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Grand Rounds from HSS: Management of Complex Cases

In Grand Rounds from HSS: Management of Complex Cases, HSS physicians detail the diagnosis and treatment of some of their most complicated cases. Each patient presentation includes relevant images, follow-up data, and a discussion of clinical decisions based on a review of the available evidence. We hope you find these cases to be of interest and the principles presented informative. Comments are always welcome at complexcases@hss.edu.

 

Current Issue: November 2022 | Volume 11, Issue 3 | Rheumatology Issue

Expecting the Unexpected in Rheumatology

Karmela Kim Chan, MD

It is my honor to present our annual rheumatology issue, 4 cases that highlight the vital importance of curiosity when a patient’s clinical course does not fit neatly into a physician’s expectations. They also illustrate how often rheumatologic care is a team endeavor, requiring multidisciplinary expertise.

In Case 1 from Jheel Pandya, MD, and Sarah Faith Taber, MD, a 4-year-old presents with a diagnosis of anti-synthetase syndrome, only for the diagnosis to be revised when an observant clinician notices that the child’s mother has the same unusual rash.

In Case 2 from Caroline H. Siegel, MD, MS, and Lisa R. Sammaritano, MD, a woman with recurrent amaurosis fugax and antiphospholipid antibodies, and prior late pregnancy loss, carries a pregnancy to term with anticoagulation and a tumor necrosis factor-alpha inhibitor.

In Case 3 from Jeong Min (April) Yu, MD, David Pisapia, MD, and Kyriakos A. Kirou, MD, DSc, FACP, a presumed diagnosis of neurosarcoidosis is upended when the patient does not respond to high doses of glucocorticoids.

Finally, in Case 4 from Robert Spandorfer, MD, a patient with longstanding myeloperoxidase-positive vasculitis with seemingly small and large vessel involvement presents with new acute kidney injury and a surprising finding on renal biopsy.

Cases in the Current Issue

Consultants

Andrew D. Schweitzer, MD
Associate Professor of Clinical Radiology
Weill Cornell Medicine

Yaxia Zhang, MD, PhD
Associate Attending Pathologist
Associate Professor of Pathology and Clinical Laboratory Medicine
Weill Cornell Medicine

 

Past Issues

August 2022 | Volume 11, Issue 2 | Orthopaedic Surgery Issue

Limb Lengthening: Innovation in Motion

David M. Dines, MD, and Austin T. Fragomen, MD

In this issue of Grand Rounds from HSS: Management of Complex Cases, we highlight the field of limb lengthening and complex reconstruction, presenting 3 cases involving application of the latest technologies in limb salvage and prosthetic replacement at HSS.

In Case 1, Taylor J. Reif, MD, and Austin T. Fragomen, MD, discuss limb reconstruction after bone tumor resection and several failed intercalary allograft surgeries in a 54-year-old man. They found that the patient had a femur nonunion with infection in the allograft bone and a 10-cm leg length discrepancy. His surgeons used a magnetic internal lengthening nail in compression mode to heal the nonunion and simultaneously lengthen the femur and tibia using the same implant.

In Case 2, Austin T. Fragomen, MD, and Stephen J. Wallace, MD, report on the care of a 57-year-old man with an infected, hypertrophic nonunion of the tibia and who, after many failed surgeries, was facing transtibial amputation. They highlight the use of circular external fixation with automatic adjusting struts to resolve this limb-threatening condition.

In Case 3, Jason S. Hoellwarth, MD, and S. Robert Rozbruch, MD, present a case involving traumatic above-knee amputation that left the 25-year-old patient with a very short residual femur and prosthetic socket difficulties. His surgeons implanted a prosthetic stem into his femur using osseointegration technology to provide superior function.

Click here for a variety of videos related to the procedures involved in the complex care of these patients.

Cases in the Current Issue

March 2022 | Volume 11, Issue 1 | Orthopaedic Surgery Issue

The Increasing Complexities of Pediatric Orthopaedics

David M. Dines, MD, and Roger F. Widmann, MD

In this issue of Grand Rounds from HSS: Management of Complex Cases, we present 3 fascinating cases that detail treatments for children with not uncommon yet complex conditions.

In Case 1, Danielle E. Chipman, BS, Nicolas Pascual-Leone, BA, and Daniel W. Green, MD, MS, FACS, present a complicated knee reconstruction for patellar instability in an 8-year-old boy. They outline an approach using several techniques, including medial patellofemoral ligament reconstruction with gracilis allograft, to stabilize the patella and prevent future dislocation.

In Case 2, Peter D. Fabricant, MD, MPH, Nicolas Pascual-Leone, BA, and Samuel A. Taylor, MD, present a multiplanar proximal humeral osteotomy and long head of biceps tendon transfer for a painful humerus deformity in a teenager resulting from physeal injury earlier in life. This procedure relieved pain and increased shoulder range of motion by correcting the proximal humeral deformity. Their success in this case demonstrates the promise of this technique for treating children with humerus deformity, whether secondary to congenital, idiopathic, traumatic, hematologic, or infectious conditions.

Finally, in Case 3, Tyler Uppstrom, MD, Arkady Blyakher, MD, and Roger F. Widmann, MD, present a procedure for treating a 10-year-old with tibial nonunion and multilevel deformity secondary to previous osteomyelitis. Through the use of staged distraction osteogenesis and osteotomy in a ring external fixator with a 6-axis correction system, the surgeons corrected the deformity while permitting early range of motion and weight bearing, with excellent results.

We hope the cases presented in this issue help you in treating patients with similarly challenging pediatrics conditions in your practice.

Cases in the Current Issue

November 2021 | Volume 10, Issue 2 | Rheumatology Issue

The Art and Science of Rheumatology

Karmela Kim Chan, MD

It is my honor to serve as editor of this issue of Grand Rounds from HSS: Management of Complex Cases focused on rheumatology. We at HSS take pride in our thoughtful and thorough approach to caring for patients, and we are excited to share with you lessons we have learned in 4 cases involving complex rheumatologic conditions.

One principle encountered in medical training is that when a diagnosis is elusive, consider the triad of pathologies of malignancy, infection, or rheumatic disease. The cases in this issue confirm that there is a good reason this heuristic endures.

Case 1 from Kevin Yip, MD, and Anne R. Bass, MD, suggests that in a patient with a previously treated hepatitis B virus infection, vaccination against SARS-CoV-2 may have activated the immune system in an unexpected way. Case 2 from Diane Zisa, MD, Roberto A. Garcia, MD, and Susan M. Goodman, MD, demonstrates the challenge of identifying new-onset inflammatory arthritis after total joint arthroplasty. Case 3 from David R. Fernandez, MD, PhD, details the diagnostic curveballs encountered in a patient with proximal muscle weakness, rash, and nailfold capillary changes. Finally, Case 4 from Lauren Robinson, MD, and Sarah Taber, MD, presents a 2-year-old boy with macrophage activation syndrome, whose underlying diagnosis of Kikuchi–Fujimoto disease was revealed several years later.

I hope you find the cases in this issue as stimulating as I did. These cases show the complexities and uncertainties that rheumatologists face routinely as we practice this art form we call medicine.

The Management of Glenohumeral Bone Loss in Shoulder Arthroplasty

Michael C. Fu, MD, MHS, and David M. Dines, MD

As the biomechanically complex link between the upper extremity and the rest of the body, the shoulder provides stability, range of motion, and strength for activities of daily living and recreation. Over the past several decades, with an ever more active aging population, the incidence of shoulder arthritis, and consequently of shoulder arthroplasty, has risen. This has naturally led to a large number of innovations in shoulder arthroplasty. When successful, shoulder arthroplasty consistently relieves pain, improves function, and increases patient satisfaction.

With the high volume of primary shoulder arthroplasty performed comes a corresponding rise in the number of complex cases, failures, and revisions. Remarkably, most shoulder replacements in the United States take place at low-volume centers. As the top hospital for orthopaedics in the country, HSS is a referral center for patients needing complex reconstructions and revision surgery. Accordingly, the HSS shoulder service possesses a wealth of experience in addressing difficult shoulder cases.

In this issue, we highlight 3 clinically challenging cases. In Case 1, Ryan C. Rauck, MD, and Lawrence V. Gulotta, MD, describe the use of a convertible metal-backed glenoid component with bone grafting in total shoulder arthroplasty (TSA) for glenohumeral osteoarthritis with significant glenoid deformity. In Case 2, Allen D. Nicholson, MD, and Theodore A. Blaine, MD, highlight the use of a custom 3D-printed titanium implant for glenoid reconstruction in reverse TSA for treating postinfectious glenohumeral arthritis. In Case 3, Ryan S. Selley, MD, and Samuel A. Taylor, MD, explain the use of an allograft–prosthetic composite in reverse TSA of the humeral component in a young patient with a history of proximal humerus malunion, multiple revisions, and a failed reverse TSA in the setting of massive proximal humeral bone loss.

Solving the Puzzles of Rheumatic Diseases

S. Louis Bridges Jr., MD, PhD

As we send this issue to press in October 2020, clinicians and patients at HSS have transitioned to a “new normal” after the disruptive emergence of COVID-19 earlier this year. During my first few weeks at HSS, I have been deeply impressed by the dedication of our faculty, staff, and trainees to safety amid this unprecedented challenge. Our rheumatology faculty and staff have inspired me by their commitment to improving the lives of patients affected by rheumatic diseases, as demonstrated by the cases presented in this issue.

In Case 1, Caroline H. Siegel, MD, and Doruk Erkan, MD, MPH, discuss the treatment of a man with pain and cyanosis of the digits caused by antiphospholipid syndrome. In Case 2, Diane Zisa, MD, and Kyriakos A. Kirou, MD, DSc, FACP, review the case of a woman with systemic lupus erythematosus whose nephritis and cutaneous vasculitis were refractory to treatment. In Case 3, Kimberly Showalter, MD, MSJessica K. Gordon, MD, MS, and Bella Mehta, MBBS, MS, present the case of a woman with Sjögren’s syndrome with rapidly progressive interstitial lung disease. And in Case 4, David R. Fernandez, MD, and Linda A. Russell, MD, discuss the progression of a 32-year-old woman’s dermatomyositis—a rash that resolved during pregnancy, only to recur postpartum, along with fatigue and weakness.

Innovative Options for Patients with Devastating Upper Extremity Injuries

Edward C. Jones, MD, MA

The HSS Center for Brachial Plexus and Traumatic Nerve Injury epitomizes the surgical expertise, collaboration, and multimodal techniques necessary to properly diagnose and treat very complex conditions. The 3 cases presented in this issue highlight teamwork as an essential aspect of ensuring good outcomes for patients with devastating injuries to the brachial plexus and upper extremity.

A meticulous diagnostic process begins with physical examination and the identification of subtle, elusive physical signs. These findings serve to direct a purposeful, multidisciplinary patient evaluation using sophisticated electrodiagnostic testing, nerve-specific high-resolution magnetic resonance imaging (MRI), and ultrasonography. Surgical planning involves collaboration among specialties and in some cases 2 surgical teams operating simultaneously, using multiple approaches.

All 3 cases were authored by Drake G. LeBrun, MD, MPH, Darryl B. Sneag, MD, Joseph H. Feinberg, MD, Ogonna K. Nwawka, MD, Steve K. Lee, MD, and Scott W. Wolfe, MD. In the first case, multiple surgical windows were used for neurolysis and nerve transfer for iatrogenic axillary nerve injury following the arthroscopic repair of a humeral avulsion of the glenohumeral ligament. In the second case, high-resolution MRI was used to pinpoint anterior interosseous fascicles of the median nerve and to guide surgeons in decompressing fascicular constrictions using micro–internal neurolysis to treat anterior interosseous nerve syndrome resulting from Parsonage–Turner syndrome. In the third case, 2 surgical teams used multiple approaches to achieve triple nerve transfer and sural nerve grafting to restore function to a patient with an extensive brachial plexus injury.

Diagnostic and Treatment Challenges in Rheumatology

Mary K. Crow, MD

This issue features descriptions of several fascinating and challenging patients successfully managed by our Hospital for Special Surgery rheumatologists. The cases highlight examples of the diagnostic and treatment challenges that our physicians address every day.

Case 1, presented by Halide Ozge Basaran, MD, Samir K. Trehan, MD, and Sarah F. Taber, MD, highlights the diagnosis of a 15-year-old boy with pachydermodactyly, a rare form of fibromatosis that can result from minor mechanical trauma to the fingers.

In Case 2, Nilasha Ghosh, MD, and Anne R. Bass, MD, describe the successful treatment of an 83-year-old woman who had checkpoint inhibitor–associated myositis and myocarditis, the result of her prior immunotherapy with nivolumab.

In Case 3, Pantelis P. Pavlakis, MD, PhD, and David R. Fernandez, MD, PhD, discuss a 56-year-old man whose neuropathy associated with trisulfated heparin disaccharide responded well to IV immunoglobulin treatment.

Case 4, presented by Kimberly Showalter, MD, Xiaoping Wu, MD, MS, and Jessica K. Gordon, MD, MS, features a previously healthy 36-year-old man with progressive shortness of breath and dry cough ultimately diagnosed with antisynthetase syndrome responsive to monthly IV cyclophosphamide for 6 months.

From the Editor

Edward C. Jones, MD, MA

The foot and ankle, our weight-bearing pedestal, are also one of the most common sources of functional limitation, intractable pain, and progressive disability. The congruous interaction of so many essential structures in the foot and ankle provides balanced support for a range of activities, from walking to running to vigorous sports—remarkable, when you consider the foot and ankle may repeatedly bear up to five times body weight while walking and up to 13 times body weight during activities such as running.

We rarely give this much thought until things begin to go wrong. Often, when a structure is injured or undergoes progressive wear, balanced function is disrupted, and other co-dependent structures may deteriorate. Foot and ankle specialists are quite innovative, treating a broad range of patients at all stages of life, from newborns with foot deformity to active teens, adults, and seniors.

As the cases presented in this volume demonstrate, foot and ankle specialists are often confronted with complex conditions that include articular cartilage wear, bone fracture and deformity, ligamentous incompetence, and tendon rupture or insufficiency. HSS surgeons are prominently involved in research that leads to new techniques that improve treatment outcomes for these conditions.

In this issue, we highlight how HSS foot and ankle surgeons have expertly evaluated and corrected anatomical deficiencies in managing three very challenging conditions.

In Case 1, Scott J. Ellis, MD, Lauren E. Roberts, MD, and Aoife MacMahon, BA, describe reconstruction of complex flatfoot deformity with a dorsal bunion. In Case 2, Mark C. Drakos, MD, and James Davies, MD, explain the innovative soft tissue procedures required to repair a large Achilles tendon defect using quadruple-bundle hamstring autograft with flexor hallucis longus transfer. In Case 3, Constantine Demetracopoulos, MD, and Amelia Hummel, BA, describe a two-stage procedure to carry out a complex total ankle arthroplasty in a patient with flatfoot deformity and deltoid insufficiency.

From the Director

Thomas P. Sculco, MD

The Stavros Niarchos Foundation Complex Joint Reconstruction Center (SNF CJRC) was created to provide specialized care to patients with the most difficult joint problems, primarily in failed joint replacements. The SNF CJRC has an active educational component, with a clinical and research fellowship, and research at the SNF CJRC has been awarded over $225,000 in grants this year. In addition, more than 1200 patients have been entered into a prospective registry that will provide outcomes data to improve techniques for the treatment of patients with these complex conditions. The SNF CJRC will host two international symposia in 2019, one on acetabular bone loss in revision total hip replacement and another on the difficult challenge of bacterial biofilms in the treatment of periprosthetic joint infection.

In this issue, 3 cases are presented involving revision total knee arthroplasty (TKA). Many of our patients have undergone revision TKA elsewhere, presenting to us with often debilitating complications such as chronic periprosthetic joint infection, soft-tissue defects, bone loss, and damaged implants. In Case 1, authors Shawn S. Richardson, MD, and Thomas P. Sculco, MD, discuss a severe adverse outcome of TKA, chronic extensor mechanism disruption with global knee instability, which required an extensor allograft and hinge prosthesis to improve patient function and restore knee extensor power. In Case 2, authors Jason L. Blevins, MD, Victoria Tam, and Michael B. Cross, MD, highlight the successful treatment of a patient with long-term periprosthetic joint infection who had severely compromised bone and soft tissue. In Case 3, authors Ivan De Martino, MD, Michael-Alexander Malahias, MD, PhD, and Peter K. Sculco, MD, describe the complex treatment course for a patient with massive metaphyseal bone deficits requiring prosthetic and bone augmentation.

All three cases illustrate the need for a multidisciplinary approach to management of complex cases—including collaboration among experts in infectious disease, specialized imaging, biomechanics, and surgical treatment. The SNF CJRC is committed to caring for patients with these most challenging conditions and strives through clinical and basic science research to provide the most innovative and evidence-based treatment to alleviate the major dysfunction caused by these failures.

From the Editor

Mary K. Crow, MD

In this issue, we feature four complex cases demonstrating the varied diagnostic and treatment challenges that rheumatologic disorders can present.

In Case 1, Jonathan T.L. Cheah, MBBS, and Theodore R. Fields, MD, FACP, discuss a woman in whom hypergammaglobulinemic purpura of Waldenström was diagnosed based on a recurrent rash, a history of hypergammaglobulinemia, and positive results for several autoantibodies.

In Case 2, Kimberly Showalter, MD, and Anne R. Bass, MD, present the case of a man who developed immune-related adverse events—inflammation involving the prostate, parotid, and lacrimal glands, the liver, and the joints—after receiving immunotherapy as treatment for metastatic renal cell carcinoma.

In Case 3, Sarah B. Lieber, MD, and Michael D. Lockshin, MD, describe the dangers to both mother and fetus of escalating hypertension during pregnancy. They discuss a 21-year-old pregnant woman with systemic lupus erythematosus who in her 23rd week of gestation developed preeclampsia.

In Case 4, Sebastian E. Sattui Cortes, MD, and Steven K. Magid, MD, describe a patient with finger swelling and a persistent lower-extremity rash. He was eventually diagnosed with pancreatitis, panniculitis, and polyarthritis (PPP) syndrome, a rare condition that can accompany pancreatic cancer.

Perioperative Multimodal Anesthesia in High-Risk Patients

Edward C. Jones, MD, MA

Anesthesiologists at Hospital for Special Surgery carry on a long tradition of innovation in musculoskeletal care, clinical research, and practice standards that dramatically improves surgical outcomes. They have expanded the use of regional anesthesia and demonstrated the benefits of a multimodal approach to manage pain, reduce perioperative stress, and minimize postoperative complications. And they are leading efforts to address the opioid crisis. For instance, they have implemented strategies combining extended sensory nerve blocks and nonopioid pain medications that have significantly reduced the overuse of dependency-inducing drugs.

An anesthesiologist’s clear understanding of the surgical procedure and thorough preoperative evaluation of the patient’s general health are essential. Managing complex comorbidities is a crucial element in devising the best anesthesia plan for each patient. Along with administering optimal regional anesthesia, making use of a variety of multimodal approaches by anesthesia and pain-management specialists provides prolonged postoperative pain control and enables early mobilization. These techniques have resulted in minimizing postoperative opioid use and reducing the need for extended inpatient care, while improving patient satisfaction and surgical outcomes.

We present 3 cases involving patients with challenging comorbidities: hip fracture repair in a patient with Parkinson’s disease, shoulder tendon surgery in a patient with myasthenia gravis, and total knee replacement in a patient with a history of alcohol use disorder. The authors—Bradley Lee, MD, and David Kim, MD, in Case 1; Patricia Pang, MD, and Jonathan C. Beathe, MD, in Case 2; and Michael Singleton, MD, and Jiabin Liu, MD, PhD, in Case 3—demonstrate that the most complex cases often require the greatest innovation in pain management. Special thanks to Dr. Beathe for his editorial oversight with the cases for this issue.

From the Director

Thomas P. Sculco, MD

The Complex Joint Reconstruction Center (CJRC) was established at Hospital for Special Surgery in January 2017 to treat the most challenging cases in joint reconstruction. Since then, over 500 patients have been treated, with multidisciplinary input from expert revision joint-replacement surgeons and specialists from imaging, biomechanics, infectious disease, pathology, and basic science. Currently, 13 member surgeons have a dedicated clinical and research interest in this complex area of arthroplasty, and a registry has been created to closely monitor patient outcomes. Data from the registry are helping us determine mechanisms of failure and possible preventive strategies based on basic science and biomechanical research. Prospective outcome analysis will also aid us in improving algorithmic approaches to complex joint disorders.

The cases presented in this issue demonstrate 3 of the most challenging problems of acetabular bone loss, with solutions that ensure hip stability and proper implant selection. The authors—Christopher Jones, MD, PhD, and Peter K. Sculco, MD, in Case 1; Jason L. Blevins, MD, and Alexander S. McLawhorn, MD, MBA, in Case 2; and Colin Y. L. Woon, MD, Peter H. Sun, MS, and Michael B. Cross, MD, in Case 3—have clearly defined the need for thorough evaluation of anatomical deficiencies through the use of advanced imaging and 3-dimensional modeling, which provide the key to preparation for surgery. Biomechanical consultation is also crucial in aiding anatomic restoration through the use of augmentation, bone grafting, and customized implants.

These authors are experts in the treatment of such complex cases, including the potentially catastrophic complications that can arise during their management. Together, these 3 cases reinforce the need for a focused center such as the Complex Joint Reconstruction Center.

Issues prior to 2018 can be requested by emailing complexcases@hss.edu.
 

HSS Editorial Board

Editors-in-Chief
David M. Dines, MD (Orthopaedics, 2021–present)
Karmela Kim Chan, MD (Rheumatology, 2021–present)
Edward C. Jones, MD, MA (Orthopaedics, 2010–2020)
Mary K. Crow, MD (Rheumatology, 2010–2020)

Image Review
Carolyn M. Sofka, MD (Radiology, 2010–present)
Eric Bogner, MD (Radiology, 2021–present)
Helene Pavlov, MD (Radiology, 2010–2020)

HSS Education Institute
Marcia Ennis (2010–present)
Joy Jacobson (2017–present)
Laura Robbins, DSW (2010–present)

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