Integrated Endoscopic Craniosynostosis Surgery and Cranial Orthosis Program.

INTRODUCTION: Craniosynostosis results from premature fusion of the cranial sutures. There has been a continuous evolution of surgical correction techniques from an extensive open procedure to the minimally invasive technique. AIMS AND OBJECTIVES: This novel surgical technique has never been previously done in the UAE and this article documents the experience of the first team to endoscopically correct craniosynostosis with planning, surgical techniques, and customized helmet therapy. METHODS: A retrospective case series of 16 nonsyndromic craniosynostosis patients with unicoronal (6), sagittal (5), bicoronal (1), frontosphenoidal (2), and metopic craniosynostoses (2) was corrected endoscopically and followed up with customized cranial helmet. Preoperative and postoperative photography, along with anthropometric measurements were taken. RESULTS: The mean age of all patients at the time of surgery was 101 (90-117) days, mean duration of surgery was 70 minutes (38-129), mean blood loss was 120 mL (100-150), and mean duration of hospital stay 4 days (4-5). No complications and mortality were reported. Mean number of helmets was 2 (1-3). Cranial index improved in all patients with sagittal and bicoronal craniosynostosis. Close cooperation with the orthotist ensured timely helmet therapy and accurate laser scanning documentation in head molding. CONCLUSIONS: This case series has demonstrated that endoscopically craniosynostosis correction is safe, effective, and reliable and is the first reported series from the Middle East. The authors believe that every patient with nonsyndromic single­suture craniosynostosis should be offered endoscopic correction before the age of 6 months. The orthotist forms an important part of the Multi-disciplinary team, enabling successful outcomes.

Ulnar dimelia - a review of 24 cases.

Ulnar dimelia is a very rare unilateral congenital upper limb anomaly (CULA) affecting the whole extremity. Treatment remains difficult because of the complexity and multi-level involvement. Twenty-four cases with duplicated ulna, absent radius and polydactyly from seven European centres were reviewed according to a structured list of parameters. At first consultation, median age 8 months (1-178), the shoulder movement was good in 17 patients or poor in six, and the median passive elbow range of motion was 20° (0°-90°). The resting wrist position was flexed in 22/24 patients. Following stretching and splinting, elbow surgery included resection of the lateral proximal ulna in 11 patients and muscle transfers in six to improve passive movement and increase active elbow motion, respectively. Tendon transfers were performed in eight wrists and a pollicization or pseudo-pollicization in 23 patients. Overall, patients demonstrate acceptable function postoperatively. Guidelines for treatment of this severe CULA are presented.Level of evidence: IV.

Utility of a Pediatric Adaptive Sports Clinic: A Case Series Review.

BACKGROUND: Limited funding is available for athletes with disabilities in the United Kingdom. This compounds the barriers to participation and development that already exist. METHOD: To combat this growing problem, a Multi-Disciplinary Pediatric Adaptive Sports Clinic was formed. RESULT: Fifteen athletes with disabilities attended the Clinic from November 2017 to November 2019. In our cohort, there were 10 males and 5 females (age range: 13-18 years). Most athletes participated at a grassroots level (n = 9). The range of diagnoses included cerebral palsy, Ehlers Danlos syndrome and congenital hand differences. Forty-four appointments were made after the initial meeting with a 95% attendance rate. Improvements beyond the minimal clinically important differences (MCID) for the Patient Specific Functional Scale, Numerical Pain Rating Scale, and Medical Research Council Manual Muscle Testing Scale were noted in over half of cases. CONCLUSION: With a focus on injury prevention and strength and conditioning techniques, this clinic supported athletes to successfully compete from a recreational to an elite level across all types of sports and adolescent ages by providing patient-specific regimens. Our case series provides preliminary evidence to suggest the formation of similar clinics that can support athletes with disabilities across a range of sports.

Microsurgery in pediatric upper limb reconstructions: An overview.

The use of microsurgery has spread during the last decades, making resolvable many complex defects considered hitherto inapproachable. Although the small vessel diameter in children was initially considered a technical limitation, the increase in microsurgical expertise over the past three decades allowed us to manage many pediatric conditions by means of free tissue transfers. Pediatric microsurgery has been shown to be feasible, gaining a prominent place in the treatment of children affected by limb malformations, tumors, nerve injuries, and post-traumatic defects. The aim of this current concepts review is to describe the more frequent pediatric upper limb conditions in which the use of microsurgical reconstructions should be considered in the range of treatment options.

Efficacy of mTOR inhibitors (sirolimus) in isolated limb overgrowth: a systematic review.

We systematically assessed the literature on the use of sirolimus for the treatment of isolated limb overgrowth conditions and its various modalities of administration in PubMed, Scopus, Ovid MEDLINE, Web of Science, Google Scholar, Cochrane Database of Systematic Reviews, references of journals and grey literature using pre-trialled Medical Subject Headings terms and articles. Eleven articles were included, and 39 patients were identified for review. Sirolimus was given orally in 38 patients and topically in one patient. Sirolimus was found to be highly effective in treatment of isolated limb overgrowth conditions with improvement of symptoms (physical, emotional, social) reported in all but one patient. Dosage and adverse effects seemed to be closely correlated. The result of our study suggests that sirolimus should be considered as an adjuvant or first-line management in isolated limb overgrowth in prospective trials.

Wrist Ganglion Cysts in Children: An Update and Review of the Literature.

Ganglion cysts are the most common soft tissue tumor of the hand and wrist, affecting pediatric and adult populations. Despite their frequency, there is no consensus within the literature regarding the best management of pediatric wrist ganglia, and there are few recent publications examining this topic. We provide an up-to-date literature review examining the current issues and controversies in the management of pediatric wrist ganglia.

Successful Delayed Neurolysis of the Median Nerve in a Teenager 7 years After Supracondylar Fracture: A Case Report and Review of the Literature.

ABSTRACT: Supracondylar humeral fractures are the commonest elbow fractures in childhood, associated with a high rate of neurological complications. We present the case of a 14-year-old adolescent girl with persisting daily pain, lack of sensation, and strength to her left hand after a displaced supracondylar fracture 7 years earlier. At the time of the fracture, she underwent closed reduction and percutaneous Kirschner wire fixation. During revision surgery, half of the median nerve was found embedded in bone at the level consistent with the healed fracture site. Neurolysis of the fascicles adherent to the bone was performed. Postoperatively, the patient had complete recovery of sensation, reduced pain, and a 2.5-fold improvement of grip strength. This case shows that there is a role for delayed neurolysis in children.

Hand and Upper Limb Malformations in Italy: A Multicentric Study.

Background: Although hand and upper limb malformations are quite frequent, up to now very few reports have been published on epidemiology. The aim of this study is to evaluate the number of infants who presented with hand and upper limb malformations from 2010 to 2015 in Italy. Methods: A retrospective analysis of a pediatric population presenting with hand and upper extremity malformations was carried out, gathering reports achieved from eight Italian Centers of pediatric hand surgery. Other factors such as gender, date and region of birth, family distribution of malformations and associated syndromes, were analysed. Results: Out of 3,100,421 live births, 765 children presented with hand and upper limb malformations. The incidence was 2,5/10,000 live births with a predominance of males and the right side. Radial polydactyly was the anomaly with the highest percentage, closely followed by simple syndactyly, simbrachidactyly and complex syndactyly. Less common conditions were the triphalangic thumb, thumb in palm, proximal radioulnar synostosis and Sprengel deformity. Inheritance of and familial predisposition to those malformations was recorded in 25 cases, while 84 children presented with syndromes related to hand anomalies. Conclusions: In conclusion the incidence of hand and upper extremity malformations in Italy is lower than that registered in other countries. The retrospective nature of the study combined with the fact that some defects frequently evade pediatric hand surgeon consultations are some possible limitations of the study. However, our data confirmed that, in spite of the decrease in the birth rate in Italy, the trend of congenital hand disorders maintained a stable trend. We aim to integrate this study with a prospective analysis and to involve the institutional health authorities in other countries so as to register the correct incidence of hand and upper extremity defects.

Perforator-based Reverse Radial Teno-Adipo-Fascial Flap: Surgical Technique and Case-report for the Functional Reconstruction of the Dorsum of the Hand.

BACKGROUND: Dorsal complex cutaneotendinous lesions of the hand represent a reconstructive challenge. The use of composite microvascular flaps and vascularized tendon grafts represent the gold-standard. The radial anti-brachial region can still represent an excellent donor site, to the detriment of the possible sacrifice of the radial artery. The reverse radial anti-brachial flap can be either perforator-based, thus saving the radial artery or raised as an adipo-fascial flap, to spare the skin. PATIENTS AND METHODS: A case of post-traumatic highly contaminated dorsal cutaneotendinous defect of the second ray of the hand was reported. An original surgical reconstructive technique with a Revers Radial Teno-Adipo-Fascial Flap (RRTAFF) plus vascularized Palmaris Longus was described, preserving the radial artery. A simple partial thickness skin graft was performed a second time to complete dorsal cutaneous coverage. A subsequent infection was managed by trusting the complete vascularization of the tissues used for the reconstruction. RESULTS: The hand healed well with containment of the infection. The dorsal healed skin appeared elastic and pliable enough. Passive and active motion of interphalangeal and metacarpofalangeal joints were very satisfying. The donor site was well healed, with almost no morbidity. CONCLUSIONS: This reconstructive strategy provides a quick and straightforward single-stage option for the reconstruction of complex cutaneotendinous defects of the dorsum of the hand. Such a reconstruction, with a completely vascularized procedure, is particularly indicated in cases of high contamination or infection of the recipient site.

Quadriceps tendon reconstruction using a fascia lata included in a reverse-flow anterolateral thigh flap.

Quadriceps tendon re-rupture after surgical repair is an overall estimated 2% complication. We report a case of reconstruction in a large tendon and soft tissue defect using a reverse-flow anterolateral thigh (ALT) perforator flap including fascia lata in a 75-year-old man presented with septic necrosis of a reconstructed quadriceps tendon. A reverse-flow ALT flap was transferred to the knee defect; the fascia lata was sutured to the residual tendon. Post-operative flap congestion and infection were successfully treated with debridement and conservative treatment. One year after surgery, the patient was able to fully and actively extend the knee, with an acceptable aesthetic appearance. The reverse-flow anterolateral thigh flap including fascia lata may be a good option for coverage of soft-tissue defects around the knee and contemporary quadriceps tendon reconstruction, particularly in case of septic tendon necrosis, where the use of non-vascularised tissues is contraindicated.

Are there risk factors for complications of perforator-based propeller flaps for lower-extremity reconstruction?

BACKGROUND: Conventional pedicled flaps for soft tissue reconstruction of lower extremities have shortcomings, including donor-site morbidity, restricted arc of rotation, and poor cosmetic results. Propeller flaps offer several potential advantages, including no need for microvascular anastomosis and low impact on donor sites, but their drawbacks have not been fully characterized. QUESTIONS/PURPOSES: We assessed (1) frequency and types of complications after perforator-based propeller flap reconstruction in the lower extremity and (2) association of complications with arc of rotation, flap dimensions, and other potential risk factors. METHODS: From 2007 to 2012, 74 patients (44 males, 30 females), 14 to 87 years old, underwent soft tissue reconstruction of the lower extremities with propeller flaps. General indications for this flap were wounds and small- and medium-sized defects located in distal areas of the lower extremity, not suitable for coverage with myocutaneous or muscle pedicled flaps. This group represented 26% (74 of 283) of patients treated with vascularized coverage procedures for soft tissue defects in the lower limb during the study period. Minimum followup was 1 year (mean, 3 years; range, 1-7 years); eight patients (11%) were lost to followup before 1 year. Complications and potential risk factors, including arc of rotation, flap dimensions, age, sex, defect etiology, smoking, diabetes, and peripheral vascular disease, were recorded based on chart review. RESULTS: Twenty-eight of 66 flaps (42%) had complications. Venous congestion (11 of 66, 17%) and superficial necrosis (seven of 66, 11%) occurred most frequently. Eighteen of the 28 complications (64%) healed with no further treatment; eight patients (29%) underwent skin grafting, and one patient each experienced total flap failure (2%) and partial flap failure (2%). In those patients, a free anterolateral thigh flap was used as the salvage procedure. No correlations were found between complications and any potential risk factor. CONCLUSIONS: We were not able to identify any specific risk factors related to complications, and future multicenter studies will be necessary to determine which patients or wounds are at risk of complications. Propeller flaps had a low failure rate and risk of secondary surgery. These flaps are particularly useful for covering small- and medium-sized defects in the distal leg and Achilles tendon region and are a reliable and effective alternative to free flaps. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

Oculodentodigital dysplasia: ulnar-sided syndactyly and its associated disorders.

PURPOSE: Hand surgeons are often the first specialists to see patients with oculodentodigital dysplasia (ODDD), when infants with ulnar-sided syndactyly are referred. Major associated problems include neurologic, ophthalmologic, dental, and other skeletal abnormalities. The purposes of this study were to investigate the incidence of the reported associated conditions in the families of our patients with ODDD, correlate them with the severity of syndactyly, and provide better counseling with more accurate information for these patients and families. METHODS: We reviewed medical records from Texas Scottish Rite Hospital for Children from 1980 to 2009 to identify patients with ODDD. These patients and families were invited to return for a detailed medical and family history and physical examination documenting hand, foot, eye, dental, and facial findings. RESULTS: A total of 73 pediatric patients from 47 families were diagnosed with ODDD, and 38 individuals in 31 families agreed to participate in the study. We observed bilateral syndactyly in 32 patients, with symmetric involvement in 31 of these. Abnormalities of the shape and size of the middle phalanx of the small finger were common. Ophthalmological findings were present in 31 and dental abnormalities identified in 33 patients. Neurologic findings likely related to ODDD were found in 11 patients. There appeared to be an association between the severity of syndactyly and the severity of dental and urologic findings, but not ophthalmologic or neurologic findings such as paraparesis or cognitive deficits. CONCLUSIONS: We found a 29% incidence of neurologic manifestations in patients with ODDD. In addition, associated ophthalmologic, dental, and developmental conditions are frequent and a heightened awareness will allow appropriate referrals for patients with ulnar-sided syndactyly in ODDD. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

Long-term results of replantation of the proximal forearm following avulsion amputation.

This study reports on the long-term functional outcomes of a homogeneous series of 10 cases of successful replantation of an avulsed proximal forearm and its acceptance on the part of patients. After a minimum follow-up of 3 years (average, 4.7 years), muscular and sensory recovery was evaluated with the Medical Research Council scale, and global function according to the demerit score system of Chen (China Med 5:392-397, 1967). Subjective evaluation and patient satisfaction were investigated by means of a questionnaire. One patient was classified as grade 2, 4 patients as grade 3, and 5 patients as grade 4 according to Chen (China Med 5:392-397, 1967). However, in spite of the poor objective results, patient satisfaction was obtained in 90% of cases, and the replanted extremity was considered of help for common activities of daily living. In conclusion, replantation of an avulsed proximal forearm should be considered only in patients who are strongly motivated to maintain body integrity, and who are aware of the expected functional limitations.