Late Hip Dysplasia After Normal Ultrasound in Breech Babies: Implications on Surveillance Recommendations.

BACKGROUND: There is significant controversy in the literature over rates of late-developing dysplasia following normal screening ultrasound in breech babies, with reported rates varying from 7% to 29%. The purpose of this study is to investigate the rates of radiographic dysplasia in breech babies after a normal ultrasound with a minimum of 1 year of radiographic follow-up. METHODS: This study was an institutional review board-approved prospective study of all patients referred by their pediatrician for concern for developmental dysplasia of the hip between July 2008 and August 2014. We identified all subjects with breech presentation and excluded those with an abnormal initial examination/ultrasound or with <12 months of radiographic follow-up. Anterior-posterior pelvis films were obtained after >12 months and acetabular indices (AIs) were measured and compared with contemporary normative data. Dysplasia was diagnosed as >2 SDs above the mean. RESULTS: A total of 654 patients were referred with a history of a breech presentation, and 150 (22.9%) were found to have clinical instability or sonographic evidence of dysplasia on initial presentation and were observed with serial imaging or treated. Of the remaining 504 subjects with a normal clinical examination and screening ultrasound, 133 (26.4%; 74.4% females, 25.6% males) were followed until at least 12 months of age. Of those presenting at age 12 to 14 months, the mean AI was 0.42±0.83 SD above the mean with a skew towards elevated AIs. At the final follow-up (mean: 20.7±6.7 mo), the mean AI was 0.05±0.92 SD above the mean, and only 3/133 (2.2%) patients had a dysplastic hip. No patients underwent treatment other than an observation during the study period. CONCLUSIONS: One in 5 breech babies have dysplasia at presentation, but late dysplasia following normal screening ultrasound may be less common than previously reported and may be due to our prolonged follow-up period. We recommend surveillance of breech babies with follow-up visits after 12 months of age since earlier visits may offer limited benefits. LEVEL OF EVIDENCE: Level II-prospective prognostic study.

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.

Elbow Release and Tricepsplasty in Arthrogrypotic Patients: A Long-Term Follow-Up Study.

PURPOSE: Obtaining elbow flexion to improve hand-to-mouth reach capability is an essential component of achieving functional independence in pediatric patients with arthrogryposis. This study analyzed the long-term outcomes of elbow release and tricepsplasty in a series of children with arthrogryposis at a tertiary institution. METHODS: Medical records of patients with arthrogryposis who underwent elbow release and tricepsplasty from 1993 to 2015, with at least 2 years of follow-up, were reviewed. Collected measures included preoperative elbow passive range of motion (ROM), postoperative elbow passive and active ROM, shoulder passive and active ROM, and Pediatric Outcomes Data Collection Instrument (PODCI) scores. Our analysis compared pre- and postoperative follow-up of elbow passive ROM and reviewed PODCI scores with age-adjusted normative values. RESULTS: Seventeen patients (4 female and 13 male) with 24 affected upper extremities (10 left elbow and 14 right elbow) were included in final analysis. Age at final follow-up averaged 11 years (range, 4-20 years), mean age at surgery was 2.7 years (range, 9.6 months-9.3 years) with mean follow-up by extremity at 8.3 years (range, 2-18 years). Differences in pre- and post-operative passive elbow ROM were significant for extension, flexion, and total arc of motion. Most parent and self-reported PODCI scores were less than the age-adjusted normal population, except in the domains of Comfort and Pain and Happiness. CONCLUSION: Long-term follow-up of elbow release and tricepsplasty in patients with arthrogryposis indicates both increased and sustained elbow flexion and arc of motion. Although PODCI scores were lower compared with the age-adjusted norm, pediatric patients with arthrogryposis were just as happy and had no more discomfort than their unaffected age-adjusted norms. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

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.

A Comparison of Pavlik Harness Treatment Regimens for Dislocated But Reducible (Ortolani+) Hips in Infantile Developmental Dysplasia of the Hip.

BACKGROUND: Variation exists in the Pavlik harness (PH) treatment regimen for infantile developmental dysplasia of the hip (DDH). The purpose of this study was to determine if the daily PH wear duration (23 vs. 24 h) and frequency of follow-up visits affect the clinical and radiographic outcomes of infants with dislocated but reducible (Ortolani+) hips. METHODS: This study reviewed prospectively enrolled patients with DDH in a single center who presented at age <6 months with Ortolani+ hips and were treated with PH. Recommended daily PH wear duration (23 vs. 24 h) and the frequency of clinic visits in first 4 weeks after the initiation of PH treatment were analyzed. The clinical success (stable hip that did not require closed or open reduction or the use of an abduction orthosis) and radiographic success based on the acetabular index at 2-year follow-up were compared between different PH regimen groups. RESULTS: Sixty-two patients (74 hips, 53 females) with Ortolani+ hips had a mean age of presentation of 23±28 days (range, 4 to 128 d) and mean follow-up of 33.2±18.4 months (range, 8 to 85 mo). Overall clinical success rate of PH for Ortolani+ hips was 93% (69/74 hips) and radiographic success rate at 2 years was 84% (48/57 hips). There was no difference in clinical or radiographic success rate between the 23- and 24-hour wear groups (P>0.99, 0.73) or between hips assessed almost weekly compared with once or twice during the first 4 weeks of PH treatment (P>0.99 for both). CONCLUSIONS: The 23- versus 24-hour PH regimen and frequency of clinic visits in the first 4 weeks of PH treatment did not affect the clinical or radiographic success rate of Ortolani+ hips in infantile DDH. A strict weekly clinic visit and 24-hour PH regimen may not be necessary to obtain stable reduced hips in infants presenting <6 months of age with Ortolani+ hips. LEVEL OF EVIDENCE: Level III-therapeutic.

Comparison of Pavlik Harness treatment regimens for reduced but dislocatable (Barlow positive) hips in infantile DDH.

OBJECTIVE: Although the Pavlik Harness (PH) is the most utilized treatment for developmental dysplasia of the hip (DDH), the ideal treatment protocol (frequency of clinic visits in the first month and daily wear duration) for Barlow â€‹+ â€‹hips (reduced but dislocatable) has yet to be defined. METHODS: This study compared DDH patients with Barlow hips who were treated with 23 vs 24 h per day PH wear and weekly vs every other week visits. Clinical success was defined as a stable hip that did not require closed or open reduction, or the use of an abduction orthosis prior to achieving clinical stability. Radiographic success was based on the acetabular index at 2-year follow up. RESULTS: Sixty-five patients (75 hips/58 females) with Barlow hips had a mean age of presentation of 15 ±â€¯12 days (range 4-70) and mean follow-up of 33 ±â€¯17 months (range 6-90). There was no difference in clinical or radiographic success rate between 23 h vs 24 h wear groups (p > 0.99 both) or the Frequently vs Infrequent visit groups (p = 0.49 both). Overall clinical success rate was 97% (73/75 hips) and radiographic success rate at 2 years was 97% (58/60 hips). CONCLUSION: A strict, weekly clinic visit and 24-h PH regimen may not be necessary to obtain good clinical and radiographic outcomes in infants presenting <6 months of age with Barlow positive hips. LEVEL OF EVIDENCE: Therapeutic, Level III.

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.

Treatment and outcomes of arthrogryposis in the upper extremity.

Upper extremity involvement in patients with arthrogryposis multiplex congentia is quite frequent. Treatment initially consists of stretching and splinting as significant gains can be seen in the first years of life. The goal of any surgical procedure is to improve upper extremity function and performance of daily living activities, yet it is important to treat each patient individually and understand that areas do not always need to be addressed surgically. Despite overall lower functioning scores in this patient population, quality of life scores are comparable to the general aged adjusted population. This article will discuss the clinical presentation, treatment procedures and outcomes when addressing the upper extremities of patients presenting with arthrogryposis.

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.

Treatment Patterns and Outcomes of Stable Hips in Infants With Ultrasonic Dysplasia.

INTRODUCTION: No clear practice guideline exists for the management of stable hip with ultrasonic dysplasia (UD). This study assessed the treatment patterns for stable UD and determined the outcomes of Pavlik harness (PH) treatment or observation (OB). METHODS: This is a prospective study of 80 infants (107 hips) aged ≤12 weeks at presentation diagnosed with stable UD. RESULTS: Sixty-five hips were treated with PH, whereas 42 hips were observed. Patients who were older at the time of initial sonogram and those with lower head coverage were more likely to be treated with PH. The mean head coverage was lower in the PH group, indicating more severe UD. At a 2-year follow-up period, 93% of patients in the OB group and 87% in the PH group had a good radiographic outcome. DISCUSSION: Head coverage and age at first sonogram had a significant influence on the treatment decision for PH. A milder ultrasonic hip dysplasia can be observed because 93% of the patients who were observed had a good outcome. LEVEL OF EVIDENCE: Level II: prospective cohort study.