Encephalocraniocutaneous Lipomatosis: A Case Report.

ABSTRACT: Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital syndrome and subclassification of oculoectodermal syndrome. Encephalocraniocutaneous lipomatosis may be associated with postzygotic mutations. However, absence of an identifiable mutation does not preclude a diagnosis of ECCL. Encephalocraniocutaneous lipomatosis commonly causes skin, eye, and central nervous system anomalies. Diagnosis can be made through genetic sequencing or standardized clinical criteria. One clinically apparent major criterion for the diagnosis of ECCL is nevus psiloliparus (NP), a fatty nevus with overlying nonscarring alopecia. In this case, a 50-day-old female infant with uncomplicated birth history presented to dermatology clinic for evaluation of 2 superficial cranial masses that had been present since birth without regression or evolution. One of the masses was located within the hairline and demonstrated overlying nonscarring alopecia, suspicious of NP. Because of concern for ECCL, brain magnetic resonance imaging was ordered and revealed 2 intracranial lipomas. Genetic testing was inconclusive. Excision of the masses was performed at the request of the parents for cosmetic purposes. Histologic evaluation of the surgical specimens confirmed the diagnosis of NP and ECCL. A suspected NP should raise concern for ECCL and prompt further evaluation for systemic involvement. In particular, patients with suspected ECCL should be screened for ocular and CNS involvement. Early identification and diagnosis are important for prognostication because patients with ECCL are at increased risk of developing neoplasms of the head and neck and may require more frequent screening examinations.

Ulnar Wrist Denervation: Articular Branching Pattern and Selective Blockade of the Dorsal Branch of the Ulnar Nerve.

PURPOSE: Ulnar wrist denervation has been a successful treatment for patients with ulnar-sided wrist pain. The purpose of this study was to characterize the articular branches of the dorsal branch of the ulnar nerve (DBUN) and validate a technique for selective peripheral nerve blockade. METHODS: In cadavers, we performed simulated local anesthetic injections using 0.5 mL of 0.5% methylene into the subcutaneous tissue at a point midway between the palpable borders of the pisiform and ulnar styloid. We then dissected the DBUN, characterized its articular branching pattern, and measured staining intensity of the DBUN and the ulnar nerve relative to a standard. RESULTS: The DBUN branched from the ulnar nerve 7.0 ± 1.2 cm proximal to the ulnar styloid. Among 17 specimens, the DBUN provided an average of 1.2 (range, 0-2) ulnocarpal branches and 1.0 (range, 0-2) carpometacarpal articular branches. A simulated local anesthetic injection successfully stained 100% of the DBUN articular branches at or proximal to their takeoff. There was no staining of the proper ulnar nerves. In all specimens, the DBUN supplied at least one articular branch. CONCLUSIONS: A point midway between the palpable border of the pisiform and ulnar styloid may be an effective location for selectively blocking the DBUN articular afferents. CLINICAL RELEVANCE: In this study, we were able to identify a point halfway between the pisiform and ulnar styloid that has the potential to produce a selective peripheral nerve block of the portion of the DBUN that supplies articular fibers to the ulnocarpal joint and the fifth carpometacarpal joint. This technique may prove useful to surgeons treating ulnar-sided wrist pain.

Incidence and Treatment Efficacy of Trigger Finger in the Breast Cancer Population on Aromatase Inhibitors.

BACKGROUND: Trigger finger is a common hand complaint of the general population. Limited literature exists implicating a low-estrogen state in patients on aromatase inhibitor (AI) therapy for breast cancer who develop trigger finger. The authors' objective was to determine the incidence and treatment outcomes of this population. METHODS: A single-center retrospective chart review was conducted on patients with a diagnosis of breast cancer on AI who developed trigger finger from 2010 to 2019. The total population of patients during this time served as our population, and patients with breast cancer not on AI with trigger finger served as our control. Primary outcomes included total number of injections and need for surgery. Secondary outcomes included risk factors for surgery. χ2 analysis and logistical regression model determined the significance of primary and secondary outcomes, respectively. RESULTS: In all, 192 patients of a population size of 664 751 met our study group criteria. The study group showed a higher incidence of trigger finger (5.1% vs 1.3%; P < .001) compared with our population. Patients treated with AI for breast cancer had both higher incidence of trigger finger (5.1% vs 1.5%, P < .001) and injections (77.1% vs 66.5%, P < .001) compared with patients not on AI therapy. Independent risk factors requiring surgical treatment were found in patients with diabetes (odds ratio [OR], 3.54; P = .01) and in patients with concomitant radiation therapy (OR, 3.17; P = .02). CONCLUSIONS: This study demonstrates for the first time the incidence, treatment outcomes, and surgical risk factors of trigger finger in patients on AI therapy for breast cancer.