Congenital Forearm Pseudarthrosis, a Systematic Review for a Treatment Algorithm on a Rare Condition.

BACKGROUND: A congenital forearm pseudarthrosis is a rare condition and is strongly associated with neurofibromatosis type 1. Several surgical techniques are described in the literature, but the most optimal treatment strategy remains unclear. This systematic review aims to develop a treatment algorithm that may aid in clinical decision making. METHODS: The PROSPERO registration number for this study was CRD42018099602 and adheres to the PRISMA guidelines for systematic reviews. Embase, MEDLINE, Cochrane Central, Web of Science, and Google Scholar databases were searched for published studies reporting on congenital forearm pseudarthrosis not related to other underlying pathologies like bacterial infection or fibrous dysplasia. Results were not restricted by date or study type, only English literature was allowed. Studies were assessed for quality using the critical appraisal checklist for case reports from the Joanna Briggs Institute. Patient characteristics, underlying disease, type of surgery, union rate, and functional outcome were extracted from included studies. RESULTS: Of 829 studies identified, 47 were included in this review (17 case series and 30 case reports, a total of 84 cases). A one-bone forearm procedure showed highest union rates (92%), however, it results in loss of forearm rotation. Free vascularized fibula grafting showed high union rates (87%) and was related to good functional outcome of elbow flexion and forearm rotations. Other procedures showed disappointing outcomes. CONCLUSIONS: Congenital forearm pseudarthrosis is best treated with a free vascularized fibula grafting, a one-bone forearm procedure should be used as a salvage procedure. Evidence extracted from the case reports was sufficient to generate a treatment algorithm to be used in clinical pediatric practice. LEVEL OF EVIDENCE: Level IV-therapeutic.

Randomized Trial Comparing Preliminary Results of Radialization and Centralization Procedures in Bayne Types 3 and 4 Radial Longitudinal Deficiency.

BACKGROUND: The choice of surgical procedure in severe (Bayne and Klug types 3 and 4) radial longitudinal deficiency (RLD) is contentious. Existing studies have reported varying results with both centralization and radialization procedures. The purpose of this study was to compare the clinical and radiologic outcome of radialization and centralization procedures at a short-to-intermediate-term follow-up for the treatment of types 3 and 4 RLD. METHODS: Fourteen patients with 17 affected limbs having types 3 or 4 RLD were recruited in this prospective, randomized, controlled trial. After initial application of successive casts for soft tissue distraction, patients were randomized to 2 wrist alignment procedures-centralization and radialization. Clinical and radiologic parameters recorded at stipulated intervals until a final follow-up of 24 months included hand-forearm angle, ulnar bow, forearm length, arm length, total angulation, and range of motion at elbow, wrist, and fingers. RESULTS: Centralization was performed in 9 affected limbs, whereas radialization was performed in 8 affected limbs. Nine affected limbs had type 4 RLD, and 8 affected limbs had type 3 RLD. There was no significant difference in the hand-forearm angle in the immediate postoperative period. At 3 months, the radiologic hand-forearm angle increased to 19 degrees in the centralization group, while the radialization group showed an average increase to 4 degrees. This increase in the hand-forearm angle continued at 6-, 12-, and 24-month follow-up assessments. Worsening of the deformity was more in the centralization group, as compared with the radialization group. The forearm length also significantly differed in the 2 groups at 6-, 12-, and 24-month follow-up; however, when adjusted for preoperative lengths, the difference was significant only at 12- and 24-month follow-up. CONCLUSIONS: At a short-to-intermediate-term follow-up, radialization fares better than centralization in terms of recurrence of deformity and in terms of affecting the forearm length. Longer follow-up with a larger sample size is needed to draw definitive conclusions. LEVEL OF EVIDENCE: Level I.

Congenital limb deficiency in Japan: a cross-sectional nationwide survey on its epidemiology.

BACKGROUND: Congenital limb deficiency is a rare and intractable disease, which impairs both function and appearance of the limbs. To establish adequate medical care, it is necessary to reveal the actual conditions and problems associated with this disease. However, there have been no extensive epidemiological surveys in Japan addressing this disease. This is the first nationwide epidemiological survey of congenital limb deficiency in this country. METHODS: With the cooperation of epidemiology experts, we performed a two-stage nationwide survey to estimate the number of patients with congenital limb deficiency and reveal basic patient features. We targeted orthopaedic surgery, paediatric, and plastic surgery departments. Hospitals were categorized according to the institution type and the number of hospital beds; hospitals were randomly selected from these categories. We selected 2283 departments from a total 7825 departments throughout Japan. In this study, we defined congenital limb deficiency as partial or total absence of the limbs, proximal to the proximal interphalangeal joint of the fingers/lesser toes or interphalangeal joint of the thumb/great toe. We distributed the first survey querying the number of initial patient visits from January 2014 to December 2015. Targets of the second survey were departments that reported one or more initial patient visits in the first survey. RESULTS: In the first survey, 1767 departments responded (response rate: 77.4%). Among them, 161 departments reported one or more initial patient visits. We conducted the second survey among these 161 departments, of which 96 departments responded (response rate: 59.6%). The estimated number of initial visits by patients with congenital limb deficiency was 417 (95% confidence interval: 339-495) per year in 2014 and 2015. The estimated prevalence of congenital limb deficiency in Japan was 4.15 (95% confidence interval: 3.37-4.93) per 10,000 live births. The sex ratio was 1.40. Upper limbs were more affected than lower limbs. CONCLUSIONS: We revealed the estimated number of initial patient visits per year and birth prevalence of congenital limb deficiency in Japan. Our results will contribute to establishing the disease concept and grades of severity of congenital limb deficiency.

Upper Limb Dimelia.

A patient with upper limb dimelia including a double scapula, humerus, radius, and ulna, 11 metacarpals and digits (5 on the superior side, 6 on the inferior side) was treated with a simple amputation of the inferior limb resulting in cosmetic improvement and maintenance of range of motion in the preserved limb. During the amputation, the 2 limbs were found to be anatomically separate except for the ulnar nerve, which, in the superior limb, bifurcated into the sensory branch of radial nerve in the inferior limb, and the brachial artery, which bifurcated into the radial artery. Each case of this rare anomaly requires its own individually carefully planned surgical procedure.

Reversed Palmaris Longus Muscle Causing Volar Forearm Pain and Ulnar Nerve Paresthesia.

A case of volar forearm pain associated with ulnar nerve paresthesia caused by a reversed palmaris longus muscle is described. The patient, an otherwise healthy 46-year-old male laborer, presented after a previous unsuccessful forearm fasciotomy for complaints of exercise exacerbated pain affecting the volar forearm associated with paresthesia in the ulnar nerve distribution. A second decompressive fasciotomy was performed revealing an anomalous "reversed" palmaris longus, with the muscle belly located distally. Resection of the anomalous muscle was performed with full relief of pain and sensory symptoms.

A Graphic Overlay Method for Selection of Osteotomy Site in Chronic Radial Head Dislocation: An Evaluation of 3D-printed Bone Models.

PURPOSE: Three-dimensional (3D) computed tomography imaging is now being used to generate 3D models for planning orthopaedic surgery, but the process remains time consuming and expensive. For chronic radial head dislocation, we have designed a graphic overlay approach that employs selected 3D computer images and widely available software to simplify the process of osteotomy site selection. METHODS: We studied 5 patients (2 traumatic and 3 congenital) with unilateral radial head dislocation. These patients were treated with surgery based on traditional radiographs, but they also had full sets of 3D CT imaging done both before and after their surgery: these 3D CT images form the basis for this study. From the 3D CT images, each patient generated 3 sets of 3D-printed bone models: 2 copies of the preoperative condition, and 1 copy of the postoperative condition. One set of the preoperative models was then actually osteotomized and fixed in the manner suggested by our graphic technique. Arcs of rotation of the 3 sets of 3D-printed bone models were then compared. RESULTS: Arcs of rotation of the 3 groups of bone models were significantly different, with the models osteotomized accordingly to our graphic technique having the widest arcs. CONCLUSIONS: For chronic radial head dislocation, our graphic overlay approach simplifies the selection of the osteotomy site(s). Three-dimensional-printed bone models suggest that this approach could improve range of motion of the forearm in actual surgical practice. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Shoulder joint replacement can improve quality of life and outcome in patients with dysmelia: a case series.

BACKGROUND: Arthroplasty is a proven treatment option for glenohumeral osteoarthritis. Common indications include primary or posttraumatic osteoarthritis, avascular necrosis of the humeral head, rotator cuff tear arthropathy and rheumatoid osteoarthritis. Arthroplasty is rarely performed among patients with glenohumeral dysmelia. An overuse of the upper limb in patients with thalidomide-induced phocomelia and people with similar congenital deformities like dysmelia results in premature wear of the shoulder joint. This study aims to evaluate our experience with cases of glenohumeral osteoarthritis caused by dysmelia and treated with arthroplasty. To date, few reports on the outcome of shoulder arthroplasty exist on this particular patient group. CASE PRESENTATION: We included four dysmelic patients (five shoulders) with substantial glenoid dysplasia in a prospective database after approval by the local ethics committee. Once conservative treatment options had been exhausted, the patients were treated with shoulder arthroplasty and assessed clinically and radiographically before and after surgery. The mean patient age at the time of surgery was 50.4 years. The minimum follow-up time was 24 months (24-91 months). All patients experienced a considerable improvement of range of motion (ROM) and a relief of pain. No intra- or postoperative complications appeared. CONCLUSION: Patients with dysmelia have acceptable short and mid-term results with resurfacing hemiarthroplasty. It is an effective although somewhat complicated method to relieve pain and improve movement. Long-term performance of arthroplasty in patients with dysmelia remains to be seen, particularly with regard to the remaining problem of the altered and often deficient glenoid.

Overgrowth of the Hand and Upper Extremity and Associated Syndromes.

The family of overgrowth disorders affecting the hand and upper extremity constitutes a wide spectrum of clinical phenotypes. These conditions and malformations may be isolated to the upper limb or part of an underlying syndrome. When present, these conditions will challenge even the most experienced hand surgeon. Overlapping clinical presentations and a lack of insight into the fundamental pathogenesis that drives overgrowth in these conditions have created confusion in diagnosis and classification, and have also hampered treatment outcome research. In recent years, advances in molecular biology have identified genetic mutations within the affected tissues of overgrowth patients that appear to mediate these disorders. This may elucidate further understanding, classification, and treatment of these conditions. The purpose of this article is to discuss a range of overgrowth conditions, review some of the newer biological insights, and delineate the general treatment principles.

Ulnar Distraction Osteogenesis in the Treatment of Forearm Deformities in Children With Multiple Hereditary Exostoses.

PURPOSE: To report on the outcomes of using ulnar lengthening combined with acute angular correction for the treatment of forearm deformities in patients affected by multiple hereditary exostoses (MHE). Our hypothesis was that this procedure would improve both radiographic measurements and clinical outcomes with minimal complications. METHODS: A retrospective chart review was performed on patients who had a diagnosis of MHE and had undergone ulnar lengthening via a uniplanar external fixator over a 12-year period. Clinical outcomes such as range of motion, pain, and surgical complications were assessed. Radiographic changes were measured using interval radiographs. RESULTS: The series included 17 patients. Median age at surgery was 7 years (range, 3-14 years). Median follow up was 55 months (range, 5-125 months). Improvements occurred in radial and ulnar radii of curvature, carpal slip, ulnar variance, and carrying angle at the elbow. There was 1 major pin track infection. There were 2 failures of the external fixator requiring exchange. Premature consolidation occurred in 1 case. Elbow, forearm, and wrist motion was not affected. Radiocapitellar joint congruency did not change. No patient reported pain at final follow-up. CONCLUSIONS: Our approach of using distraction osteogenesis of the ulna with angular correction in the radius and ulna as needed is able to correct carpal slip as well as to improve forearm bowing and elbow carrying angle. All of the patients maintained congruency of the radiocapitellar joint with no postoperative dislocations. Because of the low complication rate, the resolution of pain in patients who presented with pain, and the improvement of forearm bowing, this approach should be considered as a treatment option for children with MHE who are at risk for radiocapitellar dislocation. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Effect of the Proximal Abducting Ulnar Osteotomy on Intra-Articular Pressure Distribution and Contact Mechanics of Congruent and Incongruent Canine Elbows Ex Vivo.

OBJECTIVE: To determine the effects of the Proximal Abducting Ulnar Osteotomy (PAUL) on contact pressures of congruent and incongruent (short radius) canine elbows. STUDY DESIGN: Ex vivo biomechanical study. SAMPLE POPULATION: Unpaired normal cadaveric canine forelimbs (n=16). METHODS: A servohydraulic testing frame and thin-film sensors were utilized to measure intra-articular contact area (CA), mean contact pressure (mCP), and peak contact pressure (pCP) for medial and lateral elbow compartments. Percent contribution of the medial compartment relative to the whole (%Med) was also examined. Baseline data were collected in 9 congruent elbows and 7 incongruent elbows where the radius was shortened. Both sets of elbows were tested following ulnar osteotomy and sequential placement of 2 and 3 mm PAUL plates and paw repositioning (to account for any medial to lateral shift of transarticular forces). Paired t-tests compared sequential procedural steps. P<.05 was significant. RESULTS: For congruent elbows, the 2 mm PAUL plate decreased CA in both compartments compared to baseline; lateral pCP increased with subsequent paw repositioning. Induction of radio-ulnar incongruity decreased CA and increased mCP medially, decreased pCP laterally, and increased %MedCA and %MedmCP compared to baseline. Both PAUL plates decreased mCP and pCP medially, with no effect laterally. Paw repositioning had no effect. CONCLUSION: The PAUL procedure had no effect on medial compartment pressure in the congruent elbow. It may ameliorate increased medial compartment pressure in the incongruent elbow. This change does not result from a medial to lateral compartmental shift and deserves further investigation.

[Congenital deformities of the hand and upper limb]. / Les malformations congénitales de la main et du membre supérieur.

Congenital deformities of the hand and upper limb include a significant number of clinical situations. Their expression is, as in all congenital diseases, variable. Therefore, we can almost consider that each clinical situation is a bit unique. The difficulty, as any congenital disease, is the fact that the clinical cases are extremely diverse and difficult to classify. So what to do and surgical strategies are often matter of "School". This is even more true that the evaluation of results is very difficult due to low series (poor statistical value) and functional assessment to be partly due to the growth of the child. It is impossible in a few pages to describe all malformations of the hand and upper limb, summarize the indications and evaluate the results. Also, this chapter is not exhaustive and we will focus primarily on the most frequent pathologies.

Clinical and molecular characterisation of Holt-Oram syndrome focusing on cardiac manifestations.

BACKGROUND: Holt-Oram syndrome is characterised by CHD and limb anomalies. Mutations in TBX5 gene, encoding the T-box transcription factor, are responsible for the development of Holt-Oram syndrome, but such mutations are variably detected in 30-75% of patients. METHODS: Clinically diagnosed eight Holt-Oram syndrome patients from six families were evaluated the clinical characteristics, focusing on the cardiac manifestations, in particular, and molecular aetiologies. In addition to the investigation of the mutation of TBX5, SALL4, NKX2.5, and GATA4 genes, which are known to regulate cardiac development by physically and functionally interacting with TBX5, were also analyzed. Multiple ligation-dependent probe amplification analysis was performed to detect exonic deletion and duplication mutations in these genes. RESULTS: All included patients showed cardiac septal defects and upper-limb anomalies. Of the eight patients, seven underwent cardiac surgery, and four suffered from conduction abnormalities such as severe sinus bradycardia and complete atrioventricular block. Although our patients showed typical clinical findings of Holt-Oram syndrome, only three distinct TBX5 mutations were detected in three families: one nonsense, one splicing, and one missense mutation. No new mutations were identified by testing SALL4, NKX2.5, and GATA4 genes. CONCLUSIONS: All Holt-Oram syndrome patients in this study showed cardiac septal anomalies. Half of them showed TBX5 gene mutations. To understand the genetic causes for inherited CHD such as Holt-Oram syndrome is helpful to take care of the patients and their families. Further efforts with large-scale genomic research are required to identify genes responsible for cardiac manifestations or genotype-phenotype relation in Holt-Oram syndrome.

Ulnar Rotation Osteotomy for Congenital Radial Head Dislocation.

PURPOSE: To evaluate an ulnar rotation osteotomy for congenital anterior dislocation of the radial head. METHODS: Nine patients (5 boys and 4 girls aged 6 to 13 years) with congenital anterior dislocation of the radial head were treated with ulnar rotation osteotomy. Magnetic resonance imaging of the elbow showed the proximal radioulnar joint on the anterior-lateral side of the ulna rather than on the lateral side in patients with congenital anterior dislocation of the radial head. On the basis of this finding, we performed an osteotomy on the ulna and laterally rotated the proximal radioulnar joint achieving radial head reduction and restoring the anatomical relationship between the radial head and the capitellum. Clinical and radiographical evaluation of the elbow was performed before surgery and at postoperative follow-up. RESULTS: All patients were followed for 13 to 45 months after surgery. Elbow radiography showed that the radiocapitellar joint was reduced in all patients at the last follow-up visit and that the carrying angle was decreased relative to that in the preoperative condition. Elbow stability and the range of elbow flexion motion were improved at the last follow-up. We did not observe ulnar osteotomy site nonunion or elbow osteoarthritis in these patients. Furthermore, radial head dislocation did not recur. CONCLUSIONS: At early follow-up, ulnar rotation osteotomy was a safe and effective method for the treatment of congenital anterior dislocation of the radial head. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Treatment of supination deformity for obstetric brachial plexus injury: a systematic review and meta-analysis.

PURPOSE: To conduct a systematic review and meta-analysis of the literature evaluating treatment outcome for supination deformity in obstetric brachial plexus injury. METHODS: We included studies on brachial plexus and supination deformity with follow-up of at least one year and quantitative measurements of forearm mean passive pronation and position at rest. Meta-analysis was used to explore modifying factors. RESULTS: An elaborate search strategy resulted in 366 studies, of which 13 were included totaling 238 patients (157 osteotomies and 71 biceps rerouting). There was a 75° gain in position at rest and a 65° gain in passive pronation for the osteotomy group, compared to a 79° gain in position at rest for the biceps rerouting group. More severe deformities had greater gains. No influence of age was found. Important adverse effects were hardware failure and a biceps rupture. Recurrence in the osteotomy group was 20% to 40%, versus none in the soft-tissue group. CONCLUSIONS: This review showed an overall benefit for forearm osteotomies and for biceps rerouting for supination deformity with the gain proportionate to the severity of the deformity. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III.

Novel prosthesis with proximity sensor for upper extremity phocomelia.

We herein report on the application of a novel motorized prosthetic hand in a child with upper extremity phocomelia.

Disruption of the radial/ulnar axis:congenital longitudinal deficiencies.

Radial, ulnar, and central deficiencies represent a spectrum of abnormalities in the development of the upper limb. Radial longitudinal deficiency is often associated with abnormalities in other organ systems, such as cardiac and renal, and so requires a comprehensive medical evaluation. On the other hand, ulnar longitudinal deficiency tends to be associated only with other musculoskeletal abnormalities. In all of these conditions, there is a high incidence of ipsilateral thumb abnormalities. Given the importance of the thumb in overall hand function, abnormalities of the thumb often guide treatment for these conditions. Surgical treatment of the wrist and forearm in radial longitudinal deficiency is controversial, as will be outlined in this review.

Correction of forearm deformities in congenital ulnar club hand: one-bone forearm.

PURPOSE: To describe our experience in the correction of congenital ulnar club hand, using the one-bone forearm procedure. METHODS: Fifteen cases of congenital ulnar club hand treated at Gaslini Children's Hospital of Genoa, Italy, from 1996 to 2008 were evaluated retrospectively. The one-bone forearm procedure was proposed for all 9 cases of type 2 (following the Bayne classification) and then performed in 8 patients with an average age of 5.5 years (range, 3-12 y). At surgery, the patients presented paradoxical hyperextension of the elbow (up to 45° of extension). RESULTS: Average follow-up was 5.3 years (range, 1 to 13 y). Union of the osteotomy was obtained in all cases. In 7 cases, union occurred in an average of 65 days (range, 45-90 d); in 1 case (surgery at 12 y of age), union was delayed, with recovery 5.5 months after surgery and no need for further surgical procedures. All treated cases showed improvement of forearm function and of grasping ability of the hand (generally tridactyl). In addition, the paradoxical hyperextension movement that was present before surgery disappeared in all patients. Long-term radiographic follow-up showed in all cases the formation of a structure that was morphologically similar to that of the previously resected radial head. CONCLUSIONS: The one-bone forearm procedure improves malformed forearm functions, thus increasing the possibility of carrying out daily life activities. The creation of the one-bone forearm should be preceded by the release of the distal ulnar anlage, which maintains fixed wrist deformity; this procedure should be performed within the first year of life.

The fate of the index metacarpophalangeal joint following pollicization.

PURPOSE: To characterize the complications that occur at the index metacarpophalangeal (MCP) joint following pollicization and to identify the blood supply of the index MCP joint. METHODS: Eighty-five pollicized digits in 74 patients (1974-2007) were followed after surgery and had documented clinical examinations and radiographs to evaluate physeal arrest, nonunion at the pollicized digit base, and instability of the new carpometacarpal joint at a minimum of 2 years following surgery. RESULTS: Proximal phalanx physeal arrest was the most common complication. Radiographic nonunion at the juncture of the index metacarpal head and base occurred with and without instability. Twenty-one of 85 pollicized digits showed radiographic evidence of physeal arrest, 12 of which were complete and 9 partial. No clinical factor was found to significantly correlate with a physeal arrest, although the 9 patients with the diagnosis of Holt-Oram syndrome trended toward a higher percentage, with 6 digits in 5 patients with Holt-Oram syndrome showing this complication. Twenty pollicized MCP joints did not have bony union to the base of the index metacarpal, but only 3 were clinically unstable and required surgical stabilization. Ten pollicized digits developed some degree of instability and subluxation at the new carpometacarpal joint, but only one required surgical intervention. In recent cases, a search for the blood supply to the MCP joint has demonstrated a consistent vessel deep to the interosseous muscles that arborizes on the volar metacarpal neck. Our surgical technique has evolved to preserve this vessel whenever possible. CONCLUSIONS: Our complications are most likely due to technical factors. Careful dissection of the index MCP joint during pollicization should help reduce physeal growth arrest. Patients with Holt-Oram syndrome might have an increased risk of growth arrest. However, the majority of patients did not require secondary surgery and have good function. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Taylor spatial frame in the treatment of upper extremity conditions.

BACKGROUND: Taylor spatial frame (TSF) is a modern multiplanar external fixator that combines ease of application and computer accuracy; it provides the capability of 1 to 6 axes of deformity correction sequentially or simultaneously by adjusting 6 connecting struts between 2 circular rings. Previous reports have documented the effectiveness of the TSF in acute fracture care, nonunion treatment, and in bone lengthening and deformity correction in the lower extremity. To the authors' knowledge, no previous case series in the English literature have documented the use of the TSF in treating upper extremity conditions. Our experience with the use of this external fixator in the treatment of upper extremity length abnormality, angulation, and bone transport is summarized. METHODS: Over a period of 7 years, TSF was used in 12 patients with varying upper extremity pathologies that were collected from our prospective external fixator database. The classic TSF planning strategy was adopted and the TSF web-based program was used. All cases were followed for a minimum of 2 years. The database and radiographs were reviewed to obtain demographic data, malalignment parameters, final correction, time in the TSF, and complications. RESULT: Patients' ages ranged from 8 to 18 years. Eight humeral and 4 radial cases were identified. These included 4 cubitus varus and 1 cubitus valgus deformity, 1 neglected supracondylar fracture, 2 humeral nonunion, 2 radial malunion cases, and 1 radial shaft septic nonunion. Time in the TSF varied according to patient age and bone involved. Five patients had superficial pin site infections that resolved with oral antibiotics. Postoperatively mean final angulation on the anteroposterior radiograph was 1 degree (range, 0 to 5 degrees) and the mean final angulation on the lateral radiograph was 0.5 degrees (range, 0 to 2 degrees). Union of bone was achieved in all cases. CONCLUSIONS: The TSF is an external fixator that can be successfully used as a treatment alternative for the definitive treatment of upper extremity conditions involving a deformity and or shortening or bone transport in the pediatric and adolescent patient population. LEVEL OF EVIDENCE: Level IV.

Textiloma mimicking a pericardial hydatid cyst: a case report.

Textiloma is unusual and uncommon diagnosis which is rarely considered. We report the case of a 13 year-old patient with Holt-Oram syndrome. He was operated on in 2006 for ostium secundum atrial septal defect. The postoperative course was uneventful until 2010 when the child presented paroxysmal dyspnoea. Investigations revealed para-cardiac mass which was thought to be a hydatid cyst, but operative findings showed textiloma.