Techniques for toddlers: linear band incision for harlequin ichthyosis with associated compartment syndrome.

Harlequin ichthyosis (HI) is a rare autosomal recessive disorder of cornification in which children are born with an extremely thick stratum corneum that becomes a restrictive circumferential encasement around the orifices, limbs, chest, and abdomen, resulting in limb contractures. We present a neonate diagnosed in utero with HI. The infant was born with encasing bands of thickened skin creating strictures that were causing digital and limb cyanosis (compartment syndrome). We treated the child using a new technique of lysis of the encasing bands that we call linear band incision, using a new escharotomy-like procedure while the infant was undergoing a 3-week course oral acitretin therapy. The technique involved linear incision and lysis of encasements that resulted in reperfusion of the injured limbs and prevention of further digital necrosis. The child is currently a healthy 8-year-old boy with skin manifestations resembling congenital ichthyosiform erythroderma. He has use of all of the limbs that were released in the procedures and is maintained on frequent application of bland emollients. Linear band incision is a potentially life- and limb-saving technique in children with HI.

Peritoneal tuberculosis in a 15-month-old male: surgical diagnosis of an insidious disease.

BACKGROUND: Peritoneal involvement is a relatively rare complication of tuberculosis, accounting for approximately 3.3% of extrapulmonary disease in the United States. Clinical diagnosis relies on a preponderance of indirect evidence and is often delayed. We describe such a patient. METHODS: An otherwise healthy 15-month-old male presented with fever, abdominal distention, vague abdominal pains, and a few episodes of watery diarrhea. Standard laboratory and radiologic work-up was unrevealing, and after a prolonged hospitalization, caseating granulomas were identified at diagnostic laparotomy. RESULTS: Definitive treatment was further delayed pending culture results, and the patient's condition worsened until fulminant cardiovascular collapse led to his demise. CONCLUSIONS: Despite effective chemotherapeutic regimens, the overall mortality of tuberculous peritonitis may be as high as 51%. The diagnosis must be considered and empiric antituberculous treatment started early in the course of the disease, even if definitive diagnosis is still pending.

The non-diagnostic ultrasound in appendicitis: is a non-visualized appendix the same as a negative study?

PURPOSE: The purpose of this retrospective study was to investigate outcomes in children who underwent a non-diagnostic ultrasound (US) evaluating for appendicitis and to identify predictors of a negative diagnosis. METHODS: An IRB-approved retrospective chart review was performed on patients age 0-18, who underwent an abdominal US evaluating for acute appendicitis from 2004 through 2013. Clinical data and specified outcomes were recorded, and exams were categorized into non-diagnostic studies and further separated into studies where the appendix was non-visualized. RESULTS: Of the 1383 studies included for analysis, 876 were non-diagnostic for acute appendicitis (63.34%) with 777 specifically because the appendix was non-visualized. Seven hundred forty of the 876 non-diagnostic studies and 671 of the 777 non-visualized studies were ultimately considered true negatives, corresponding to a negative predictive value (NPV) of 84.47 and 86.36%, respectively. In patients with WBC <7.5×10(9)/L, the NPV of non-diagnostic and non-visualized studies increased to 97.12 and 98.86%, respectively. Patients with WBC <11.0×10(9)/L have similarly high NPVs of 95.59 and 96.99% (non-diagnostic and non-visualized). CONCLUSION: Based on the high NPV of a non-diagnostic US in children without leukocytosis, these patients may safely avoid further diagnostic imaging for the workup of suspected appendicitis.

Three-dimensional imaging improves surgical performance for both novice and experienced operators using the da Vinci Robot System.

BACKGROUND: This study was designed to evaluate the impact of 3-dimensional vision on the performance of resident and experienced surgeons using the da Vinci Robot System (Intuitive Surgical, Sunnyvale, CA). METHODS: Four tasks were performed by 12 surgeons with varying experience. Performance times and errors were recorded using both 2-dimensional and 3-dimensional vision for each task. RESULTS: Performance time and error rates for all 4 skills confirm a significant advantage using 3-dimensional vision. Performance times were reduced by 34% to 46% using 3-dimensional imaging for all participants with statistical significance. Error rates were reduced by 44% and 66%. CONCLUSION: Independent of the biomechanical advantages of the da Vinci Robot System, 3-dimensional vision allows for significant improvement in performance times and error rates for both inexperienced residents and advanced laparoscopic surgeons.

A case confirming the progressive stages of pyomyositis.

A teenage boy presented in the early stage of pyomyositis. He had neck pain, tenderness, and fever. A computed tomography scan showed inflammation in the sternocleidomastoid muscle with no fluid collection. This progressed to a pus-filled drainable mass caused by Stapylococcus aureus. The authors describe this case to highlight the predictable stages and increase the index of suspicion to enhance its early recognition.