Dry Eye & Ocular Surface Center by specially trained ophthalmologists who have a particular interest in caring for patients with dry eye and other types of ocular surface disease. The Center involves collaboration with specialists in cornea and external disease, oculoplastics, contact lens, rheumatology, dermatology and endocrinology.

Mina Massaro-Giordano, MD
Associate Professor, Comprehensive Ophthalmology
Co-Director, Penn Dry Eye & Ocular Surface Center

Vatinee Y. Bunya, MD
Assistant Professor, Cornea & External Disease
Co-Director, Penn Dry Eye & Ocular Surface Center

Stephen E. Orlin, MD
Associate Professor, Cornea & External Disease
Director, Cornea Service

Michael E. Sulewski, MD
Clinical Associate of Ophthalmology
Chief of Ophthalmology, VA Hospital
Co-director, Cornea Service

Sonul Mehta MD
Assistant Professor of Ophthalmology

Frederick B. Vivino, MD
Professor of Clinical Medicine
Director, Sjogren’s Syndrome Center
Chief of Rheumatology, Penn Presbyterian Medical Center

Chadwick R. Johr, MD
Assistant Professor of Clinical Medicine
Co-Director of Sjogren’s Syndrome Center

Catherine M. Quirk, MD
Clinical Associate of Dermatology

David M. Finkel, MD

Contact Lens
Diane Heistand-Talecki, COT, NCLC, FNAO
Kathy McNelis, COA, NCLC
Cynthia Silvestri, NCLC

Penn Presbyterian Medical Center
Scheie Eye Institute
51 N 39th Street
Philadelphia, PA 19104

Complex Retrieval of Embedded Inferior Vena Cava Filters

 Department of Radiology  •  Division of Interventional Radiology

Interventional radiologists at Penn Medicine are performing retrieval of tip-embedded inferior vena cava (IVC) filters using rigid endobronchial forceps, a technique developed by Penn Interventional Radiology. The efficacy and safety of the technique has recently been confirmed in a clinical study published in the journal Radiology. [1]

The largest venous trunk in the body, the IVC is a conduit for thromboemboli originating in the legs.
Among hypercoagulable individuals, particularly those with contraindications for anticoagulant therapy, caval blood clots are a primary cause of catastrophic pulmonary embolism. Percutaneous placement of permanent or retrievable IVC filters is an effective way to trap these clots before they reach the lungs.

Although the FDA recommends that IVC filters be removed when no longer needed, it is estimated that fewer than half of retrievable devices are taken out each year. This number includes the 5 to 10 percent of retrieval attempts that fail because the filter tip is embedded in the vessel wall.

Tip-embedded filters must be removed because they present a substantial risk for vessel occlusion, fracture, and further penetration through the IVC into bowel, bone, arteries and other structures. Standard retrieval of IVC filters involves capturing the devices with snares or cones. Neither technique is effective, however, when the filter tip is embedded in the vessel wall (see Fig. 1).

Every tip-embedded filter retrieval is considered a high-risk procedure. Incomplete, failed or overly aggressive removal of the filter can result in vessel damage and/or further distortion/fracture of the filter.

IVC Filter Retrieval at Penn

For more than a decade, interventional radiologists at Penn Medicine have been developing methods to improve the results of IVC filter retrieval and to optimize retrieval of tip-embedded caval filters.

The approach to these complex retrievals involves the use of rigid endobronchial forceps placed into the IVC from the right internal jugular vein through a sheath and dissecting away engulfing tissue, grasping the filter tip and removing the device (Fig. 2). The technique incorporates several imaging modalities, including rotational venography, spot radiography, and CT venography.

In a recent retrospective study at Penn (see back page), the endobronchial forceps approach was used successfully to retrieve 109 of 114 (96%) tip-embedded IVC filters. Three minor complications and one major complication occurred (the latter involved a patient in whom the struts as well as the tip were embedded), but these resulted in no permanent sequelae.

Case Study

Mr. W, a 37-year-old man, presented to Penn Interventional Radiology with a filter embedded in the wall of his inferior vena cava. According to his medical records, he had a history of deep vein thrombosis, for which he’d been taking warfarin for some years. Recently, however, he’d experienced a bleeding ulcer, requiring him to temporarily cease taking the drug.

To protect Mr. W from pulmonary emboli in this interim, a retrievable filter was placed in his inferior vena cava at his community hospital. After Mr. W’s ulcer had healed and he re-started warfarin, he returned to the hospital to have the IVC filter removed. An inferior vena cavagram at this time showed the tip of the filter embedded in the vessel wall, however, and the retrieval attempt was abandoned.

Mr. W was then referred to Penn Interventional Radiology, where he was scheduled for a complex IVC filter retrieval after an office consultation. His anticoagulation was not interrupted for the one-hour procedure, which was performed in an outpatient setting. Following access at the right internal jugular, endobronchial forceps were used to cut away the tissue surrounding the embedded tip, allowing the filter to be grasped and removed.

Following the procedure, Mr. W was observed for two hours; he went home the same day. He had no adverse effects from the procedure and remains under the care of his family physician.

Faculty Team

The specialists with the Interventional Radiology Division at Penn Medicine offer the diagnosis and treatment of a variety of diseases using minimally invasive techniques. The Division is situated in six interventional radiology suites at the Hospital of the University of Pennsylvania, and has an active outpatient clinic, admitting and consulting service.

Performing Complex IVC Retrievals at Penn Medicine

Scott O. Trerotola, MD
Stanley Baum Professor of Radiology
S. William Stavropoulos, MD
Professor of Radiology

Hospital of the University of Pennsylvania
Mandeep S. Dagli, MD
Assistant Professor of Clinical Radiology
Stephen Hunt, MD
Instructor in Radiology
Maxim Itkin, MD
Adjunct Associate Professor of Radiology
Jeffrey I. Mondschein, MD
Associate Professor of Clinical Radiology
Gregory Nadolski, MD
Assistant Professor of Radiology
Richard Shlansky-Goldberg, MD
Professor of Radiology
Michael C. Soulen, MD
Professor of Radiology
Deepak Sudheendra, MD
Assistant Professor of Clinical Radiology
Micah Watts, MD
Adjunct Assistant Professor of Radiology

Penn Presbyterian Medical Center
Timothy W.I. Clark, MD
Associate Professor of Clinical Radiology
Jonas Redmond, MD
Assistant Professor of Clinical Radiology

Pennsylvania Hospital
Raymond Fabrizio, MD
Assistant Professor of Clinical Radiology
Benjamin D. Hammelman, MD
Assistant Professor of Clinical Radiology


Penn Interventional Radiology
Hospital of the University of Pennsylvania
1 Silverstein
3400 Spruce Street
Philadelphia, PA 19104

Penn Presbyterian Medical Center
4 Wright-Saunders Building
51 North 39th Street
Philadelphia, PA 19104

Penn Radiology
Hospital of the University of Pennsylvania
Ground Dulles
3400 Spruce Street
Philadelphia, PA 19104

Tuesday, March 17, 2015

Total Proctocolectomy with J-pouch Reconstruction for Ulcerative Colitis

Division of Colon and Rectal Surgery

Colorectal surgeons at Penn Medicine are performing total proctocolectomy with J-pouch reconstruction/intestinal pouch anal anastomosis (IPAA) for patients with ulcerative colitis (UC).

Since its introduction in the US in the 1980s, this procedure has undergone technical changes that make it much better tolerated, with improved outcomes and faster recovery. In selected individuals, it replaces total proctocolectomy with end ileostomy, and allows for the retention of gastrointestinal continuity—a major concern for all patients.

Emergent indications for surgery in UC include acute flares refractory to medical control, sudden, severe disease manifesting as uncontrolled bleeding in the colon, toxic megacolon, and perforation of the bowel. By contrast to Crohn’s disease, UC is cured by removal of the diseased colon and rectum.

At Penn Medicine, elective total proctocolectomy with J-pouch for UC is offered to patients who have pre-cancerous or dysplastic colonic mucosal changes and to those patients refractory to medical management with intolerable symptoms such as frequency, pain and urgency leading to a progressive decline in quality of life. Because the rate of synchronous or subsequent adenocarcinoma ranges from 10%-50% in this population, both high- and low-grade dysplasia constitute indications for proctocolectomy.

Total proctocolectomy with J-pouch reconstruction is most commonly performed in either two or three stages depending on the condition of the patient. Three-stage procedures are performed for acutely or chronically ill malnourished individuals, patients on high doses of immunosuppressive medications, or those who present emergently with the indications for surgery listed above.

The first stage is a laparoscopic total colectomy with preservation of the rectum and end ileostomy in the right lower quadrant of the abdomen (Figure 1). Sparing the rectum in these circumstances is important. Proctectomy is often the most technically challenging of the procedure. Performing this part of the operation in a well-nourished, healthy, immunocompetent individual reduces morbidity and makes J-pouch creation safer by improving outcomes and reducing septic complications. The second part of the procedure occurs about 3-4 months later depending on the patient’s performance status. This step involves removal of the rectum, creation of the J-pouch from the terminal ileum (about 20 cm) and temporary loop ileostomy to divert the fecal stream proximal to the J-pouch. About two months after J-pouch creation (after the pouch is checked via gastrograffin enema for leaks, sinus tracts or defects) the loop ileostomy is closed through a small peristomal incision (Figure 2).

Two stage procedures are done frequently in well-nourished patients who present electively for proctocolectomy for indications such as dysplasia or failure of medical management. Patients are screened for malnutrition, and are asked to stop anti-TNF therapy about one month in advance of the procedure. Prednisone doses higher than 20mg/day have been associated with J-pouch leaks/complications; thus consideration for a three stage procedure is warranted if higher doses of steroids are required.

Case Study

Mr. V, a 37-year-old with medically refractory ulcerative colitis, was referred to Penn Medicine for evaluation. A review of his medical history indicated that Mr. V’s disease began in his late teens. At this time, his symptoms included bloody diarrhea, bloating, acute pain and cramping.

In the decades since, his UC had responded for varying periods of time to mesalazine, azathioprine, prednisone and finally, infliximab. Each medication brought about a remission followed by a gradual return of symptoms and flare-ups, the most recent of which was attended by 20 to 30 bowel movements a day, dramatic weight loss and hospitalization.

At the time of admission, Mr. V was taking infliximab every two weeks, and was on 30 mg prednisone daily. After a consultation to discuss further medical therapy with other anti TNF-alpha alternatives, it was discovered that Mr. V had considered surgery, but was reluctant because he felt he was too young for an ileostomy.

After counseling, and in consultation with the Division of Gastroenterology, there was agreement that Mr. V would have a total proctocolectomy with J-pouch reconstruction, and that his acute presentation, relative malnutrition and immunosuppression required that the surgery proceed in three stages.

Mr. V returned home two days after the initial step (laparoscopic subtotal colectomy with temporary end ileostomy) in the three-step procedure. In the next four months, he gained almost twenty pounds while gradually weaning himself from prednisone. His sleep improved and for the first time in several years, he was able to begin moderate exercise. Returning for the proctectomy and creation of the J-pouch and temporary loop ileostomy, Mr. V spent another three days in the hospital, then returned home. Two months later his ileostomy was reversed.

Today, at a year post-surgery, he has between four and six bowel movements a day, with perfect control. He is exercising regularly, eating previously forbidden foods and has no activity restrictions or limitations.

Faculty Team

The Division of Colon and Rectal Surgery at Penn Medicine provides the highest quality diagnostic and surgical options for patients with colon, rectal and anal cancer, inflammatory bowel disease (Crohn’s disease and ulcerative colitis), diverticular disease and many other diseases of the colon, rectum and anus. The division offers sphincter-preserving colon and rectal surgery for cancer and benign disease, laparoscopic and robotic colorectal surgery, treatment for fecal incontinence and rectal prolapse and both operative and medical therapies for anal diseases.

Performing Total Proctocolectomy and J-Pouch Reconstruction for Ulcerative Colitis at Penn Medicine

Najjia N. Mahmoud, MD
Chief of Colon and Rectal Surgery
Associate Professor of Surgery

Cary B. Aarons, MD
Assistant Professor of Clinical Surgery

Joshua I. S. Bleier, MD
Associate Professor of Clinical Surgery

Skandan Shanmugan, MD
Assistant Professor of Clinical Surgery


Department of Surgery

Perelman Center for Advanced Medicine
3400 Civic Center Blvd
Philadelphia, PA 19104

Penn Medicine University City
3737 Market Street, 4th Floor
Philadelphia, PA 19104

Penn Medicine Washington Square
800 Walnut Street, 20th Floor
Philadelphia, PA 19107

Wednesday, January 28, 2015

Medical and Surgical Management of Myasthenia Gravis and Thymoma

 Department of Thoracic Surgery • Department of Neurology

A multidisciplinary Myasthenia and Thymoma Program at Penn Medicine has been established to coordinate and expedite the management of patients with myasthenia gravis (MG), thymoma and related diseases.

Staffed by neurologists, thoracic surgeons, radiologists, ophthalmologists and many other specialists, the program has the objective of providing accurate diagnoses and medical management for MG and surgical intervention for thymoma and other thymic disorders.

Myasthenia gravis is an autoimmune disorder caused in most patients by antibodies that destroy acetylcholine receptors (AChR) at the neuromuscular junctions of striated muscles. The resulting
loss of AChR manifests in patients with MG as progressive muscle weakness.

The course of MG is variable and symptoms are generally nonspecific, leading frequently to missed or mistaken diagnoses. Treatment for MG can substantially improve the symptoms of the disease. Thus, a missed diagnosis means, at the least, a continued deterioration in quality of life. However, for myasthenia gravis patients with thymoma (an associated disorder that affects up to a third of patients with the disease), or any of a host of comorbidities treated with drugs that inhibit neuromuscular transmission, a missed diagnosis can have profoundly deleterious effects.

At Penn, specialists across the therapeutic spectrum are trained to recognize the early signs of MG so that a confirmatory diagnosis can be made early in the course of the disease. This is typically achieved by blood tests for the presence of antibodies to acetylcholine receptors, electromyography (EMG) and if needed, single-fiber electromyography. Once confirmed, patients have radiographic scans to check for thymoma.

Medical treatment for MG at Penn may include cholinesterase inhibitors (e.g., Mestinon®), steroids and immunosuppressants, which increase the relative amount of acetylcholine by reducing the immune-mediated attack against the AChR. Two procedures are also commonly used to treat MG at Penn. The first, plasmapheresis, removes AChR antibodies from the blood through a process resembling dialysis; the second, intravenous immune globulin (IVIg) therapy, involves infusing pooled gamma globulin to restructure the immune response to AChR. Expanding upon these options, Penn is also a thriving source of clinical trials for patients who are refractory to therapy.

With improvement in MG symptoms, patients with early stage thymoma and selected individuals without thymoma may be candidates for thymectomy. When possible, robotic surgery is preferred as an alternative to sternotomy and transsternal procedures, which are associated with longer hospital stays, increased operative duration and greater blood loss.

Case Study

Mr. D, a 68-year-old man, was referred to a specialist at Penn Otorhinolaryngology-Head and Neck Surgery after a 15-month history of episodic choking and recurrent aspiration pneumonia. Noting mild ptosis that increased in severity upon sustained upward gaze, Mr. D was referred to Penn Neurology, where an anti–acetylcholine receptor (AChR) antibody (Ab) test and electromyography confirmed myasthenia gravis. A chest CT subsequently identified a 4 cm mass in the anterior mediastinum consistent with a thymoma.

Following plasmapheresis and IVIg with concomitant Mestinon® (pyridostigmine bromide) therapy, Mr. D’s symptoms improved sufficiently to permit surgery, and he was scheduled for a robotic thymectomy.

Prior to surgery, an epidural was placed to optimize postoperative pain management; a right chest approach was utilized along with a 3-port technique. Robotic dissection began with the right pericardial fat pad and progressed both cranially and to the left. Both phrenic nerves could be well visualized using the 3-dimensional camera system, and both were preserved during skeletonization, which included all the surrounding fat and tissue (Figure 1). There was no evidence of pericardial involvement as the specimen was dissected free. The brachiocephalic vein was skeletonized and dissection proceeded into the neck to include both upper poles.

On postoperative day 1, Mr. D’s single chest tube was removed and he was discharged home that afternoon on oral narcotics. He required the narcotics for only the first week, resumed his dose of Mestinon, and was able to return to his normal activities after the second week. He continues on Mestinon therapy but at a declining dose two years later.


Penn Neurology
Penn Medicine University City
8th Floor
3737 Market Street
Philadelphia, PA 19104

Thoracic Surgery
Penn Medicine University City
4th Floor
3737 Market Street
Philadelphia, PA 19104

Faculty Team
The Penn Medicine Myasthenia and Thymoma Program is comprised of a multidisciplinary team of thoracic surgeons, neurologists and other specialists dedicated to the comprehensive management
of patients with myasthenia gravis and its associated effects and conditions, including thymoma.

Myasthenia and Thymoma Program Faculty Team

Alan D. Askenase, MD
Clinical Associate Professor of Medicine

Evan W. Alley, MD, PhD
Clinical Associate Professor of Medicine

Sami L. Khella, MD
Chief, Department of Neurology, Penn Presbyterian Medical Center
Professor of Clinical Neurology

Madhura A. Tamhankar, MD
Assistant Professor of Ophthalmology

Joel L. Deitz, MD
Clinical Associate Professor of Medicine

Radiology (Chest)
Warren B. Gefter, MD
Professor of Radiology

Radiation Oncology
Abigail T. Berman, MD

Thoracic Surgery
Taine T.V. Pechet, MD
Interim Chief of Surgery, Penn Presbyterian Medical Center
Associate Professor of Clinical Surgery

Transfusion Medicine
Donald L. Siegel, MD, PhD
Professor of Pathology and Laboratory Medicine

Nicole Aqui, MD
Assistant Professor of Clinical Pathology and Laboratory Medicine

Additional Specialists
Christine H. Hosay, BSN,CMSN
Jeanmarie Salonia, PharmD

Computed Tomography for Thymoma
At Penn Medicine, patients with myasthenia gravis typically undergo computed tomography (CT) imaging to rule out concomitant thymoma. When a thymoma is identified (as in Figure 2), CT is used to characterize the tumor and to investigate the presence and extent of local invasion.

CT scans are also an important source of incidental findings of thymoma in patients assessed for diseases other  than myasthenia gravis.

Tuesday, January 6, 2015

Current Aortic Aneurysm Stent Device Clinical Trials

Vascular Surgery and Endovascular Therapy

Researchers with the Division of Vascular Surgery and Endovascular Therapy at Penn Medicine are conducting clinical trials to evaluate investigational stent grafts for the endovascular repair of abdominal, juxtarenal and pararenal aortic aneurysms.

Three of the studies currently enrolling at Penn are examining devices manufactured by Cook Medical (Bloomington, IN) under the Zenith brand name: the p-Branch stent graft, the Low Profile AAA Endovascular Graft, and the Branch Endovascular Graft-Iliac Bifurcation. Under the direction of principal investigator Ronald M. Fairman, MD, the studies are seeking to ascertain the safety and efficacy of these investigational devices.

The Division of Vascular Surgery and Endovascular Therapy has participated in virtually every stent graft clinical study in the United States since 1996. For information about enrolling in the Zenith studies and other endovascular clinical trials at Penn Medicine, please contact:

Heidi Martin, MS, Clinical Research Coordinator.

Phone: 215-662-4320


Zenith® p-Branch OTS Multicenter Study

This study is investigating the safety and effectiveness of the Zenith p-Branch stent graft as an off-the-shelf option for the treatment of pararenal or juxtarenal abdominal aortic aneurysms. The p-Branch has a unique “off-the-shelf” design with pivoting renal portals that accommodate a
comprehensive range of patients.

Advantages include fenestrations incorporated in the design of the graft to maintain perfusion through the renal arteries and visceral vessels (celiac artery and superior mesenteric artery) and avoidance of open surgery. The study device(s) are inserted through a small incision near each hip and guided into place in the aorta.

Zenith® Low Profile AAA Endovascular Graft Clinical Study

This multi-center, prospective non-randomized clinical investigation is designed to evaluate the safety and effectiveness of the Zenith Low Profile AAA Endovascular Graft in conjunction with the Zenith Spiral-Z AAA Iliac Leg Graft. Study endpoints include freedom from major adverse events at 30 days and (for the treatment cohort) device success at 12 months.

These findings will be compared to performance goals derived from the results of the Zenith AAA Endovascular Graft clinical study. The Zenith Spiral-Z AAA Iliac Leg Graft is indicated for use during a primary or secondary procedure in patients who have iliac/femoral access that is both adequate and compatible with the graft’s Z-Trak® introduction system.

PRESERVE-Zenith® Iliac Branch Clinical Study

The purpose of this study is to evaluate the safety and effectiveness of the Zenith Branch Endovascular Graft-Iliac Bifurcation in combination with the commercially available Atrium* iCAST™ covered stent in patients with an unsuitable distal sealing site for a Zenith iliac leg component proximal to the common iliac bifurcation.

PRESERVE is an extended, multi-center, prospective, non-randomized trial. Patients with anatomy amenable to endovascular repair who meet study criteria will be enrolled. Because the Branch Endovascular Graft-Iliac Bifurcation is intended to maintain blood flow to the internal iliac artery and minimize the risk of associated clinical symptoms with the need for re-intervention, the primary assessment will be based on six-month freedom from patency-related intervention.

The Branch Iliac Endovascular Graft-Iliac Bifurcation was designed to reduce the risks of complications for patients with iliac aneurysms by preserving blood flow to the internal iliac. The Atrium iCAST balloon expandable covered stent offers a low foreshortened design and a one-step deployment technique that enhances placement accuracy.

Faculty Team

The Division of Vascular Surgery and Endovascular Therapy at Penn Medicine is currently the regional leader for carotid, aortic, and peripheral arterial repair surgeries, and is among the handful of research centers nationwide involved in clinical trials to expand the indications for endovascular stent grafts. These new indications will include previously underserved patient populations and complex and complicated aneurysmal disease, including juxtarenal and pararenal aneurysms.

Performing Endovascular Clinical Trials at Penn Medicine

Scott M. Damrauer, MD
Assistant Professor of Surgery

Ronald M. Fairman, MD
Chief, Division of Vascular Surgery and Endovascular Therapy, Clyde F. Barker-William Maul Measey Professor of Surgery

Paul J. Foley, III, MD
Assistant Professor of Clinical Surgery

Michael A. Golden, MD*
Associate Professor of Surgery

Benjamin M. Jackson, MD
Assistant Professor of Surgery J

Venkat R. Kalapatapu, MD
Assistant Professor of Clinical Surgery

Paul L. O’Donnell, DO‡
Clinical Assistant Professor of Surgery

Grace J. Wang, MD, FACS
Assistant Professor of Surgery

*Penn Presbyterian Medical Center
†Penn Medicine University City
‡Cape Regional Medical Center


Patient appointments are available at:

Hospital of the University of Pennsylvania
3400 Spruce Street
4 Silverstein Pavilion
Philadelphia, PA 19104

Perelman Center for Advanced Medicine
Penn Heart and Vascular Center
East Pavilion, 2nd Floor
3400 Civic Center Boulevard
Philadelphia, PA 19104

Penn Presbyterian Medical Center
Department of Surgery
266 Wright Saunders Building
39th & Market Streets
Philadelphia, PA 19104

Penn Medicine University City
4th Floor
3737 Market Street
Philadelphia, PA 19104

Penn Medicine Radnor
250 King of Prussia Road
Radnor, PA 19087

Penn Medicine Bucks County
Suite 140
777 Township Line Road
Yardley, PA 19067

Cape Regional Physician Associates
217 North Main St., Suite 104
Cape May Court House, NJ 08210

All images ©Cook Medical (Bloomington, IN), 2014.

Visit Penn Physician VideoLink to view physician-focused videos, Clinical Briefings and more from Penn Medicine.

Friday, December 5, 2014

Paired Kidney Exchange for Kidney Transplantation

The Penn Transplant Institute is a leading center for paired kidney exchange for kidney transplantation, a practice that depends largely upon the benevolence of anonymous, altruistic donors.

Principles and Principals of Paired Kidney Exchange
A common perception of paired kidney exchange is that the process involves single, two-way exchanges between recipients and altruistic donors within their circle of acquaintance or relation. In fact, this is rarely the case.

Almost always, despite friends and family members who were evaluated as live kidney donors, the recipient has failed to find an appropriate match. Incompatibility between donor/recipient pairs can come about not only because of blood type, but because the recipient has been sensitized by previous blood transfusions, previous organ transplants or previous pregnancies.

More often, paired kidney exchanges occur as chains initiated between a “non-directed,” anonymous, altruistic donor and a matched recipient and continued when a friend or relative of this recipient repeats the process by donating to a second matched anonymous recipient. If this person, too, is linked to an unmatched donor, that donor may be enlisted to continue the chain as a donor to another anonymous recipient. Donors in the chain who do not immediately find a matched recipient may choose to begin a separate chain as “bridge” donors from the original chain (see Figure 1).

At Penn, recipients and donors who are willing to participate in paired kidney exchange must undergo a comprehensive medical evaluation. During this stage, the kidney transplant and living donor transplant teams complete a series of evaluations to determine if the procedures are safe and appropriate for the respective recipients and living donors. Only when the team feels it is safe to proceed are the donor and recipient surgeries scheduled.

The benefit of paired kidney exchange is that it offers kidney recipient and donor pairs who aren’t blood and/or tissue-type compatible an alternative to deceased donor transplantation, thus increasing the number of kidney donors while diminishing competition for deceased donor kidneys.

Paired kidney exchanges also play a critical role in expediting transplants and thereby improving outcomes. According to the United States Department of Health and Human Services, the median waiting time among patients aged 18 to 65 in the US for a deceased-donor kidney in 2014 was 4.5 years, versus 185 days for patients participating in paired kidney exchange. The estimated six-month graft survival rate for patients having paired kidney exchange was 98.8%, compared to 95.1% for deceased donor kidney transplants. [1]

Case Study

Upon learning that 32-year-old Mr. B had end-stage kidney disease, his uncle, Mr. C, volunteered to donate a kidney to him. However, tests at the Penn Transplant Institute showed that Mr. C and Mr. B were not a compatible match. Subsequently, Mr. B entered the home dialysis program at Penn and was placed on the waiting list for a deceased kidney.

Several months later, a transplant coordinator at Penn contacted Mr. C and Mr. B to ask if they would be interested in participating in a paired kidney exchange. She explained that an anonymous altruistic donor, Mr. A, had been found compatible with Mr. B and had agreed to donate a kidney to him. The coordinator asked if Mr. C would consider becoming a kidney donor for an anonymous recipient for whom he was compatible.

Mr. C agreed to become a donor, and at Penn, was paired with Mrs. D. The chain would thus involve Mr. A donating a kidney to Mr. B, and Mr. C donating a kidney to Mrs. D. All four participants in the resulting exchange were found to be physically and psychologically prepared for transplant surgery. The four surgeries were scheduled to take place at Penn Medicine on the same day in separate operating rooms.

On the morning of the kidney exchange, Mr. A had surgery first, and his kidney was transplanted into Mr. B. Later that day, a similar exchange took place. This time, Mr. C’s kidney was transplanted into Mrs. D.

All four patients recovered without incident. In the days after surgery, Mr. C and Mr. B decided to meet the pair with whom they had been matched. All donors currently have functioning transplants, due in large part to the initial intervention of Mr. A, the initiating altruistic donor.

Adding to the success of this exchange, Mr. E, the husband of Mrs. D, initiated a second chain of paired exchange kidney transplants two months later by donating a kidney to Mr. F, thus becoming an altruistic “bridge” donor to this new chain.

1. State of the OPTN/UNOS KPD Pilot Program, DHHS 2014]

Faculty Team
The Penn Transplant Institute is home to kidney, liver, lung, heart, pancreas and hand transplantation programs, and is ranked among the top 10 multi-organ transplant centers in the country.

Performing Kidney Transplantation at Penn Medicine

Transplant Surgeons

Ali Naji, MD, PhD
Surgical Director, Kidney and Pancreas Transplant Program
Professor of Surgery

Peter L. Abt, MD
Associate Professor of Surgery

Matthew H. Levine, MD, PhD
Assistant Professor of Surgery

Paige Porrett, MD, PhD
Assistant Professor of Surgery

Transplant Nephrologists

Roy D. Bloom, MD
Medical Director, Kidney and Pancreas Transplant Program
Professor of Medicine

Melissa B. Bleicher, MD
Assistant Professor of Clinical Medicine

Simin Goral, MD
Professor of Medicine

Robert A. Grossman, MD
Professor of Medicine

Mary Ann Lim, MD
Assistant Professor of Clinical Medicine

Peter P. Reese, MD, MSCE*
Assistant Professor of Medicine and Epidemiology

Deirdre L. Sawinski, MD
Assistant Professor of Medicine

Karen Warburton, MD*
Associate Professor of Clinical Medicine

Other Renal Physicians
Meera N. Harhay, MD, MSCE

Attending Physician
Jonathan S. Maltzman, MD, PhD
Assistant Professor of Medicine

Renal Nurse Practitioners
Christina Braun, MSN, CRNP
Carolyn Mylowe, MSN, CRNP
Gail Eastman, MSN, CRNP, CCTC
Jenna Angerstein, MSN, CRNP, CCTN

Transplant Surgery Nurse Practitioners
 Alva Moy-Daley, MSN, CRNP
 Julie Spaulding, MSN, CRNP

*Living Donor Team Nephrologists

Paired Kidney Exchange at Penn Medicine
Penn performed the first successful kidney transplant in 1966, and the Penn Transplant Institute is now a leader in the effort to increase paired kidney exchanges for kidney transplantation.
In partnership with the National Kidney Registry (NKR), a consortium of 72 transplant centers nationwide, the Penn Transplant Institute recently took part in the largest kidney exchange to be concluded in under 40 days and the second largest in history (ultimately involving 28 donors and 28 recipients).

In 2012, the US Department of Health and Human Services awarded the Penn Transplant Institute’s Kidney Transplant program a Silver Level Award. Penn is the only transplant center in the region and one of a select group of programs in the nation to receive this distinction.

In addition to its partnership with the NKR, the largest and most successful kidney transplant matching program in the US, the Penn Transplant Institute is a member of the United Network for Organ Sharing (UNOS). Outside of its participation with these organizations, the Transplant Institute has a large enough waiting list to initiate donor chains within its own programs.


Penn Transplant Institute Kidney Transplant Program

The Perelman Center for Advanced Medicine
2 West Pavilion
3400 Civic Center Boulevard
Philadelphia, PA 19104

Renal Electrolyte and Hypertension
Perelman Center for Advanced Medicine
South Pavilion, 1st Floor
3400 Civic Center Boulevard
Philadelphia, PA 19104

Patients also evaluated at:

Penn Medicine Radnor*
250 King of Prussia Road
Radnor, PA 19087
* A facility of the Hospital of the University of Pennsylvania

Penn Medicine Bucks County
777 Township Line Road
Yardley, PA 19067

Penn Medicine—1400 East Route 70
1400 East Route 70
 Cherry Hill, NJ 08034

Penn Medicine Woodbury Heights
1006 Mantua Pike
Woodbury Heights, NJ 08097

The contact number for recipients and donors is 215.662.6200.

Visit Penn Physician VideoLink to view physician-focused videos, Clinical Briefings and more from Penn Medicine.

Thursday, November 20, 2014

Periacetabular Osteotomy for Complex Structural Hip Deformity

Department of Orthopaedic Surgery  •  Center for Hip Preservation

Orthopaedic surgeons at Penn Medicine are performing periacetabular osteotomy (PAO) surgeries for native hip preservation in adolescents, young adults and adults (generally up to 45 years of age) with dysplasia and other structural hip deformities.

Structural hip deformities affect a large proportion of younger adults who present with symptomatic hip pain in the United States. These conditions are often of congenital, developmental, or traumatic origin, and typically involve morphologic abnormalities of the acetabulum or femur leading to instability and/or impingement, a pathological conflict between the two bones during movement.

The mechanical aberrations identified with structural hip deformity commonly involve dysplasia and femoroacetabular impingement (FAI) and their associated pathologies. Hip pathology can also involve malalignment or rotational deformities of the femur (e.g., issues with femoral torsion), among other anomalies.

Impingement occurs when the proximal femur contacts the acetabulum during range of motion, such as flexion and internal rotation. Impingement is often caused by asphericity of the femoral head (cam type) or over-coverage of the acetabulum (pincer type). With dysplasia, insufficient coverage of the femoral head by the acetabulum can lead to symptomatic instability and early labral and cartilage degeneration. This continuum—from impingement to instability—is an important concept in evaluating younger patients with hip pain.

The tantamount consideration in younger patients with dysplasia and/or FAI is to preserve the native hip by addressing underlying structural abnormalities. Accordingly, orthopaedic surgeons at Penn Medicine employ a spectrum of corrective hip preservation surgeries for this population, including both arthroscopic and open techniques such as re-orientation of the acetabulum.

Periacetabular osteotomy (PAO), for example, addresses the underlying structural deficiencies of a shallow or poorly oriented acetabulum. In PAO, a series of osteotomy cuts encompass the acetabulum, preserving the hip abductors and the posterior, weight-bearing column of the pelvis. The socket is then freed from the pelvis and reoriented in a position of better coverage of the femoral head. This offers the opportunity to restore more normal hip joint mechanics and ideal loading of the articular cartilage. The goal is long-term durability of the native joint in adolescent and young adult patients with dysplasia.

PAO is not a “standard” surgery, and patient selection is important. The procedure is demanding, in that osteotomy and precise reorientation are required, and each step requires a certain level of experience and training. However, the potential advantages of PAO, which include greater long-term joint stability and durability of the cartilage, may help younger patients to avoid or delay joint-replacement surgery, such as total hip arthroplasty.

Case Study

Mr. J, a 25-year-old man, was referred to the Center for Hip Preservation at Penn Medicine with structural deformity of the right hip, the result of a segmental fracture of the proximal femur at age four. His hip and leg were placed in a cast, and his fracture healed in a malaligned position.

As a young adult, Mr. J had chronic, progressive and disabling pain (especially in the setting of his high-demand work as a manual laborer) that he managed with daily opioid medications. In the months prior to evaluation at Penn, he had extensive physical therapy and an intra-articular injection for pain control. He was unemployed for much of this time.

At Penn, Mr. J was deemed a candidate for hip preservation. Imaging determined that he had severe acetabular dysplasia, a tear of his acetabular labrum, a torsional deformity of the femur (45 degrees of retrotorsion) and FAI due to a cam lesion of the femoral head-neck junction (Fig. 1).

Despite these abnormalities, however, there was no evidence of overt osteoarthritis at his hip. After discussion of the risks, benefits and treatment alternatives, Mr. J elected to have corrective surgery. Pre-procedural imaging and modeling were completed to ensure proper intra-operative alignment targets of the reoriented hip and femur.

At PAO was performed to correct the acetabular dysplasia. Nerve monitoring and fluoroscopic imaging were used to ensure safe and accurate surgery. Bone grafting was completed at the acetabular osteotomy sites, with autograft harvested from the pelvic osteotomy mobile fragment. A surgical hip dislocation was also performed, with careful preservation of the critical blood supply to the femoral head. A labral repair and femoral head-neck osteochondroplasty were completed to address the intra-articular sequelae of FAI.

Although Mr. J’s intra-articular causes of FAI were optimized, the extra-articular rotational deformity of the femur did not allow for adequate impingement-free range of motion. Therefore, a subtrochanteric derotational osteotomy with internal fixation was performed to bring the femur into normal rotational alignment. Bone autograft harvested from the greater trochanteric bed was used to supplement the osteotomy fixation (Fig. 2).

Mr. J was on crutches the day after his procedure and was in the hospital for three days, where he received physical therapy, as well as a continuous passive motion machine. Partial weight-bearing was permitted on discharge. He was weaned from all pain medications by several months post-operatively. At six months, he was walking with no gait aids, and by seven months he had found full-time work.

Faculty Team

The Penn Medicine Center for Hip Preservation is comprised of a multi-disciplinary team dedicated to the diagnosis and treatment of hip pain in adolescent, young adult and adult patients. Patients evaluated for hip pain at Penn also have access to the region’s first integrated Musculoskeletal Center, which includes state-of-the-art motion analysis, neuromuscular testing, and advanced metabolic measurements through the Center for Human Performance.

The exceptional procedures offered at the Penn Center for Hip Preservation include hip arthroscopy with labral repair, arthroscopic and open treatment of focal cartilage injury, management of avascular necrosis of the femoral head with stem-cell therapy, treatment of sequelae of childhood disease like Perthes disease and slipped capital femoral epiphysis (SCFE), complex femoral osteotomy and limb deformity correction, repair of abductor and hamstring tendon injury, microsurgery and vascularized fibular grafting, surgical hip dislocation, and periacetabular (Ganz/ Bernese) osteotomy, among other advanced treatments.

Performing Periacetabular Osteotomy for Hip Preservation at Penn Medicine

Atul F. Kamath, MD
Director, Center for Hip Preservation
Assistant Professor of Orthopaedic Surgery
Consultants to the Penn Center for Hip Preservation

L. Scott Levin, MD, FACS
Paul B. Magnuson Professor of Bone and Joint Surgery
Chair, Department of Orthopaedic Surgery
Professor of Surgery, Division of Plastic Surgery

Charles L. Nelson, MD
Chief, Adult Reconstruction Section
Associate Professor of Orthopaedic Surgery

John D. Kelly, IV, MD
Associate Professor of Clinical Orthopaedic Surgery

J. Bruce Kneeland, MD
Section Chief, Musculoskeletal Imaging Division
Professor of Radiology

To hear Dr. Kamath discuss hip preservation for younger patients, visit the ReachMD(TM) series Medical Breakthroughs from Penn Medicine.

Tuesday, October 14, 2014

Sialendoscopic Management of Salivary Stones and other Salivary Duct Pathologies

Surgeons with Penn Otorhinolaryngology-Head and Neck Surgery are performing novel high-tech diagnostic and interventional sialendoscopy procedures to treat patients with diseases of the parotid and submandibular salivary glands.

At Penn, the primary objective for patients with sialolithiasis (stone disease) and inflammation of the salivary gland (sialadenitis) is to make a diagnosis, clear the duct and preserve the native salivary gland, if possible, and to achieve these ends using the safest, least invasive and most appropriate therapy.

In many cases, diagnosis is aided by radiography, usually after the onset of classic symptoms. Sialoliths of small to moderate size may be treated by sialendoscopy, a relatively recent innovation that is used at Penn Medicine for both diagnosis and treatment.

About Sialendoscopy
Sialendoscopy is a minimally invasive technique that has the potential to avoid nerve injury and the facial and oral scarring associated with traditional open surgery. The sialendoscope combines a delicate, semi-rigid (1.3 mm) fiber-optic endoscope, an irrigation port and a working channel in a single instrument. The endoscope broadcasts high definition images to a monitor. (See Fig 1).
Irrigation is used to dilate the ducts, permitting exploration of the branches of the salivary duct system. The working channel is the conduit for the instruments used to remove obstructions such as salivary duct stones, including custom-designed baskets, micro-burrs and guidewires.

The approach to larger salivary stones during sialoendoscopy sometimes employs hybrid treatments such as laser fragmentation. In these cases, otorhinolaryngologists at Penn collaborate with urologists, who use similar techniques to treat kidney stones. If accessible, larger stones can thus be broken up into smaller fragments, permitting them to be eliminated by irrigation or basket retrieval.
Multiple or deeply placed stones may require a combined approach or a more limited open approach where the sialendoscope is used to transilluminate the duct.

In addition to sialolithiasis, the indications for sialendoscopy at Penn Medicine include ductal injuries, duct stenoses, radioactive-iodine induced sialadenitis and autoimmune sialadenitis, including Sjogren’s Syndrome.

Case Study
JG, a 23-year-old male, came to Penn Medicine for suspected parotitis after experiencing repeated episodes of post-prandial facial swelling over a three month period. A CT scan at Penn found a 3mm density in JG’s left parotid duct deemed highly suspicious for a salivary stone (see Fig. 2).
After a consultation to review his treatment options, JG opted for sialendoscopy.

At the start of the procedure, the left parotid duct papilla was dilated to permit irrigation of the duct. A 1.3 mm scope was then advanced and navigated within the duct to the obstruction, a compact sialolith, lodged at a bifurcation distal to the parotid gland.

With further irrigation to dilate the duct, a six-wire basket was placed over a guide wire and extended until it grasped the stone. At this point, the stone was gently drawn beyond the bifurcation, but floated into the opposite duct. A micro-sialendoscopic burr was then introduced, freeing the stone, which was grasped by a 3-wire basket and extracted by rotating past the muscle to the papilla. JG’s recovery from surgery was unremarkable, and he was discharged the same day. At his one-year follow-up visit, there was no evidence of evolving sialoliths in the cleared duct or elsewhere.

Faculty Team
The faculty of Penn Otorhinolaryngology-Head and Neck Surgery are leaders in the field in patient care, surgical innovation and clinical and laboratory research. The Department logs more than 86,000 patient visits each year—the highest volume in the nation of any center or program performing otorhinolaryngology-head and neck surgery—and offers comprehensive and multidisciplinary programs to manage every disease or disorder affecting the organs and tissues of the nose, ears, throat, face and skull base.

Performing Sialendoscopic Procedures at Penn Medicine

Christopher H. Rassekh, MD, FACS
Associate Professor of Otorhinolaryngology-Head and Neck Surgery
Director, Penn Medicine Sialendoscopy Program

Erica R. Thaler, MD
Professor of Otorhinolaryngology-Head and Neck Surgery

Assisting with Laser Lithotripsy
Keith N. Van Arsdalen, MD
Director, Lithortriptor Center
Professor of Urology in Surgery

Key Team Members
Joshua H. Atkins, MD, PhD
Assistant Professor of Anesthesiology and Critical Care

Laurie A. Loevner, MD
Professor of Radiology

Rita Glenn-West, BSN, RN, CNOR
Coordinator, Operating Room Sialendoscopy Procedures

Tashara Nicholson
Clinical Administrative Assistant
Assistant to Dr. Rassekh in Sialendoscopy Clinic

Hospital of the University of Pennsylvania
5 Silverstein
3400 Spruce Street
Philadelphia, PA 19104

Sialendoscopy Clinical Briefing