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Autosomal-dominant hyperimmunoglobulin E syndrome (HIES), or Job syndrome, is a rare, multisystem, primary immunodeficiency disorder. Additionally, patients may also suffer from connective tissue, dental, and bone malformations. While current management of HIES is directed at prophylactic antibiotics to prevent infections, there is limited work describing surgical considerations for these patients, particularly with respect to hardware placement. Here we report a case of a patient with HIES who underwent orthognathic surgery for maxillary advancement and mandibular setback to address his severe class III malocclusion. The patient's postoperative course was complicated by significant infection, requiring multiple operations and ultimately, hardware removal after bone healing. Although this patient ultimately had a good outcome, the role of orthognathic surgery with implant placement in patients with HIES should be approached with caution and careful consideration.

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RESUMEN

Adolescents with macromastia face serious physical, emotional, and social burdens because of their large breast size. Studies have shown that reduction mammoplasty results in improvement in physical symptoms and quality of life for these patients. Shared medical appointments (SMAs), defined as individual patient-physician encounters that occur in a group setting, have been successfully applied to clinics for adult patients seeking breast reduction for this condition. We decided to apply the SMA model to our clinic for preoperative evaluation of adolescent patients with macromastia. The purpose of this article is to describe how our clinic implemented SMAs, evaluate changes in clinic efficiency, measure patient quality of life before surgery, and assess patient and provider satisfaction with the SMA model.From February to June 2016, our department instituted SMAs for preoperative evaluation of female adolescent patients with macromastia. We measured days from referral to appointment for 25 patients who attended SMAs and compared this with a retrospective cohort of 21 patients who attended traditional visits from 2013 to 2015.Clinic efficiency was measured in new patients per hour. During SMAs, we also administered the BREAST-Q, Pediatric Quality of Life Inventory, and patient and provider satisfaction surveys. Mean days between referral and office visit was reduced from 75.3 with traditional visits to 40.3 with shared appointments, although this finding was not statistically significant (P = 0.69). New patients per hour increased from 1.33 with traditional visits to 3 with SMAs, without reducing time spent on education or with the surgeon. The mean preoperative BREAST-Q scores were less than 40 for the 4 tested domains, and the mean (SD) total Pediatric Quality of Life Inventory score was 56.7 (14.8). Patients and the provider reported high satisfaction with SMAs, and the provider wishes to continue using SMAs in the clinic.In conclusion, SMAs resulted in high patient and provider satisfaction and increased clinic efficiency, without sacrificing time spent on education or with the surgeon. Low quality-of-life scores demonstrate a need for these patients to be evaluated and treated for their condition. Measuring patient-reported outcomes with validated surveys and improving clinic efficiency without sacrificing patient care have the potential to increase value at our institution.

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Orthopedic hardware infections are much feared and costly complications that can occur when these devices are implemented both in traumatic cases as well as in joint replacement surgery. Because these infections can lead to great morbidity, it is important to understand their pathophysiology as well as the principles behind their diagnosis and initial treatment. Plastic surgeons are frequently consulted as part of a multidisciplinary team to provide stable soft tissue coverage of the associated defects that result from these infections. A review of the existing literature was performed to identify the potential causes of these infections, to provide established diagnostic criteria guidelines, and to explain how these prosthetic infections are managed from an orthopedic surgery perspective prior to consulting the plastic surgery team.

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Orthopedic prosthetic infections are potentially devastating complications. Plastic surgeons are frequently consulted to aid in the management of the soft tissue defects that are created by these infections. A review of the existing literature was performed to identify established treatment methods for soft tissue coverage of orthopedic hardware infections for a variety of anatomic locations. The following treatment guidelines and soft tissue reconstructive options were identified as viable options for the management of exposed or infected orthopedic hardware. This review provides descriptions of the various soft tissue reconstructive options available as well as adjunctive treatment methods.

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Fetal ultrasonography is an important tool used to prenatally diagnose many craniofacial conditions. Pierre Robin sequence (PRS) is a rare congenital deformation characterized by micrognathia, glossoptosis, and airway obstruction. PRS can present as a perinatal emergency when the retropositioned tongue obstructs the airway leading to respiratory compromise. More predictable and reliable diagnostic studies could help the treating medical team as well as families prepare for these early airway emergencies. The medical literature was reviewed for different techniques used to prenatally diagnose PRS radiologically. We have reviewed these techniques and suggested a possible diagnostic pathway to consistently identify patients with PRS prenatally.

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