Isolated Post-Shunt Metopic Synostosis and Neural Tube Defects.

BACKGROUND: Craniosynostosis is an uncommon complication after shunting procedures for congenital hydrocephalus. We report a case of a child with myelomeningocele and normocephaly at the time of birth. She underwent ventricular shunting for Chiari malformation and hydrocephalus at 3 days of age. An immediate postoperative CT scan confirmed all sutures were open. Serial CT scans document an open metopic suture at 2 months, closed metopic suture at 5 months, and trigonocephaly at 11 months with concomitant slit ventricle syndrome, and collapsed lateral and third ventricles. METHODS: An Ovid MEDLINE search within the dates of 1948 through 2017, using the keywords "synostosis AND shunt" was carried out. A tabulation of all patients and their respective synostosis patterns were recorded. RESULTS: We identified 8 case series and 2 case reports during 43 years (1966-2017). Seventy-eight patients with 79 suture synostosis patterns were identified (one patient underwent a second cranial reconstruction for identification of a separate, newly formed synostosis). Eighteen (30.5%) cases were associated with a neural tube defect (NTD). Patients with NTD and secondary craniosynostosis had on average earlier age of shunt placement (P = 0.001), craniosynostosis presentation (P = 0.146), and cranioplasty (P = 0.325) than secondary craniosynostosis patients without NTD. CONCLUSIONS: Ventricular shunt drainage in treating hydrocephalus rarely may lead to early synostosis and cranial deformity, especially in patients with NTDs. Early shunt placement poses significant risk in patients with NTD. Close follow-up may be necessary to evaluate overdrainage and cranial deformity after shunting procedures.

Massive Intradural Dermoid Cyst Without Sinus Tract.

Dermoid cysts can present as a rare, benign, congenital intracranial tumor of neuroectoderm origin trapped during embryogenesis. Past clinical reports have reported lesions in the posterior fossa, at the midline, and in the intradural region all in conjunction with a superficial sinus tract. The authors present a unique patient of a completely intracranial, intradural, dermoid tumor of the midline cerebellum devoid of any evidence of sinus tract. The histological characteristics, radiological features, and management of this unusual patient are described.

Aesthetic refinements in the treatment of graves ophthalmopathy.

BACKGROUND: Graves ophthalmopathy is a chronic, multisystem, autoimmune disorder characterized by increased volume of intraorbital fat and hypertrophic extraocular muscles. Proptosis, impaired ocular motility, diplopia, lid retraction, and impaired visual acuity are treated with orbit decompression and fat reduction. The authors present the addition of skeletal augmentation to further improve periorbital aesthetics. METHODS: Through a transconjunctival with lateral canthotomy incision, a balanced orbital decompression was executed, removing medial and lateral walls and medial floor. Intraorbital fat was excised. All patients underwent placement of porous polyethylene infraorbital rim implants and midface soft-tissue elevation, increasing inferior orbital rim projection and improving the globe-cheek relationship. From 2009 to 2012, 13 patients (11 female and two male; 26 eyes) with Graves ophthalmopathy underwent surgery at two institutions. Outcomes were evaluated for improvements of proptosis, diplopia, dry eye symptoms, and cosmetic satisfaction. RESULTS: Postoperative follow-up ranged from 0.5 to 3 years (median, 1.5 years). The mean improvement on Hertel exophthalmometry was 5.4 mm. Diplopia resolved in three patients (23 percent). No patients had worsening diplopia, and 12 (92 percent) discontinued use of eye lubricants. All patients had cosmetic satisfaction. One patient suffered temporary inferior orbital nerve paresthesia. There were no infections, hematomas, or ocular complications. CONCLUSIONS: Skeletal augmentation is a useful adjunct to orbital decompression and fat excision for treating Graves ophthalmopathy. Balanced orbital decompression with infraorbital rim implants is reliable, effective, and safe, with good, lasting results. Resolution of ocular symptoms is improved, as are the patient's personal well-being and social life, with a high-benefit-to-low-risk. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Scalp metastasis from esophageal adenocarcinoma: comparative histopathology dictates surgical approach.

Cutaneous metastasis of esophageal cancer, in particular esophageal adenocarcinoma, is rare and metastasis to the scalp is extremely rare. We describe such a case that was originally diagnosed as an adnexal carcinoma. A 77-year-old male with a history of esophageal adenocarcinoma status after esophagectomy at our institution 4.5 years prior, presented to our plastic surgery clinic with a 2-month history of 2 temporoparietal scalp lesions. He was referred to our clinic by a community dermatologist who had performed a shave biopsy of the lesions. The clinical diagnosis was adnexal cyst. The history of esophageal carcinoma was not provided to the pathologist. The dermatopathology report came back as malignant adnexal neoplasm and considerations included apocrine carcinoma. We reexamined the pathologist's slides from the outside facility, comparing them to the histopathology from his esophagectomy. Histopathologic changes were identical. Thus, our surgical and postoperative approach changed significantly. Clinical suspicion should be high for cutaneous metastases in patients with a history of solid organ cancers. It is important for clinicians to illicit a history of malignancy. A biopsy should be performed on any suspicious lesions, and clinical data along with histopathology of the prior cancer resection(s) should be provided to the pathologist for comparison. Diagnosis of the suspicious lesion should be made before definitive excision, as this may change the approach, with the potential for postoperative chemotherapy and radiation. The definitive operative approach consists of surgical debulking with the evidence of negative margins. On the scalp, we feel that 5-mm margins are appropriate to obtain clear margins. One should appreciate the subdermal extent of metastases and adjust the margins accordingly. We recommend excising the galea with the skin as an en bloc resection. This will both assure clear deep margins of resection and assist in a tension-free closure of the scalp.

Cytomegalovirus and other infectious issues related to face transplantation: specific considerations, lessons learned, and future recommendations.

BACKGROUND: Composite tissue allotransplantation is fraught with complexities similar to those of solid organ transplantation, including donor-related cytomegalovirus transmission. With this in mind, the authors' objective was to (1) report their team's experience with infections and donor-related cytomegalovirus transmission in relation to face transplantation and (2) review the facial composite tissue allotransplantation literature as it pertains to cytomegalovirus and other various infections. METHODS: A MEDLINE literature search and article review was performed in July of 2010 on all published articles specific to face transplantation, cytomegalovirus disease, and all related infections and/or complications. In addition, the authors retrospectively reviewed their own institution's experience with face transplantation. RESULTS: Two of the world's first four face transplant recipients acquired cytomegalovirus viral infection by means of their donated facial organs. Also, the French experience, and our own, has been challenged by cytomegalovirus reactivation and graft rejection, therefore necessitating a critical evaluation. The authors have also learned, from their own experience, that facial composite tissue allografts containing mucosa and paranasal sinuses present a distinct challenge with regard to their accompanying flora. CONCLUSIONS: Although the risk of donor-derived cytomegalovirus is acceptable in life-saving solid organ transplantation, for face transplantation patients, the scenario is different. When the authors' team performed the first nearly total face/maxilla transplantation (December of 2008), there was little known regarding the consequences of cytomegalovirus-related donor transmission in face transplantation. Therefore, the authors now recommend that all candidates be fully informed as to the risks of cytomegalovirus/infectious transmission and that aggressive viral, bacterial, and fungal prophylaxis be instituted.

Confirmatory factor analysis of the Short Form McGill Pain Questionnaire with burn patients.

The Short Form McGill Pain Questionnaire (SF-MPQ) is an abbreviated version of McGill Pain Questionnaire (MPQ) developed for pragmatic reasons to improve the clinical utility of the MPQ. Although the SF-MPQ has been used in more than 250 published studies, few studies have examined the core constructs it measures. The objective of this study was to evaluate in a sample with burn injuries whether the factor structure of the SF-MPQ is consistent with the theoretic pain constructs it purports to measure. Participants (n = 338) met American Burn Association's criteria for major burn injury and had a mean total body surface area burned of 14%. They were mostly male (70.1%) and Caucasian (63.4%) with an average age of 41.25 years. There were 2 primary findings. First, confirmatory factor analysis yielded fit index values demonstrating viability of a 2-factor, oblique, solution composed of sensory and affective latent constructs. These findings were consistent with previous work and the theoretic constructs. Second, results from a relatively new model consisting of 8 SF-MPQ items demonstrated potential viability for measuring similar constructs.