History Of Connective Tissue Disorders Research Articles

Naproxen-induced liver injury in a case of adult-onset Still's disease.

We present the case of a young female with fever, rash, and joint pain without any other history of connective tissue disorder, who was found on evaluation to have neutrophilic leucocytosis, elevated inflammatory markers and lymphadenopathy with hepatosplenomegaly. After extensive workup, the possibility of adult onset Still's disease was suggested by Yamaguchi criteria. She was started on naproxen, but later on developed drug-induced liver injury. She already had central serous retinopathy due to steroid use, and it was difficult to treat the adult-onset Stills disease due to the liver injury in view of contraindication to use of anti-inflammatory drugs but her illness was monocyclic and did not require further immunosuppression.

Bilateral Peripheral Ulcerative Keratitis Associated with Dupilumab

A 57-year-old woman without a history of connective tissue disorders started dupilumab (initial dose 600 mg) for atopic dermatitis. Ocular history was noncontributory, and the patient had undergone a normal eye examination recently. Ten days later, she developed bilateral ocular pain. Slit-lamp biomicroscopy revealed conjunctival injection and extensive peripheral corneal infiltrate bilaterally (Fig A, right eye; Fig B, left eye) with focal areas of ulceration (inset) consistent with peripheral ulcerative keratitis in both eyes. Dupilumab was discontinued. Frequent preservative-free tears, and later, prednisolone 1.0% four times daily were initiated. Peripheral ulcerative keratitis resolved after 3 weeks (Fig C, right eye; Fig D, left eye). Conjunctivitis occurs commonly in patients receiving dupilumab for atopic dermatitis; however, reports of an association with corneal ulceration are exceedingly rare (Magnified version of Fig A-D is available online at www.aaojournal.org).

Criteria for endovascular intervention in type B aortic dissection.

The use of thoracic endovascular aortic repair (TEVAR) for the management of uncomplicated type B aortic dissection (un-TBAD) remains controversial. There is a lack of consensus over whether pre-emptive TEVAR should be carried out in patients with un-TBAD at risk of progression to complicated TBAD. We present a review of current evidence and seek to suggest criteria where endovascular intervention in un-TBAD may prove beneficial relative to pharmacotherapy alone. PubMed and Cochrane databases were searched using terms including: type B aortic dissection, risk factors, medical therapy, TEVAR, false lumen (FL) expansion, and mortality. Papers were selected based on title and abstract. Optimal medical therapy remains the mainstay treatment for patients with un-TBAD, however, patients with un-TBAD present with varying degrees of disease progression risk. Factors such as age, aortic morphology, history of connective tissue disorders, FL thrombosis, and aortic branch involvement may potentiate progression from un-TBAD to complicated TBAD. Short- and long-term outcomes associated with TEVAR for TBAD remain promising. Pre-emptive TEVAR may be beneficial in patients with un-TBAD presenting with the above factors, however, further prospective research into the optimal timing for TEVAR in un-TBAD is required.

Post-COVID isolated subclavian artery dissection with multiple cerebral infarctions

BackgroundCoronavirus disease 2019 (COVID-19) is a pandemic disease which predominantly affects the respiratory system with high critical care mortality and morbidity, yet it also causes multiple organs dysfunction in affected patients. There is a strong evidence that it increases the susceptibility of cerebrovascular strokes in such patients. Besides this prothrombotic complication, arterial dissection can be one of its mechanisms increasing the risks of stroke.Case presentationHerein, we report a case of spontaneous isolated subclavian artery dissection in a COVID-19 patient. Sixty-one-year-old female presented with spontaneous isolated subclavian artery dissection without any traumatic events nor history of connective tissue disorders. She had left upper limb ischemia followed by cerebellar, thalamic and occipital infarctions. Whether this patient’s subclavian artery dissection was triggered by exaggerated inflammatory response or arteriopathy secondary to COVID-19 remains speculative.ConclusionsNonetheless, arterial dissection can be one of its complications, it is essential for treating physicians to be attentive for the diversity of COVID-19 clinical manifestations.

Ex vivo biaxial load testing analysis of aortic biomechanics demonstrates variation in elastic energy distribution across the aortic zone zero

ObjectivesWe hypothesized that tissue characteristics vary significantly along zone zero, which may be reflected by regional differences in stored elastic energy. Our objectives were to (1) characterize the regional variation in stored elastic energy within tissues of the aortic zone zero and (2) identify the association between this variation and patient characteristics. MethodsFrom February 2018 to January 2021, 123 aortic tissue samples were obtained from the aortic root and proximal and distal ascending aortas of 65 adults undergoing elective ascending aorta replacement. Biaxial biomechanics testing was performed to obtain tissue elastic energy at the inflection point and compared with patient demographics and preoperative computed tomography imaging. Coefficient models were fit using B-spline to interrogate the relationship among elastic energy, region, and patient characteristics. ResultsMean elastic energy at inflection point was 24.3 ± 15.6 kJ/m3. Elastic energy increased significantly between the root and proximal, and root and distal ascending aorta and decreased with increasing age. Differences due to history of connective tissue disorder and bicuspid aortic valve were significant but diminished when controlled for other patient characteristics. Among covariates, age and region were found to be the most important predictors for elastic energy. ConclusionsAortic tissue biomechanical metrics varied across regions and with patient characteristics within the aortic zone zero. Assessment of endovascular outcomes in the ascending aorta must closely consider the region of deployment and variable tissue qualities along the length of the landing zone. Regional variation in tissue characteristics should be incorporated into existing patient-specific models of aortic mechanics.

Maternal risk factors for paediatric inguinal hernia.

Risk factors for paediatric inguinal hernia are poorly understood. This longitudinal cohort study assessed whether children with a maternal history of inguinal hernia or connective tissue disorders have a higher risk of developing inguinal hernias before 13 years of age. The study included children followed up between birth and 13 years of age in Quebec, Canada, 2006-2019. Newborns whose mothers had inguinal hernias or connective tissue disorders were followed over time to identify future hospital admissions for inguinal hernia. Cox proportional hazards regression adjusted for patient characteristics was used to estimate hazard ratios (HRs) and 95 per cent confidence intervals for the association between maternal hernia or connective tissue disorders and future childhood hernias. Associations in girls and boys were examined separately. The study included 786 322 children with 6 186 448 person-years of follow-up. There were 6861 children with inguinal hernias, corresponding to an incidence of 11.1 per 10 000 person-years. Children with a maternal history of inguinal hernia had 2.92 (95 per cent c.i. 2.39 to 3.58) times the risk of having inguinal hernias relative to children whose mothers had no such history. Children with a maternal history of connective tissue disorders had 1.30 (1.00 to 1.68) times the risk. Maternal hernias were strongly associated with risk of inguinal hernias in girls (HR 5.34, 3.82 to 7.47), whereas maternal connective tissue disorders were associated with inguinal hernias in boys (HR 1.35, 1.02 to 1.79). Paediatric inguinal hernias may be associated with maternal inguinal hernias and connective tissue disorders, but the underlying reason for this relationship requires further investigation.

P5606Incidence and clinical impact of type A aortic dissection detected during invasive coronary procedures. Results from an international, multicentre, all-comer registry

Abstract Background Type A aortic dissection (AD) is an extremely severe condition with high mortality. Its clinical presentation may mimic other conditions such as myocardial ischemia. Furthermore, coronary hypoperfusion may occur. This may result in misdiagnosis and subsequent referral to the cath-lab, implying potential catastrophic consequences. Purpose To determine the incidence and clinical impact of type A AD detected at the cath-lab in all-comers referred for invasive coronary procedures (both diagnostic and therapeutic). Methods and results Data was obtained from three high-volume centers from different countries. 41.186 procedures performed in all-comers between 2011–2018 were analyzed, of which 20.067 (49%) were PCIs. Iatrogenic AD resulting from the procedure were excluded. In 17 patients (0.002%; age 75±11 years, 72% male) a type A AD was detected during the procedure. The diagnosis was established by aortic angiography in 15 (88%); in 2 (12%) the diagnosis was suspected based on difficult coronary cannulation or the presence of a fluoroscopically-visible fluttering flap, requiring confirmation with aortic imaging tests. The procedure was performed in a emergent fashion in 14 patients (89%): due to suspected acute coronary syndrome in 11 (65%) (8 [72%] STEMI and 3 [18%] NSTEMI) and because of out of hospital cardiac arrest in 4 (24%). Loading dose of antiplatelets (aspirin 76%, P2Y12 inhibitors 100%) and heparin (100%) were administered in the majority of patients. Either the left main or right coronary artery ostia were involved in 8 patients (47%), although in a significant number of cases coronary anatomy was not fully evaluated due to problematic cannulation of the coronary arteries (left main 18%, right coronary artery 30%). Previous history of aortic root dilatation was present in 5 cases (30%). None of the patients had previous history of connective tissue disorders or bicuspid aortic valve. CT scan was feasible in 9 cases (59%). A DeBakey type I AD was observed in 8 patients (47%) and a DeBakey type II in 9 cases (53%). Overall in-hospital mortality rate was 59% (10 patients). 3 patients (30%) died during the procedure. 10 (59%) underwent surgical treatment (4 of them with concomitant aortic valve replacement). Perioperative mortality rate was 30% (1 due to hemorrhagic stroke, 1 due to massive bleeding during surgery; 1 due to cardiac tamponade). All patients who were not candidates for surgery (7 cases, 41%) died during admission. Patients successfully discharged presented a median survival of 17 months (4–78). Conclusions The detection of a type A AD during invasive coronary procedures is rare and is predominantly observed in patients referred for urgent interventions. The overall in-hospital mortality is very high (59%) and therefore prompt identification by the operator is mandatory. Surgery, when feasible, is the treatment of choice, although perioperative mortality rate is high, mostly due to bleeding complications. Acknowledgement/Funding None

01: Minimally invasive sacrocolpopexy versus vaginal uterosacral ligament suspension: Comparing anatomic outcomes at 1 year

Our aim was to compare anatomic outcomes 1-year after surgery between patients who underwent minimally invasive sacrocolopopexy (MISC) (laparoscopic or robotic) with synthetic mesh to those who underwent vaginal uterosacral suspension (USLS) for management of apical prolapse. Secondary outcomes included comparing intra and postoperative complications. This was a multi-center, retrospective cohort study through the Fellows Pelvic Research Network. Patients with prolapse who underwent apical suspension with USLS or MISC from January 2013 to January 2016 with at least 1-year postoperative anatomic data were included. Patients with prior apical repairs, prior vaginal repair with biologic/synthetic graft, or history of connective tissue disorders were excluded. Eligible patients were identified using current procedural terminology codes. Relevant pre, intra, and postoperative data were abstracted from medical records. Prolapse recurrence beyond the hymen and/or POPQ stage 2 or higher were identified at 1-year postoperatively. Categorical data were compared using chi-square and Fisher’s exact tests, and continuous data were compared using the Wilcoxon rank sum test. During the study period, 311 patients underwent MISC (159 laparoscopic and 152 robotic) and 129 patients underwent USLS. They all had 1-year POPQ data available. The proportion of patients in MISC and USLS group were similar with respect to stage of prolapse (47.3% vs. 44.2% with stage 2 and 47.9% vs. 46.5% with stage 3). The median (interquartile range) preoperative apical point C was higher in the MISC group at -2.5 (-4.0, 1.0) than in the USLS group at 0.0 (-4.0, 2.5). The anterior and posterior walls were similar: Aa/Ba at 1.0 (0.0, 2.0) and Ap/Bp at -1.0 (-2.0, 0.0). Intra and perioperative complications such as blood loss (P < 0.0001), conversion to laparotomy (P = 0.002), urinary dysfunction (P = 0.02), and fever (P = 0.007) were higher in the USLS group, but operative time was longer in MISC group (P = 0.002). Three patients in the MISC group had mesh erosion and 5 patients in the USLS group had suture erosions. Postoperative pelvic pain, dyspareunia, voiding and bowel dysfunction were comparable in the two groups. At 1-year postoperatively, 50 patients (38.8%) in the USLS group had apical descent beyond the hymen compared to 77 (24.8%) patients in the MISC group (P = 0.003). Recurrence in the anterior compartment beyond the hymen was also higher in the USLS versus MISC group: 29 (22.5%) vs 19 (6.1%) patients (P < 0.0001), respectively. Posterior compartment recurrences were comparable: 5 (3.9%) versus 16 (5.1%) patients in the USLS versus MISC group (P = 0.57), respectively. At 1-year postoperatively, stage 2 prolapse or greater were noted in 69 (45.7%) patients in the USLS group versus 108 (34.7%) patients in the MISC group. While MISC had longer operative times and risk of mesh erosion, anterior and apical compartment prolapse recurrence rates were lower at 1 year compared to USLS. Future studies comparing bothersome bulge symptoms and need for repeat surgery or pessary placement are currently underway to better assess both symptomatic and anatomic failure within 1-year from initial prolapse surgery.

Abstract CT227: MC1273: Phase II evaluation of aggressive dose de-escalation for adjuvant chemoradiation in HPV associated oropharynx cancer

Abstract Background: Traditional adjuvant therapy for oropharyngeal squamous cell carcinoma (OPSCC) consists of 60-66 Gy of radiation therapy (XRT) given in 2 Gy daily fractions along with high dose cisplatin if the patient has high risk factors. Despite the excellent cure rates for HPV+ OPSCC, one in three patients treated with conventional treatment will develop grade &amp;gt;3 long-term sequelae from therapy. There is intense interest in de-intensifying adjuvant therapy for this patient population in order to maximize quality of life while maintaining excellent historical rates of disease control. Methods: MC1273 is a phase II non-randomized trial open at Mayo Clinic Rochester testing a novel course of aggressive therapy de-escalation following surgery for HPV+ OPSCC. The primary endpoint is local/regional control at 2 years while secondary endpoints include toxicity and quality of life (QOL). The eligibility criteria include all patients with p16-positive OPSCC with less than a ten pack-year smoking history who have had a complete surgical resection. Exclusion criteria include positive surgical margins, prior history of malignancy, and history of connective tissue disorders. Patients are divided into two prospective cohorts depending upon risk factors found at surgery. Patients with intermediate risk disease (≥T3, ≥N2, lymphovascular invasion, or perineural invasion) are enrolled in MC1273A while patients with extracapsular extension (ECE) are enrolled in MC1273B. Patients on MC1273A receive 30 Gy of radiation delivered in 1.5 Gy twice-daily fractions over the course of two weeks along with weekly docetaxel (15 mg/m2) given on day 1 and day 8. Patients on MC1273B receive a similar treatment regimen but also have the nodal level with positive ECE concurrently boosted to 36 Gy in 1.8 Gy twice-daily fractions. In addition to standard of care follow-up, patients receive a swallowing assessment with speech therapy immediately before XRT, one month post-XRT, and one year post-XRT. Patients also have QOL assessment consisting of the XeQOLS, Eq-5D, FACT H&amp;N (Vers 4) and Dermatology Life Quality Index assessed at pre-XRT and 3, 12, and 24 months post-XRT. Results: Each cohort of MC1273 is powered to detect a 10% local/regional failure rate with 85% confidence. Each cohort will accrue 35 evaluable patients and 5 additional patients to account for ineligibilities and violations (40 patients total per cohort.) MC1273A began accrual in September 2013. The first five patients were monitored for grade ≥4 acute toxicities before proceeding to open accrual. MC1273B began accrual in May 2014 and has also proceeded to open accrual. Accrual will also begin in Mayo Clinic Scottsdale in the first quarter of 2015. Conclusions: MC1273 is meeting its accrual targets and should finish accrual by 2016. We anticipate that preliminary results for toxicity will be available by 2017 and local/regional data will be available by 2018. Citation Format: Daniel J. Ma, Katharine A. Price, Eric J. Moore, Joaquin J. Garcia, Scott H. Okuno, Daniel L. Price, Jeff A. Sloan, Nathan R. Foster, Robert L. Foote. MC1273: Phase II evaluation of aggressive dose de-escalation for adjuvant chemoradiation in HPV associated oropharynx cancer. [abstract]. In: Proceedings of the 106th Annual Meeting of the American Association for Cancer Research; 2015 Apr 18-22; Philadelphia, PA. Philadelphia (PA): AACR; Cancer Res 2015;75(15 Suppl):Abstract nr CT227. doi:10.1158/1538-7445.AM2015-CT227

Supraglottic Obstruction in an Adult with Inspiratory Arytenoid Cartilage Prolapse

Laryngomalacia (LM) has become the default diagnostic term for any patient who might struggle with stridor and dyspnea secondary to floppy, insufficient, hypotonic, or passively collapsing supraglottal tissue characteristics. Despite the increasing incidence of this diagnosis, the etiology, work-up, and pathophysiology of LM remain poorly understood and controversial to date. Objectives: The purpose of this paper is to illustrate the history, unique physical examination findings, and multivaried and creative treatments rendered to an adult patient of ours who struggled with profound signs and symptoms of Type 1 LM. Methods: This is a case study of idiopathic aerodynamic supraglottic collapse that occurred in an otherwise healthy individual who had no prior history of connective tissue disorder, neurodegenerative disorder, or trauma. The patient’s history, examination, treatment, and review of the literature are discussed. Results: Laryngoscopy is the definitive test to evaluate supraglottic airway collapse. Supraglottoplasty is a safe and effective treatment for adult LM. Conclusions: The diagnosis of adult aerodynamic supraglottic collapse is an important entity to consider in patients presenting with dyspnea, stridor, or other breathing complaints. This case description illustrates the importance of laryngoscopy when evaluating patients with such signs and symptoms.

Spontaneous Acute Aortic Dissection in Pregnancy

Acute aortic dissection in pregnancy is a rare condition with a potentially catastrophic outcome. The low rate of incidence together with variable presenting features in such circumstances makes diagnosis elusive. A high degree of clinical suspicion and diagnostic acumen is especially required in pregnant women without any history of connective tissue disorder, congenital aortic valvular malformation, or chronic hypertension. In this report, we present a case of aortic dissection with multiple aortic intimal tears in a medicolegal autopsy of a young pregnant woman who developed circulatory collapse and died during labor.

α1-Antitrypsin Deficiency in Fraternal Twins Born With Familial Spontaneous Pneumothorax

We report a case of spontaneous familial pneumothorax in fraternal twin boys. The twins' family history is remarkable for reactive airway disease and a female sibling also born with spontaneous pneumothorax. The family had no history of connective tissue disorders, renal cancer, or dermatologic diseases. Analysis of the twins' α(1)-antitrypsin (AAT) genotype, phenotype, and serum concentration revealed that both were compound heterozygous for rare SERPINA1 alleles. These findings suggest a role for AAT deficiency in spontaneous pneumothorax of the newborn. To our knowledge, these are the first genetic data to support etiology of neonatal spontaneous familial pneumothorax.

Quantitative assessment of the entire thoracic aorta from magnetic resonance images

Although magnetic resonance imaging is a primary modality for following patients with connective tissue diseases, only a limited amount of the image data is utilised. The purpose of this study was to show the clinical applicability of an automated four-dimensional analysis method of magnetic resonance images of the aorta and develop normative data for the cross-sectional area of the entire thoracic aorta. Magnetic resonance imaging was obtained serially over 3 years from 32 healthy individuals and 24 patients with aortopathy and a personal or family history of connective tissue disorder. Graph theory-based segmentation was used to determine the cross-sectional area for the thoracic aorta. Healthy individual data were used to construct a nomogram representing the maximum cross-sectional area 5th-95th percentile along the entire thoracic aorta. Aortic root diameters calculated from the cross-sectional area were compared to measured diameters from echocardiographic data. The cross-sectional area of the entire thoracic aorta in patients was compared to healthy individuals. Calculated aortic root diameters correlated with measured diameters from echo data - correlation coefficient was 0.74-0.87. The cross-sectional area in patients was significantly greater in the aortic root, ascending aorta, and descending aorta compared to healthy individuals. The presentation of the dimensional data for the entire thoracic aorta shows an important clinical tool for following patients with connective tissue disorders and aortopathy.

Anatomical repair of a congenital aneurysm of the distal abdominal aorta in a newborn

Congenital (primary) neonatal abdominal aortic aneurysm (AAA) is an extremely rare truncular arterial abnormality among numerous congenital vascular malformations. Only seven cases have been reported as congenital origin in newborns. This report presents the case of a male infant in whom a 33-mm congenital AAA was diagnosed prenatally and was successfully treated 10 days after birth without exogenous graft material or aneurysmorrhaphy. Follow-up study at 39 months demonstrated excellent clinical, ultrasound scan, and computed tomography scan findings. Anatomic reconstruction with native vessels is the preferred surgical technique to ensure the child's potential for harmonious growth.

Dissecting aneurysm of the thoracic posterior spinal artery

rapidly by complete tetraplegia. On presentation he had no lowerextremity motor function and a T-11 sensory level (American Spinal Injury Association [ASIA] Grade A; ASIA motor score 50). A small bowel obstruction had been diagnosed 1 week prior to presentation, and he had undergone a laparotomy and lysis of adhesions for similar pain; there had been no other features of a bowel obstruction. The patient’s medical history was significant for hypertension. His surgical history was significant for having undergone an open cholecystectomy for cholecystitis 15 years prior to the current event. There was no history of connective-tissue disorders in the patient or his family. Spinal magnetic resonance (MR) imaging demonstrated a subdural and intramedullary hemorrhage in the lower thoracic spine (Fig. 1A). Spinal angiography revealed an aneurysm arising from the posterior spinal artery at T-12 (Fig. 1B). Surgical exploration confirmed the subdural hematoma and an area of hemorrhage within the left posterior aspect of the spinal cord, and the fusiform aneurysm was identified in the hemorrhage cavity (Fig. 1C). The aneurysm was resected. Histopathological analysis revealed a disrupted elastic lamina and organized thrombus in the media and adventitia, consistent with an arterial dissection (Fig. 2). At the 2-month follow-up examination the patient had minimal improvement in function—specifically, only subjective improvements in sensation and no change in sensory level, motor score, or ASIA grade. Intraspinal aneurysms are rare lesions and have been identified in association with arteriovenous malformations and vessels collateralizing around a thrombosed vessel,1–3 suggesting that they may form as a result of high-flow states in the vessel of origin. The most common vessel of origin is the anterior spinal artery, and the most common region of involvement is the cervicomedullary region.1,4 This case is unique in that the aneurysm arose from the thoracic posterior spinal artery and it was secondary to a dissection. Several features suggest a dissection as the cause. These include the history of hypertension, at least two episodes of acute severe abdominal pain, the fusiform nature of the aneurysm, and the histological features of the lesion.

Usual Interstitial Pneumonia Complicating Dyskeratosis Congenita

Usual Interstitial Pneumonia Complicating Dyskeratosis Congenita

Shoulder Instability - Collegiate Diver

HISTORY The patient was an 18 year old collegiate diver who had insidious onset of shoulder pain during the fall of his freshman year. This pain was associated with swimming and not with diving. One day in practice he was diving and when he hit the water and his shoulder sustained an axial load, driving his shoulder posteriorly. He felt his shoulder slide out of the socket and spontaneously reduce. He stopped diving that day but could not return despite a month's rest. He continued to have shoulder pain with activity but no sense of instability. He did not have parasthesias, neck pain or weakness. He was in good health with no history of connective tissue disorders. PHYSICAL EXAMINATION He had no atrophy or deformity of his shoulders. He had full range of motion but had a painful are. His strength was normal except pain with resisted external rotation with his arm at the side. He had a positive Neer and Hawkins impingement sign. He had pain with posterior apprehension testing which he reported to be deep in his posterior shoulder. He had a grade 2 sulcus sign which did not reproduce his symptoms. His posterior drawer revealed a posterior subluxation which produced pain only. He had a positive active compression test. He was otherwise entirely neurologically intact for sensation, motor and reflexes. DIFFERENTIAL DIAGNOSIS Posterior subluxation Superior labrum (SLAP) lesion Rotator cuff tear Rotator cuff tendonitis Strain of posterior capsule of shoulder TEST AND RESULTS Plain radiographs (AP in internal and external rotation, axillary views): reverse bony Bankart lesion MRI shoulder: Reverse Bankart lesion FINAL WORKING DIAGNOSIS Posterior traumatic instability shoulder TREATMENT AND OUTCOMES Patient had failed non-operative rehabilitation for 5 months Diagnostic arthroscopy revealed posterior labrum detachment (reverse Bankart) Open posterior capsular shift with posterior bony Bankart External rotation brace for 6 weeks Progressive rehabilitation program Return to diving over 9 months

Shoulder Instability - Collegiate Diver

HISTORY The patient was an 18 year old collegiate diver who had insidious onset of shoulder pain during the fall of his freshman year. This pain was associated with swimming and not with diving. One day in practice he was diving and when he hit the water and his shoulder sustained an axial load, driving his shoulder posteriorly. He felt his shoulder slide out of the socket and spontaneously reduce. He stopped diving that day but could not return despite a month's rest. He continued to have shoulder pain with activity but no sense of instability. He did not have parasthesias, neck pain or weakness. He was in good health with no history of connective tissue disorders. PHYSICAL EXAMINATION He had no atrophy or deformity of his shoulders. He had full range of motion but had a painful are. His strength was normal except pain with resisted external rotation with his arm at the side. He had a positive Neer and Hawkins impingement sign. He had pain with posterior apprehension testing which he reported to be deep in his posterior shoulder. He had a grade 2 sulcus sign which did not reproduce his symptoms. His posterior drawer revealed a posterior subluxation which produced pain only. He had a positive active compression test. He was otherwise entirely neurologically intact for sensation, motor and reflexes. DIFFERENTIAL DIAGNOSIS Posterior subluxation Superior labrum (SLAP) lesion Rotator cuff tear Rotator cuff tendonitis Strain of posterior capsule of shoulder TEST AND RESULTS Plain radiographs (AP in internal and external rotation, axillary views): reverse bony Bankart lesion MRI shoulder: Reverse Bankart lesion FINAL WORKING DIAGNOSIS Posterior traumatic instability shoulder TREATMENT AND OUTCOMES Patient had failed non-operative rehabilitation for 5 months Diagnostic arthroscopy revealed posterior labrum detachment (reverse Bankart) Open posterior capsular shift with posterior bony Bankart External rotation brace for 6 weeks Progressive rehabilitation program Return to diving over 9 months

A new explanation for an old finding: the ductus bump

Received: 1 May 2004 Revised: 25 May 2004 Accepted: 3 June 2004 Published online: 31 July 2004 Springer-Verlag 2004 In this issue of Pediatric Radiology, Kaushik et al. [1] present an asymptomatic neonate with a 7.5-mm-diameter mediastinal mass. The mass was thought to be a ‘‘ductus bump’’ on the initial film and seemed to disappear within 3 days; over time there was calcification in the region of the ligamentum arteriosum [2]. However, what is most interesting about this child is that both CT and ultrasound showed this ‘‘ductus bump’’ to be a clot containing a ductus arteriosus aneurysm (DAA), which indeed did resolve, but not within 3 days. This case report is important because it prompts us to reconsider the various warnings in the literature concerning a DAA. Many authors have discussed the dangers of these aneurysms and in the past most were surgically removed [3–10]. Newer findings and larger studies using echocardiography show us that a DAA is relatively common (1–8.8%) in normal neonates [11]. All of the DAAs which were less than 11.2 mm in diameter disappeared although 30% closed after thrombus formation [11]. The warnings of spontaneous dissecting DAAs, Marfan’s syndrome or aneurysm of the ductal region in adults are probably an entirely different subgroup. Extensive follow-up, invasive imaging or surgery in most neonates is not advisable [12]. Follow-up ultrasound during the first 1– 2 months is the conservative approach. Those DAAs still present after 1–2 months may need treatment. Patients with larger DAAs, or those with family history of connective tissue disorder, may represent a high-risk segment in which more frequent follow-up is important. In the description of calcification of the ligamentum arteriosum by Currarino and Jackson [13], they suggested that calcification was present when the ductus arteriosus was closed. These new echocardiographic data support their 1970 hypothesis but it may be that the DAA has closed. Kaushik’s [1] report ties the ‘‘ductus bump,’’ DAA and calcification of the ligamentum arteriosum together. The concept of the ‘‘ductus bump’’ may not be viable—all of these are most probably DAAs. The approach of noninvasive observation seems quite appropriate.

Posterior Shoulder Instability in a Competitive Golfer

0943 HISTORY: A 17 year old male who played golf competitively for his high school team was hitting a ball off the tee when he injured his left, non-dominant shoulder. When in his backswing he felt pain, but in the follow through he felt a pop. The pop was accompanied by severe pain and he was unable to continue to play golf. He was seen by his primary care physician who examined him and performed and MRI of the shoulder. The patient consulted with us three weeks after his injury. At that time, he complained of primarily posterior shoulder pain which was non-radiating. He did not have any parasthesias, weakness or any sense that his shoulder was unstable. He did not have a history of any previous injury to his shoulder. He had no history of connective tissue disorders or of any systemic illnesses. PHYSICAL EXAMINATION: The patient had no atrophy or winging of the scapula. He had full elevation of his shoulder with pain posteriorly with elevation. He had equal motion compared to the other shoulder for internal and external rotation both with the arm elevated and with the arms at the side. His neurological examination was entirely normal with no weakness or sensory deficits. He had a negative anterior apprehension test, posterior apprehension test and a grade II sulcus sign bilaterally. He had negative impingment signs and a negative active compression test. He could not relax for laxity testing on the involved shoulder. He was tender only along his posterior joint line. DIFFERENTIAL DIAGNOSIS: Strain posterior shoulder muscle Tear rotator cuff Labrum tear Posterior instability TEST AND RESULTS: Plain radiographs: Anterior-posterior and axillary radiographs: normal MRI with gadolinium: tear of the posterior labrum FINAL WORKING DIAGNOSIS: Posterior instability of the shoulder TREATMENT AND OUTCOMES: Non-operative Strengthening program initiated within six weeks post injury showed no improvement in shoulder function or alleviation of symptoms after 6 months Diagnostic arthroscopy: reverse Bankart lesion Arthrotomy with repair reverse Bankart and capsular shift Placed in a brace post operatively Initiated and completed a structured rehabilitation program Returned to sports 6 months postoperatively with no limitations