It is all about form and function: a new approach to giant neonatal sacrococcygeal teratomas.

INTRODUCTION: Giant sacrococcygeal teratomas (GSCTs) involve severe deformation of the buttock region in addition to potential functional impacts. Little interest has been given to improving the aesthetic post-operative appearance in children with these tumours. METHODS: We describe a new technique for immediate reconstruction of GSCTs using buried dermal-fat flaps and a low transverse scar in the infragluteal fold. RESULTS: Our technique allows wide exposure for tumour resection and functional restoration of the pelvic floor while placing the scars in anatomical locations and restoring buttock aesthetics including gluteal projection and infragluteal fold definition. CONCLUSION: Reestablishment of function and form should be kept in mind at initial surgery in GSCT surgery to maximize results and enhance post-operative outcomes. LEVEL OF EVIDENCE: IV.

Management of Congenital Auricular Anomalies.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe normal ear anatomy and development, and evaluate the patient's ears for differences in shape, size, prominence, and symmetry. 2. Identify common congenital ear deformities, including prominent ear, macrotia, Stahl ear, cryptotia, constricted ear, and lobule anomalies. 3. Describe both early nonoperative management and operative techniques for correction of these ear deformities. 4. Be aware of advantages and disadvantages of common and emerging techniques for correction of pediatric ear deformities. SUMMARY: Whereas severe ear malformations such as microtia/anotia are rare, other ear deformities, such as prominent ear, Stahl ear, and cryptotia, are common. Although these ear deformities result in minimal physiologic morbidity, their psychological and cosmetic impact can be significant. Identifying these common deformities and understanding how they differ from normal ear anatomy is critical to their management. In cases where a deformity is identified in neonatal life, ear molding may obviate the need for surgery. Although various surgical techniques have been described for correction of common ear deformities, the surgeon should follow a careful stepwise approach to address the auricular deformity or deformities present. By using such an approach, complications may be minimized and predictable aesthetic outcomes achieved.

Kaposiform hemangioendothelioma with overlapping features of rapidly involuting congenital hemangioma and a delayed complication of necrotizing fasciitis.

We report the case of a male infant born at term with kaposiform hemangioendothelioma (KHE) of the right forearm and coagulopathy. Our case was unusual as it involuted leaving subcutaneous atrophy and prominent veins, which are more commonly observed in rapidly involuting congenital hemangioma. At 3 years of age, the child developed recurrent superficial thrombophlebitis localized to the area where the KHE had regressed. Subsequently, he developed necrotizing fasciitis and thrombotic veins in the same location and group A streptococcal septic shock.

Congenital malformations and medical conditions associated with orofacial clefts in children in Burkina Faso.

BACKGROUND: Orofacial clefts are usually isolated cases but can be associated with other congenital malformations that are either recognised or unrecognised syndromes. The reported prevalence and pattern of such associated malformations, however, vary among studies. OBJECTIVES: To assess the frequencies and aetiologies of congenital malformations and associated medical conditions in children with orofacial clefts in Burkina Faso (Western Africa). METHODS: A retrospective descriptive study was carried out at the El Fateh-Suka Clinic in Ouagadougou, Burkina Faso. All children who attended surgery for the repair of a cleft lip and/or palate were included in this study. RESULTS: The frequency of congenital malformations associated with cleft lip and/or palate was 39/185 (21.1%). In the group with multiple congenital malformations of unknown origin (34 patients; 18.4%), 66.7% had cleft lip and palate, followed by isolated cleft lip (27.4%) and isolated cleft palate (5.9%). The digestive system (35.3%), the musculoskeletal system (19.6%), and eye, ear, face, and neck (15.7%) were the most affected systems. In the group of syndromic malformations (five patients; 2.7%), amniotic band syndrome (one patient), Van der Woode syndrome (one patient), Goltz syndrome (one patient), and holoprosencephaly (two patients) were identified. Medical conditions included anaemia (39.4%), infections (9.2%), malnutrition (7.5%), and haemoglobinopathies (4.3%). CONCLUSIONS: Congenital malformations and medical co-morbidities were frequent in children with OFCs. Further studies and a National Malformations Registry are needed to improve the comprehension of OFCs in Burkina Faso.

Novel techniques for the surgical management of abdominopelvic constriction rings in amniotic band syndrome.

Constriction rings are associated with amniotic band syndrome and most often present in the extremities. Constriction bands of the trunk are rare, and a standard of surgical care remains elusive. Traditional methods of constriction ring excision rely on soft-tissue rearrangement with multiple Z-plasties, but renewed interest in linear closure and limited Z-plasty has emerged. The authors review contemporary literature and report two cases of abdominopelvic constriction ring reconstruction with long-term follow-up. Novel techniques including anterior sheath Y-V plasty, pteruges release of the Scarpa fascia, and limited Z-plasty closure may minimize the need for serrated scar patterns.

Therapy of ulcerated hemangiomas.

BACKGROUND: Cutaneous ulceration is the most common complication of infantile hemangiomas (IHs) seen in a pediatric dermatology practice. OBJECTIVE: The most effective treatments in our experience are compared to those in the current literature. METHODS: The study was a retrospective chart review of therapy of 169 ulcerated IHs at a tertiary care pediatric hospital and a literature review. RESULTS: Combination therapy was the rule. Local wound care was required in all, pain management in 72%, pulsed dye laser in 42%, infection control in 38%, diminution of the hemangioma through systemic therapy in 36%, and suppression of bleeding in 2%. LIMITATIONS: A retrospective review compared to a case-control study has inherent bias. In addition, our cases were all at a tertiary referral center. CONCLUSION: All ulcerated IHs benefit from local barrier creams or dressings. Pulsed dye laser, antibiotics, topical morphine 0.1% in hydrogel, topical becaplermin, and, most importantly, systemic therapy (especially propranolol) to reduce the hemangioma may be useful.

International plastic surgery missions: a framework for resident education using the CanMEDS competencies.

Residency education has shifted over the past decade in an attempt to graduate well-rounded physicians. There is a recognition that a physician's abilities must extend beyond medical knowledge. The Royal College of Physicians and Surgeons of Canada introduced the CanMEDS physician competency framework in 2005. The framework provides 7 areas of competencies that are aimed at providing improved patient care. These competencies are medical expert, communicator, collaborator, manager, health advocate, scholar, and professional. Teaching and evaluating many of these competencies is often challenging for residency training programs. We believe that international surgical missions provide a prime opportunity to teach and evaluate all CanMEDS' roles.Plastic surgery is a field with many different organizations involved in international surgery. Many plastic surgery training programs offer opportunities for residents to become involved in these international surgical missions. Through these trips, residents gain surgical experience, see a variety and volume of clinical cases, and have the opportunity to travel to a foreign country and experience different cultures. We believe that international plastic surgery surgical missions also provide an exceptional micro environment for the teaching of CanMEDS roles. Using examples from residents' personal experiences on international plastic surgery missions to China, Mali, and Cambodia, we describe the benefits of these missions in transferring the CanMEDS competencies to resident training.

Donor-site morbidity of the inferior gluteal artery perforator flap for breast reconstruction in teenagers.

BACKGROUND/OBJECTIVE: Few options, apart from the buttock area, are available for autologous breast reconstruction in thin teenagers. The aim of the present study was to objectively evaluate and compare donor-site morbidity of the inferior gluteal artery perforator (IGAP) flap with that of the previously described inferior gluteal musculocutaneous flap. METHOD: A retrospective review of all IGAP flaps for breast reconstruction performed in teenagers between June 2006 and April 2011 at the Centre Hospitalier Universitaire Sainte-Justine, Montreal, Quebec, was performed. Patients were invited to undergo a specific physical evaluation and complete a questionnaire on aesthetic and functional outcomes. RESULTS: Thirteen records and 11 photographic charts were reviewed. Lateral buttock flattening was noticeable in nine of 11 cases. Three patients experienced some degree of inferior displacement of the gluteal crease. All six patients available for the appointment presented with a zone of dysesthesia or hypoesthesia in the territory of the operated buttock and/or posterior thigh. No motor impairment was found. The questionnaire, completed by eight patients, revealed that six were satisfied or very satisfied with the surgery. Appearance of the operated buttock was rated 3.4 on a scale from 1 to 5 (5 = normal) compared with the normal side. CONCLUSIONS: The IGAP flap remains a suitable option for breast reconstruction in slim teenagers. Similar to the myocutaneous flap, the major donor-site morbidity of the IGAP flap remains sensory impairment involving the posterior femoral cutaneous nerve. There is, however, less visible lateral depression when it is harvested as a perforator flap.

HISTORIQUE ET OBJECTIF: Il existe peu de foyers de prélèvement, à part la région des fesses, pour effectuer une reconstruction mammaire autologue chez des adolescentes minces. La présente étude visait à évaluer et à comparer objectivement la morbidité au foyer de prélèvement du lambeau de l'artère perforante du grand fessier inférieur (APFI) à celle décrite antérieurement au foyer de prélèvement du lambeau musculocutané du grand fessier inférieur. MÉTHODOLOGIE: Les chercheurs ont effectué une analyse rétrospective de tous les lambeaux de l'APFI en vue d'une reconstruction mammaire chez des adolescentes entre juin 2006 et avril 2011 au Centre hospitalier universitaire Sainte-Justine de Montréal, au Québec. Les patientes étaient invitées à subir une évaluation physique précise et à remplir un questionnaire sur les issues esthétiques et fonctionnelles. RÉSULTATS: Les chercheurs ont analysé 13 dossiers médicaux et 11 dossiers photographiques. Ils ont remarqué un aplatissement de la partie latérale de la fesse dans neuf des 11 cas. Trois patientes ont subi un certain degré de déplacement inférieur du pli fessier. Les six patientes disponibles pour le rendezvous présentaient une zone de dysesthésie ou d'hypoesthésie dans le territoire de la fesse opérée ou de la cuisse postérieure. Aucune n'avait d'atteinte motrice. Le questionnaire, qu'avaient rempli huit patientes, a révélé que six d'entre elles étaient satisfaites ou très satisfaites de l'opération. L'apparence de la fesse opérée a obtenu une note de 3,4 sur une échelle de 1 à 5 (5=normal) par rapport au côté normal. CONCLUSIONS: Le lambeau de l'APFI demeure une possibilité acceptable pour procéder à une reconstruction mammaire chez des adolescentes minces. À l'instar du lambeau myocutané, la principale morbidité au foyer de prélèvement du lambeau de l'APFI est une atteinte sensorielle touchant le nerf cutané fémoral postérieur. La dépression latérale est toutefois moins visible lorsqu'on fait le prélèvement sous forme de lambeau de l'artère perforante.

Congenital anomalies of the breast.

Poland syndrome is a combination of chest wall deformity and absent or hypoplastic pectoralis muscle and breast associated with shortening and brachysyndactyly of the upper limb. Clinical presentation varies widely; therefore, reconstructive procedures have to be adapted to the deformity, ranging from chest wall stabilization or augmentation, dynamic muscle transfer, nipple and areola repositioning, and breast augmentation using prosthesis or autologous tissue transfer. Other congenital breast anomalies include supernumerary nipple and areola (polythelia) and breast (polymastia), which can generally be found on the embryonic mammary ridge. Absence of the nipple, areola (athelia), or the breast tissue (amastia) is less frequent.

Subperiosteal release of the floor of the mouth to correct airway obstruction in pierre robin sequence: review of 31 cases.

OBJECTIVE: The purpose of this study was to evaluate subperiosteal release of the floor of the mouth (SRFM) musculature to correct neonatal airway obstruction in children with Pierre Robin sequence and to compare this procedure with other surgical options available. METHODS: A retrospective chart review of patients who underwent a SRFM for neonatal severe airway obstruction unresponsive to conservative management was performed. Preoperative and postoperative oxygenation parameters, sleep studies, feeding difficulties, and long-term evolution were documented. RESULTS: Thirty-one consecutive patients between 1995 and 2009, 19 boys and 12 girls, had an SRFM procedure to alleviate severe neonatal airway obstruction. Eight children were syndromic, 15 had associated malformations, and eight had an isolated Pierre Robin sequence. The airway obstruction was corrected with SRFM alone in 26 children (84%), three patients remained dependent on nasopharyngeal airway, and two required subsequent mandibular osteogenic distraction procedures following the SRFM to manage persistent obstruction. No patient required a tracheostomy. The average age at SRFM was 33 days, and the patients were kept intubated for an average of 6 days postoperatively. Average hospital stay after the surgery was 45 days, and 10 patients went home with gavage feeding. CONCLUSIONS: The SRFM is effective for the treatment of severe airway obstruction in patients with Pierre Robin sequence. The procedure is simple and has fewer complications and secondary procedures than other surgical options for correction of the airway obstruction.

Donor-site morbidity of the inferior gluteal musculocutaneous flap for breast reconstruction in teenagers.

The purpose of this study is to objectively evaluate donor-site morbidity of the inferior gluteal musculocutaneous flap in teenagers. All cases of breast reconstruction performed between 1996 and 2005 using an inferior gluteal flap were reviewed. Flap size, weight, and pedicle origin were noted. Donor-site morbidity was assessed for scarring, contour deformity, muscle function, and sensation. The charts of 15 patients were reviewed, and 6 patients were available for further investigation. The average flap size and weight were 17 cm x 7 cm and 430 g, respectively. All patients had a well-concealed scar with minimal buttock asymmetry. Sensory assessment showed some degree of hypoesthesia in the territory of the posterior femoral cutaneous nerve in all patients. There was no functional loss. Donor-site morbidity of the inferior gluteal musculocutaneous flap is largely related to posterior thigh hypoesthesia despite preservation of the posterior femoral cutaneous nerve.

Treatment outcomes of unstable proximal phalangeal head fractures of the finger in children.

The authors reviewed 37 children presenting with closed, unstable fractures of the proximal phalangeal head. Fractures were classified as intra-articular (n = 15), including 14 unicondylar and 1 bicondylar, or extra-articular (n = 22), including 20 subcondylar and 2 comminuted subcondylar. Eighteen patients underwent closed reduction with K-wire fixation (n = 11) or dynamic skeletal traction (n = 7). Nineteen patients underwent open reduction with K-wire fixation (n = 14) or miniscrews (n = 5). Results were considered excellent when the active range of motion (ROM) of the proximal interphalangeal joint was > or = 90 degrees (n = 26); fair, active ROM, 70 to 89 degrees (n = 6); and poor, active ROM < 70 degrees (n = 5). The intra-articular fractures were rated 9 excellent, 5 fair, and 1 poor. The extra-articular fractures were rated 17 excellent, 1 fair, and 4 poor. The 18 closed reductions, K-wire fixation (n = 11) and traction (n = 7), were rated 16 excellent and 2 fair. The 19 open reductions, K-wire fixation (n = 14) and miniscrew fixation (n = 5), were rated 10 excellent, 4 fair, and 5 poor. Closed reduction showed better results than open reduction. Dynamic skeletal traction proved to be simple and effective in treating these injuries.

Normal human Merkel cells are present in epidermal cell populations isolated and cultured from glabrous and hairy skin sites.

The Merkel cell is a highly specialized cell that primarily acts as a slowly adapting mechanoreceptor. Merkel cells are scarce in normal skin but can be identified by the expression of distinct keratin filaments. Merkel cells constitute a very unique population and many questions still remain as to their origin, number, proliferative capacity, and functions in cutaneous biology. The dissociation of epidermal cells from skin is a widely used technique to extract and culture keratinocytes. We took advantage of a two-step extraction method to quantify keratin-20-expressing Merkel cells among total cutaneous cells obtained from either hairy or glabrous skin biopsies. Flow cytometry analysis revealed that keratin-20-labeled Merkel cells represent between 3.6% and 5.7% of freshly dissociated basal epidermal cells. No significant differences were seen between samples derived from glabrous palmar and hairy anatomic sites, from children and adult, respectively. We also report on the presence of Merkel cells in primary and first subcultures of epidermal cells indicating their capacity to remain viable after extraction from skin of various anatomic sites. To our knowledge, this is the first demonstration of nontumorigenic human Merkel cells in culture in vitro. The persistence of a small number of Merkel cells in culture suggests that, with the development of appropriate culture conditions, these cells could be amplified and further studied to unravel long-standing questions relative to their paracrine function or epithelial origin.

Physiolysis for correction of clinodactyly in children.

Congenital clinodactyly is a lateral deviation of a finger frequently caused by an abnormal middle phalanx (trapezoidal or triangular delta phalanx). The physis extends longitudinally on the short side of the middle phalanx. Resection of the abnormal longitudinal physis and fat graft interposition (physiolysis) has been reported to correct the lateral finger deviation in growing children. We reviewed 35 fingers that had a physiolysis procedure. The age at surgery varied from 2.9 to 10.9 years (mean, 6.6 y), the preoperative angulation was 20 degrees to 29 degrees in 9 fingers, 30 degrees to 39 degrees in 16 fingers, and 40 degrees or more in 10 fingers. Thirty-one fingers presented a trapezoidal phalanx and 4 fingers a triangular phalanx. Ten fingers had a second surgery using the same procedure. Follow-up time ranged from 1.2 to 5.3 years (mean, 3.2 y). After 1 procedure the degree of correction varied from 0 degrees to 30 degrees (mean, 11.1 degrees). The residual angulation was <15 degrees in 8 fingers, 15 degrees to 19 degrees in 4 fingers, 20 degrees to 29 degrees in 15 fingers, 30 degrees to 39 degrees in 6 fingers, and > or =40 degrees in 2 fingers. Correction obtained in the trapezoidal phalanges was better (mean 12.5 degrees) than in the triangular phalanges (mean, 2.8 degrees). The fingers presenting a more severe preoperative deformity (angulation > or =40 degrees) had a better correction (mean, 20 degrees) compared with fingers with a lesser deformity (mean, 7.5 degrees). The correction was also better in children who had surgery before 6 years of age (mean, 17.9 degrees) compared with older children (mean, 6.5 degrees). A second physiolysis procedure was not beneficial in 8 of 10 fingers reoperated and 2 premature fusions of the proximal transverse physis were found among these 10 fingers. There were no other complications. The physiolysis procedure is simple and effective, particularly in children presenting with a trapezoidal phalanx who have surgery before 6 years of age.