Ectopia Cordis Sternal Cleft Repair With Mandibular Distractors: A Follow-Up Case Report.

A neonate with thoracic ectopia cordis presented following an uncomplicated delivery. A mandibular distractor was placed to bridge the sternal cleft and retracted (reverse distractor activation) over 24 days to facilitate sternal closure. Follow-up at five years postoperatively demonstrated a well-healed sternum. This novel approach to ectopia cordis repair facilitates slow, steady physiologic accommodation of the heart without hemodynamic instability or long-term complications.

Parasitic rachipagus conjoined twin: case report.

A parasitic twin represents a rare developmental anomaly in which an asymmetrical, nonviable conjoined twin is attached to the host body at the time of birth. Rachipagus is among the rarest of conjoined twin subtypes and typically features a parasitic twin mass attached at the spine. Herein, the authors review the literature and describe the case of a 9-month-old girl presenting with a rachipagus parasitic twin consisting of a fully developed set of lower extremities originating from the midline upper back. After a complete workup to delineate parasite and autosite anatomy, the parasitic twin mass was successfully excised by a multidisciplinary team of surgeons, and the resulting defect was closed in a single stage.

Effectiveness of conservative therapy and helmet therapy for positional cranial deformation.

BACKGROUND: The authors investigated the effectiveness of conservative (repositioning therapy with or without physical therapy) and helmet therapy, and identified factors associated with treatment failure. METHODS: A total of 4378 patients evaluated for deformational plagiocephaly and/or deformational brachycephaly were assigned to conservative (repositioning therapy, n = 383; repositioning therapy plus physical therapy, n = 2998) or helmet therapy (n = 997). Patients were followed until complete correction (diagonal difference <5 mm and/or cranial ratio <0.85) or 18 months. Rates of correction were calculated, and independent risk factors for failure were identified by multivariate analysis. RESULTS: Complete correction was achieved in 77.1 percent of conservative treatment patients; 15.8 percent required transition to helmet therapy (n = 534), and 7.1 percent ultimately had incomplete correction. Risk factors for failure included poor compliance (relative risk, 2.40; p = 0.009), advanced age (relative risk, 1.20 to 2.08; p = 0.008), prolonged torticollis (relative risk, 1.12 to 1.74; p = 0.002), developmental delay (relative risk, 1.44; p = 0.042), and severity of the initial cranial ratio (relative risk, 1.41 to 1.64; p = 0.044) and diagonal difference (relative risk, 1.31 to 1.48; p = 0.027). Complete correction was achieved in 94.4 percent of patients treated with helmet therapy as first-line therapy and in 96.1 percent of infants who received helmets after failed conservative therapy (p = 0.375). Risk factors for helmet failure included poor compliance (relative risk, 2.42; p = 0.025) and advanced age (relative risk, 1.13 to 3.08; p = 0.011). CONCLUSIONS: Conservative therapy and helmet therapy are effective for positional cranial deformation. Treatment may be guided by patient-specific risk factors. In most infants, delaying helmet therapy for a trial of conservative treatment does not preclude complete correction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Sternal "retraction": a novel application of a mandibular distractor in treating ectopia cordis with complete cleft sternum.

Complete thoracic ectopia cordis is associated with a uniformly dismal prognosis. The primary challenges of this disorder are (1) returning the heart to the thoracic cavity, (2) stabilizing the chest wall, and (3) repairing the sternal defect. Previously published techniques for achieving these goals have demonstrated very limited success and/or significant morbidity.We present a novel application of a mandibular distraction device as part of staged repair of this historically challenging problem. This method uses immediate stabilization and gradual "retraction" of the sternal remnants, thereby allowing physiologic compensation and avoiding the cardiorespiratory embarrassment associated with returning the heart to the thoracic cavity. Moreover, this technique allows stabilization of the associated sternal cleft and chest wall without the commonly associated morbidity of the currently available techniques.