Does Preservation of Metacarpophalangeal Joint Vascularity Protect Against Physeal Arrest in Index Finger Pollicization?

PURPOSE: To determine whether preservation of blood supply to the index metacarpophalangeal joint decreases the rate of physeal arrest. METHODS: A retrospective review of 41 pollicized digits in 35 patients with 2-year minimum radiographic follow-up was conducted at a single institution. Other complications evaluated included nonunion at the pollicized digit base and clinical instability at the new carpometacarpal joint. Findings were compared to historical controls, which were performed by our group prior to routine identification and sparing of the metacarpophalangeal joint blood supply. No other modifications to surgical technique were made between the previous and current patient cohorts. RESULTS: Two pollicized digits in two different patients had radiographic evidence of physeal arrest, one of which was partial and the other complete, for an arrest rate of 4.9%. This was significantly less than the arrest rate in our historical cohort of 24.7% (21 of 85 patients). Five patients did not have radiographic bony union at the base of the index metacarpal, but only one patient had clinical instability at the new carpometacarpal joint. CONCLUSIONS: Significantly fewer patients who underwent index finger pollicization with preservation of the metacarpophalangeal joint blood supply went on to develop physeal arrest when compared to patients who underwent pollicization prior to adoption of this technique. This finding suggests that sparing of the physeal blood supply is preventative against proximal phalanx physeal arrest. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Index pollicization: an evolution of ideas and techniques from a paediatric orthopaedic institution.

Pollicization has been a very successful procedure in the treatment of specific types of hypoplastic thumb. Although much has remained the same since early descriptions of the procedure in the 1800s and 1900s, refinements over the years have made it safer and more predictable. Over the years at our institution we have studied, modified and refined our incisions to produce a new thumb that is aesthetically pleasing with excellent function. We present our technique for pollicization along with pearls and pitfalls we have discovered.

Challenging the dogma: a straight wrist should be the goal in radial dysplasia.

For centuries, various techniques have been described to stabilize the carpus on the ulna in radial dysplasia to achieve a straight wrist, in the belief that it is better for function and cosmesis. Apart from ulnocarpal fusion, none had succeeded in preventing recurrence of radial deviation. Ulnocarpal fusion, however, carries the risk of shortening an already shortened forearm by damaging the epiphysis. Fingers are often stiff in radial dysplasia, and therefore a straight wrist may actually limit function. Formal assessment of the appearance in operated versus unoperated wrists remain inconclusive. This article challenges the dogma that a straight wrist should be the ideal goal in radial dysplasia. The optimum management of this condition remains debatable.

ASSH 75 Years: An Update of Progress Over the Past 25 Years.

This article chronicles some of the major advancements made by the American Society for Surgery of the Hand over the past 25 years since the publication of William Newmeyer III's monograph, American Society for Surgery of the Hand: The First Fifty Years, in 1995. What is intangible and impossible to articulate in this article are the countless stories of relationship building, education, and research advancement that the programming and activities the American Society for Surgery of the Hand has provided.

Long-term Outcomes Following Vickers Ligament Release and Growth Modulation for the Treatment of Madelung Deformity.

BACKGROUND: Madelung deformity arises from a partial distal radial growth disturbance in combination with an abnormal hypertrophic ligament spanning the volar radius and carpus, termed, the Vickers ligament. The purpose of this study is to report long-term clinical and radiographic outcomes following Vickers ligament release and distal radial physiolysis in a population of skeletally immature patients with symptomatic Madelung deformity. METHODS: Medical records were retrospectively reviewed of patients with Madelung deformity surgically treated between 1994 and 2005. All eligible patients who underwent a Vickers ligament release and distal radial physiolysis were contacted and invited to return to the clinic for follow-up. RESULTS: Six patients (8 wrists) with Madelung deformity underwent Vickers ligament release and distal radial physiolysis. All were white females with a mean age at initial presentation of 11.4 years (10 to 12.8 y). Mean age at the time of initial surgery was 12.0 years (10.0 to 14.5 y). The median follow-up time was 10.6 years (5.8 to 21.9 y) and the average age at last follow-up was 23.1 years (17.5 to 32.2 y). Pain alone or in combination with concerns for deformity was the chief complaint in 6 of 8 of the wrists. At 1 year of clinical follow-up, 7 of 8 wrists were reported to be pain-free, and 6 of the 8 were noted to be completely pain-free at last follow-up. Motion in flexion, extension, pronation, supination, radial, or ulnar deviation was similar between the preoperative status and long-term follow-up. The average preoperative ulnar tilt was 35.1 degrees (SD: 8.5 degrees), average preoperative lunate subsidence was 1.9 degrees (SD: 1.8 degrees), and average preoperative palmar carpal displacement was 21.9 degrees (SD: 2.9 degrees). At the final follow-up, there was a large progression in lunate subsidence, but minimal change in ulnar tilt and palmar carpal displacement. At last clinical follow-up, 2 of the 6 patients had undergone a subsequent procedure including 1 radial dome osteotomy and 1 ulnar shortening osteotomy. CONCLUSION: In the skeletally immature patient population with Madelung deformity with growth potential remaining, distal radial physiolysis and Vickers ligament release is associated with relief of pain, preservation of motion, and, a reasonable rate of reoperation. TYPE OF STUDY: This was a therapeutic study. LEVEL OF EVIDENCE: Level II.

The Oberg-Manske-Tonkin (OMT) Classification of Congenital Upper Extremities: Update for 2020.

A new classification for congenital upper-extremity anomalies was first published in 2010. It has come to be known as the OMT classification highlighting the thought leaders behind it: Kerby Oberg, Paul Manske, and Michael Tonkin. Based on a dysmorphology framework, the OMT has been adopted by the International Federation of Society for Surgery of the Hand and surgeons who treat congenital upper-extremity anomalies. As predicted in the first publication, updates will be necessary based on an improved understanding of morphogenesis; the first update was in 2014 and this represents the second update to the original OMT classification. We carefully reviewed all aspects of the OMT classification, its current stratification, and updated literature on the developmental basis of limb anomalies. We also considered the clinical usefulness and challenges of the classification through discussions with stakeholders and those who care for patients with congenital upper-limb anomalies. These factors guided the current modifications of the OMT classification. In providing the updated classification, we provide the rationale for these changes. The updated OMT classification is by no means final. As our understanding of congenital anomalies progresses, we anticipate subsequent updates in the years to come.

Effect of Distal Ulna Osteochondroma Excision and Distal Ulnar Tether Release on Forearm Deformity in Preadolescent Patients With Multiple Hereditary Exostosis.

BACKGROUND: Multiple hereditary exostosis is a benign condition that can lead to significant forearm deformity secondary to physeal disturbances. As the child grows, the deformity can worsen as relative shortening of the ulna causes tethering, which may lead to increased radial articular angle, carpal slippage, and radial bowing, over time this tethering can also result in radial head subluxation or frank dislocation. Worsening of forearm deformities often require corrective reconstructive osteotomies to improve anatomic alignment and function. The purpose of this study is to evaluate the effectiveness of osteochondroma excision and distal ulnar tether release on clinical function, radiographic anatomic forearm alignment, and need for future corrective osteotomies. METHODS: The authors reviewed a retrospective cohort of preadolescent patients who underwent distal ulna osteochondroma resection and ulnar tethering release (triangular fibrocartilage complex). Patients were invited back and prospectively evaluated for postoperative range of motion, pain scores, self-reported and parent-reported Disabilities of the Arm, Shoulder, and Hand (DASH) and Pediatric Outcomes Data Collection Instrument (PODCI) scores. In addition, preoperative and final postsurgical follow-up forearm x-rays were reviewed. RESULTS: A total of 6 patients and 7 forearms were included in our study with an average age of 7.9 years at time of surgery. The average final follow-up was 7.4 years. With respect to range of motion, only passive radial deviation demonstrated improvement -20 to 14 degrees (P=0.01). Although there was not statistically significant change in radial articular angle, this study did find an improvement in carpal slip 75.7% to 53.8% (P=0.03). At final follow-up DASH score was 5.71 (σ=5.35), PODCI Global Function score was 95.2 (σ=5.81), and PODCI-Happiness score 98 (σ=2.74). Visual analogue scale appearance and visual analogue scale pain assessment were 1.67 (σ=1.21) and 1.00 (σ=1.26), respectively, at final follow-up. No patient in the cohort developed a radial head dislocation. Only one of 7 forearms required a corrective osteotomy within the study's follow-up time period. CONCLUSIONS: Surgical excision of forearm osteochondromas with ulnar tether release in the preadolescent patients improves carpal slip, may help to prevent subsequent surgical reconstruction and provides satisfactory clinical results at an average 7-year follow-up. LEVEL OF EVIDENCE: Level III-therapeutic study.

Case series: Amniotic band sequence with craniofacial abnormalities.

BACKGROUND: To objectively describe craniofacial, visual, and neurological features associated with amniotic band syndrome (ABS) and discuss likely associated multifactorial etiology. METHODS: A retrospective review of patients identified with ABS and concomitant limb involvement and craniofacial features was conducted. The following data were collected from the patients' medical records: demographic information, past medical history including birth history, surgical history, previous clinic visits/physical exams, description of craniofacial features and ABS, family history, any noted obstetric complications, visceral features, visual features, craniofacial features, intracranial features, neurological symptoms, developmental features, diagnostic tests (including radiographs, IQ testing, EEG findings, chromosomes), photographs, and treatment history. RESULTS: Seven patients were included in the final cohort, all of whom had a cleft lip with six having both cleft lip and palate. Other craniofacial abnormalities seen were facial clefts which were vertical oblique in nature, tear duct involvement, cranial deformities that required surgical correction with cranial reconstruction, recorded hypertelorism with vision and gaze abnormalities, coloboma, lagopthalmos and optic never dysplasia. CONCLUSIONS: This case series presents seven children with craniofacial involvement associated with amniotic band sequence and attempts to categorize the salient dysmorphology and neurocognitive development. Major craniofacial anomalies in patients with ABS is a rare clinical finding that cannot be completely explained on the basis of premature amniotic layer disruption alone. This study supports that the dysmorphology seen in cases of ABS with craniofacial involvement is complex and most likely multifactorial. LEVEL OF EVIDENCE: IV Case Series.

Outcomes After Primary Repair and Staged Reconstruction of Zone I and II Flexor Tendon Injuries in Children.

BACKGROUND: Delayed diagnosis of flexor tendon injury in children is common, and consequent flexor sheath scarring may necessitate a 2-stage reconstruction. Previous studies show variable outcomes after 2-stage flexor reconstruction in children, especially those below 6 years old. We evaluated functional and subjective outcomes of primary repair and staged reconstruction of zone I and II tendon injuries in children under 6 years of age. METHODS: A retrospective chart review identified 12 digits in 10 patients who had undergone surgical treatment of a zone I or II flexor tendon injury. Seven digits had a primary repair and 5 had a 2-stage reconstruction. Time delay from injury to surgery for primary repairs averaged 18 weeks and for 2-stage reconstruction averaged 24 weeks. Outcomes included total active motion, tip pinch and grip strength, sensation, and the Pediatric Outcomes Data Collection Instrument (PODCI). RESULTS: Average follow-up was 8 years. At final follow-up, mean total active and passive motion of the involved digit was similar between the primary reconstruction and staged groups, and 58% had a "good" or "excellent" American Society for Surgery of the Hand; total active motion (ASSH TAM) result (71% in the primary repair group, 40% in the 2-stage reconstruction group). All regained grip and pinch strength equal to the contralateral hand. The average PODCI Upper Extremity score was 99 (99 in the primary repair group, 98 in the 2-stage reconstruction group) and PODCI Global Function score was 94 (97 in the primary repair group, 91 in the 2-stage reconstruction group). No complications occurred. CONCLUSIONS: Our small study demonstrates that both primary repair and 2-stage flexor tendon reconstruction have acceptable long-term functional and subjective outcomes in children below 6 years old, although staged reconstruction had a lower overall ASSH TAM score and subcategorical PODCI scores. Although staged reconstruction has acceptable outcomes in this population, prompt primary repair of flexor tendon injuries in children should always be attempted. LEVEL OF EVIDENCE: Level 4-therapeutic.

Insights into the pathogenesis of macrodactyly.

Mirror-image symmetry in limbs is normal in the vertebrate phenotype. Genetic and epigenetic factors regulate the differentiation, patterning, and development of the embryo and foetus. Growth after birth is influenced by hormonal and environmental factors such as nutrition. Limb size asymmetry in a child should trigger a search for associated pathology that may include neoplastic conditions, sequelae of injury, vascular, and neurogenic factors. Macrodactyly, part of the PIK3CA Related Overgrowth Spectrum, offers the physician a clinical challenge, while at the same time an opportunity to study morphology, histology, and more recently the molecular mechanisms from which the conditions arise. Collaboration between clinicians and basic scientists offers an exceptional opportunity for coordinated study and the potential for improved patient outcomes.

Macrodactyly: decision-making and surgery timing.

Macrodactyly is a rare condition in which fingers, hands or limb growth is unregulated, resulting in overgrowth of tissues in the affected extremities. It is critical to properly assess these extremities for signalling pathway, psychological impact and potential surgical intervention, to achieve the best possible outcome for each patient. Treatment approaches can vary, and patient and family expectations weigh heavily on care complexity. Common surgical procedures may include epiphysiodeses, osteotomies, debulking procedures, carpal tunnel releases, toe transfers and amputations. The selection and timing of these surgeries is a vital component of the approach, as delayed healing and excessive scarring may occur. The purpose of this review is to assist in the navigation of decision-making and surgical timing for patients presenting with overgrowth manifesting itself as macrodactyly.

Distal Dorsal Dimelia: A Disturbance of Dorsal-Ventral Digit Development.

PURPOSE: Congenital palmar nail (distal dorsal dimelia [dDD]) of the hand is a rare malformation most commonly affecting the little finger. The purpose of this report was to review the features and associations of this rare disorder and discuss the suspected underlying etiology in light of our current understanding of developmental biology. METHODS: In this retrospective cohort study from 3 practices, we describe our collective experience and review the reported literature on this disorder both as an isolated condition and in conjunction with other anomalies. RESULTS: We examined 15 fingers with dDD, 5 of which involved little fingers. We also found dDD in 6 cases with radial polydactyly (preaxial polydactyl type II [PPD2]) and in 1 case of cleft hand involving digits adjacent to the clefted web space (the index and middle fingers). Cases of little finger dDD were also associated with prominent clefting of the adjacent web space in 4 of 5 cases. All cases had stiffness of the interphalangeal joints and loss of palmar creases consistent with dorsalization of the palmar aspect of the digit. When combined with 63 fingers reported in the literature with dDD, 3 patterns were evident. The most common form occurred in little fingers (n = 50; 64%; dDDu). The next most common form was reported in association with cleft hands (n = 16; 21%; dDDc). Radial digits in association with either radial polydactyly (PPD2) or radial longitudinal deficiency were also susceptible to dDD (n = 12; 15%; dDDr). CONCLUSIONS: Congenital dDD is a disturbance of terminal dorsal-ventral digit patterning. The distribution of this condition with little fingers, clefting, and altered radial digit formation (PPD2 or radial longitudinal deficiency), as well as recent genetic and animal studies, suggests that dDD and altered dorsal-ventral patterning are linked to abnormal apical ectodermal ridge boundary formation. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV.

Camptodactyly Treatment for the Lesser Digits.

Camptodactyly of the lesser digits is commonly seen in patients with arthrogryposis. The flexed posture of the digit can be functionally limiting and necessitate surgical treatment to improve grasp function. In digits with normal bony anatomy, a lateral stiletto-shaped transposition flap combined with flexor digitorum superficialis tendon release, can improve finger deformity and function.

Ulna Growth Patterns After Soft Tissue Release With Bilobed Flap in Radial Longitudinal Deficiency.

BACKGROUND: Centralization is commonly utilized for treating the severely deviated wrist in radial longitudinal deficiency (RLD). Individuals with RLD have congenital shortening of the ulna and previous studies have shown that traditional centralization, in particular with notching of the carpus, results in additional ulnar growth retardation. At our institution, we use a technique of soft tissue release with bilobed flap. We examined if this technique preserves the growth potential of the distal ulna, therefore, avoiding an additionally shortened forearm. METHODS: We retrospectively reviewed serial radiographs of 16 patients with 18 wrists who had at least 3 years of follow-up after a soft tissue release with bilobed flap. Radiographic lengths were measured using the method described by Heikel. Percentage of normal growth was calculated using normative data published by Maresh. Comparisons were made with preoperative, postoperative, and final follow-up studies. RESULTS: The average length of follow-up was 9.2 years (range, 3 to 16.3 y) with an average age of 11.6 years (range, 5.2 to 17.5 y). The average age at the time of surgery was 27 months (range, 14 to 48 mo). A minimum of 3 radiographic studies were available for each subject. The average ulna length preoperatively was 63.9% of age-matched normal length (51.4% to 75.3%). The average ulna length at final follow-up was 61.9% of age-matched normal length (48.5% to 70.3%). The difference was not statistically significant. In addition, there were no distal ulnar physeal arrests. CONCLUSIONS: Soft tissue release with bilobed flap does not affect ulna growth like traditional centralization procedures can. This procedure has previously been shown to retain motion and have similar recurrence rates to formal centralizations. Therefore, we advocate that it be considered more widely for use in patients with RLD and significant wrist deviation to maximize growth and improve hand position. Soft tissue release with bilobed flap can be safely used on young children and preserve ulnar growth. LEVEL OF EVIDENCE: Level IV-therapeutic.

"On-Top Plasty" for Radial Polydactyly Reconstruction.

PURPOSE: To introduce the "on-top plasty" technique and report our long-term outcomes. METHODS: We evaluated 5 thumbs in 5 patients who underwent radial polydactyly reconstruction using the "on-top plasty" technique between 1998 and 2003. This technique was used when it was felt that neither thumb possessed adequate proximal and distal structures to provide a functional and aesthetically pleasing thumb. Our study group included 1 Flatt type III and 4 Flatt type VII thumbs. The average age at time of surgery was 1.4 years and at final follow-up was 13.5 years. Subjective patient evaluation, objective outcomes values, and validated patient-oriented outcome measures were obtained. RESULTS: There was no soft tissue loss and union was achieved in all thumbs with no further surgery required in any thumb. Mean flexion-extension arc for the metacarpophalangeal joint was 60° (range, 10° extension to 70° flexion) and at the interphalangeal joint was 19° (range, 25° extension to 35° flexion). Mean percentage of age-matched norms for lateral, tripod, and tip pinch were 47.0%, 45.9%, and 47.8%, respectively. Mean grip strength was 54.2% of age-matched norm. The mean Pediatric Quality of Life Inventory (PedsQL) score for parent questionnaires was 89.0 and for teen/child questionnaires was 89.1. The Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire revealed a mean score of 4.3. CONCLUSIONS: For patients with radial polydactyly in which neither thumb possesses adequate distal and proximal components, the on-top plasty is a reliable method of polydactyly reconstruction with durable results at longer than 10 years' follow-up. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Long-term Follow-up of Neonatal Brachial Plexopathy: Psychological and Physical Function in Adolescents and Young Adults.

PURPOSE: The prevalence of neonatal brachial plexus palsy (NBPP) has been increasing since the early 1980s. No known studies have examined long-term psychological health and quality of life (QOL) in young adults. The purpose of this study was to investigate the psychosocial and intellectual aspects of NBPP during adolescence into young adulthood. METHODS: A total of 31 patients were enrolled in the adolescent group (16 to 18 y) and 25 in the young adult group (23 to 28 y). Clinical assessment included functional ability, range of motion and strength, weight and body mass index, and education level. Patients were administered measures of psychiatric symptomatology, self-concept, QOL, and cognitive function. RESULTS: Narakas injury level for the adolescent group included 11 level I, 6 level II, 8 level III, and 6 level IV. The young adult group had 10 level I, 2 level II, 9 level III, and 4 level IV. The degree of physical impairment determined by the Modified Mallet Classification showed persistent impairment in both groups. The average DASH scores were higher than the normal range for the adolescent and young adult groups. Forty-five percent of the adolescents and 68% of the young adults were either overweight or obese. All received high school diplomas with 20 of the young adults pursuing higher education.Scores on measures of psychiatric symptomatology and self-concept showed that both groups fell within the normal range. QOL for both groups was also within the normal range. All participants scored average to above average on the cognitive assessment. All measurements were patient reported. CONCLUSIONS: Patients with NBPP can adapt and participate in most activities. This patient sample demonstrated persistent functional limitations and a higher rate of comorbid obesity. However, these patients function psychologically and cognitively within the normal range and many have pursued higher education. LEVEL OF EVIDENCE: Level IV.

Flexor Digitorum Superficialis Opposition Transfer for Augmenting Function in Types II and IIIA Thumb Hypoplasia.

PURPOSE: To report the functional outcomes associated with the flexor digitorum superficialis (FDS) opposition transfer for types II and IIIA thumb hypoplasia and determine if there is any noteworthy difference in the outcome dependent on the pulley used. METHODS: We included patients who underwent a ring FDS opposition transfer and had at least 2 years follow-up. The study group consisted of 36 patients with 40 treated thumbs. All patients underwent follow-up examination and standardized testing. Outcome functional measures were recorded using the Pediatric Outcomes Data Collection Instrument (PODCI). There were 9 type II and 31 type IIIA hypoplastic thumbs. All patients underwent a ring FDS opposition transfer and a 4-flap z-plasty for first web space deepening. The pulley for opposition was flexor carpi ulnaris in 19 and the transverse carpal ligament in 21. Thirty-six thumbs had ulnar collateral ligament reconstructions, with 5 of the 36 undergoing combined ulnar collateral ligament/radial collateral ligament stabilizations. Average follow-up was 7.6 years (range, 2-16 years). RESULTS: Average postoperative Kapandji score was 8 (range, 4-10). Grip, lateral pinch, and tripod pinch strengths averaged 46%, 49%, and 48% of age- and sex-matched normal controls, respectively. There was no significant difference between surgical pulleys used. The Pediatric Outcomes Data Collection Instrument global was 91 (range, 53-100), and PODCI happiness was 87 (range, 15-100). CONCLUSIONS: We found the ring FDS opposition transfer to be an effective method for providing opposition for both type II and IIIA thumb hypoplasia. At follow-up, excellent opposition function and PODCI scores were noted, with no difference related to the type of pulley used. Type II thumbs had significantly greater grip and pinch strengths when compared with type IIIA thumbs.

Exertional Compartment Syndrome with Amniotic Band Disruption Sequence: A Report of Two Cases.

CASES: We report the cases of two female patients who presented with symptoms of lower-extremity exertional compartment syndrome years after initial amniotic band reconstruction. These patients underwent extensive fascial release for treatment of amniotic band disruption sequence (ABDS) of the lower extremity, with resolution of their symptoms. We describe our current technique that is now performed at the time of initial reconstruction for addressing all layers of the deformity. CONCLUSION: The surgical reconstruction of ABDS requires addressing all layers of the deformity. Failure to do so may lead to long-term sequelae. Both patients in this report had exertional compartment syndrome following incomplete fascial release at their primary surgery, requiring subsequent fasciotomies.

Defining Features of the Upper Extremity in Holt-Oram Syndrome.

PURPOSE: To investigate the distinguishing morphological characteristics of children with radial longitudinal deficiency (RLD) in Holt-Oram syndrome (HOS). METHODS: One hundred fourteen involved extremities in 62 patients with a diagnosis of HOS were identified at 3 institutions. Medical records and radiographs were evaluated. Radial longitudinal deficiency and thumb hypoplasia were classified according to the modified Bayne and Klug classification and Blauth classifications, respectively, when possible. Other unusual or distinguishing characteristics were catalogued. RESULTS: There was bilateral involvement in 84% of patients. The forearm was involved in 81% of the extremities and a shortened distal radius (Bayne and Klug type I RLD) was the most commonly identified forearm anomaly (40%). Radioulnar synostosis was present in 15% of the extremities, all in the proximal forearms with reduced radial heads. Thumb aplasia (Blauth type V hypoplastic thumb) was the most common type of classifiable thumb abnormality and occurred in 35% of involved thumbs. Twenty-seven percent of abnormal thumbs affected were not classifiable according to the Blauth classification, and 19% of involved thumbs (hypoplastic or absent) had first-web syndactyly. CONCLUSIONS: The upper extremity in HOS differs from the typical presentation of RLD. The forearm is more often involved and may demonstrate radioulnar synostosis. The thumb is frequently unclassifiable by the Blauth classification and has first-web syndactyly. The presence of radioulnar synostosis and syndactyly of the radial 2 digits in RLD should prompt the hand surgeon to obtain a cardiac evaluation and consider genetic testing for HOS. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic III.