Jordan W. Swanson

Tuberculosis-associated haemophagocytic syndrome

Haemophagocytic syndrome is a disorder characterised by fevers, lymphadenopathy, hepatosplenomegaly, cytopenias, and hyperferritinaemia due to dysregulated activation and proliferation of macrophages, leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes, and their haematopoietic precursors throughout the reticuloendothelial system. Primary or familial haemophagocytic syndrome appears to have a genetic aetiology, whereas secondary haemophagocytic syndrome may be associated with malignancy, autoimmune disease, or infection. Epstein-Barr virus is the most common infectious aetiology implicated in haemophagocytic syndrome, but the syndrome has been associated with a variety of other viral, bacterial, and parasitic pathogens. We describe a case of haemophagocytic syndrome associated with disseminated Mycobacterium tuberculosis. We review all cases of M tuberculosis-associated haemophagocytic syndrome reported in the English language literature and discuss important issues pertaining to the epidemiology, diagnosis, and management of this disease.

Cleft palate repair and velopharyngeal dysfunction

LEARNING OBJECTIVES After studying this article, the participant should be able to: (1) Describe the technical details common to all cleft palate repairs that optimize outcomes and minimize complications. (2) Explain the subjective and objective evaluation of speech in children with cleft palate. (3) Practice with an increased awareness of the management of complications associated with cleft palate repair. (4) Design a treatment plan for velopharyngeal dysfunction. SUMMARY Goals of a successful cleft palate repair include separation of the oral and nasal components without fistula, achieving sufficient velar length, and creating functional transverse orientation of the levator muscle sling. A number of techniques have been described to achieve these goals, but they all have the following technical details in common: elevation of oral mucosal flaps based on the greater palatine arteries, tension free nasal lining mobilization, and functional intervelar muscle dissection. After palate repair, speech evaluation needs to be performed by an objective interdisciplinary team following a standardized protocol. Identification of velopharyngeal insufficiency secondary to an incompetent nasopharyngeal port will necessitate secondary speech surgery. These secondary techniques include pharyngeal flaps, soft palate lengthening, or pharyngeal sphincters, which should be tailored to optimize speech, while minimizing the risk of obstructive sleep apnea.

Correlating Haller Index and cardiopulmonary disease in pectus excavatum.

BACKGROUND The Haller Index (HI) has become standard for determining the severity of pectus excavatum. We compared patterns of cardiopulmonary dysfunction and their relationship with HI in patients with pectus excavatum. METHODS We performed cardiopulmonary exercise testing and chest computed tomography scans on 90 patients with pectus excavatum deformities at a regional pediatric hospital. RESULTS The median HI was 4.9 in patients with combined dysfunction, 4.4 in patients with isolated pulmonary dysfunction, 3.6 in patients with isolated cardiac dysfunction, and 3.4 in patients with normal function. HI varied significantly by disease group (P < .009). HI was significantly lower in patients with normal forced vital capacity than with abnormal forced vital capacity (P = .001). However, HI was similar in patients with normal and abnormal oxygen pulse (P = .24) or peak oxygen consumption (P = .37). CONCLUSIONS Fifty-nine percent of patients had cardiac and/or pulmonary limitation. A HI greater than 3.6 is associated with pulmonary dysfunction, but not cardiac dysfunction.

Adopted children with cleft lip and/or palate: a unique and growing population.

Background: Standard clinical pathways are well established for children with cleft lip and/or palate. Treatment of internationally adopted children differs because of the late age at presentation, a newly evolving child-family relationship, and variable extent and quality of previous treatment. Methods: The authors characterized the presentation and treatment patterns of all internationally adopted children with clefts at their institution between 1997 and 2011. Results: Among 1841 children with clefts, 216 (12 percent) were internationally adopted: 78 percent had cleft lip and palate, 18 percent had cleft lip, and 4 percent had cleft palate. Patients originated predominantly from China (80 percent). Median age at presentation was 31 months, and the rate of new presentations increased five-fold during the study period. Eighty-two percent presented with prior cheiloplasty, and revision was recommended for 64 percent of them. Thirty-seven percent of patients had prior palatoplasty, of which 34 percent presented with a palatal fistula. Secondary palatoplasty/pharyngoplasty was performed more frequently for patients who underwent primary palatoplasty before adoption than after adoption (95 percent CI, 0.20 to 0.77). Overall, adoptees required secondary surgery more often than nonadoptees (49 percent versus 28 percent) regardless of where their primary surgery was performed. Changes in adoptee growth indices suggested improvements in systemic health following adoption. Conclusions: Internationally adopted children with clefts have unique treatment challenges. Children with unrepaired clefts undergo surgery late, and children with prior repairs frequently undergo revision. Compared with nonadoptees, adoptees require more revisions and have a higher fistula rate. Further detailed study is important to optimize care.

Trajectories to death in patients with burn injury.

BACKGROUND With unprecedented survival rates in modern burn care, there is increasing focus on optimizing long-term functional outcomes. However, 3% to 8% of patients admitted to burn centers still die of injury. Patterns in which these patients progress to death remain poorly characterized. We hypothesized that burn nonsurvivors will follow distinct temporal distributions and patterns of decline, parallel to the trimodality of deaths previously described for trauma. METHODS We retrospectively identified all adult deaths from 1995 to 2007 in the National Burn Repository database (n = 5,975) and at our regional burn center (n = 237). We stratified patients by age and analyzed injury and death characteristics. We used objective criteria to allocate nonsurvivors to one of four trajectories: early rapid decline, early organ failure, late sudden death, or late-onset decline. RESULTS The greatest concentration of deaths in both samples and age groups occurred within 72 hours of injury and decreased subsequently with no later mortality peak. Death was most often caused by burn shock within the first week of injury, cardiogenic shock or lung injury in Weeks 1 to 2, and sepsis/multiorgan failure after Week 2. In decreasing frequency, trajectories to death fit the pattern of early rapid decline (58%), early organ failure (20%), late-onset decline (16%), and late sudden death (6%). CONCLUSION Most burn deaths follow a pattern of early rapid decline or early organ failure manifested by death or critical illness within several days of the burn. These findings indicate that more than three quarters of burn deaths are attributable to failure or significant decompensation beginning in the resuscitation phase. Sporadic deaths later in hospitalization are uncommon. Despite significant advances in burn resuscitation, our data indicate that ongoing efforts to mitigate deaths in modern burn care should still focus on care improvements in the resuscitation phase. LEVEL OF EVIDENCE Epidemiologic study, level III.

Skin graft loss resulting from collagenase clostridium histolyticum treatment of Dupuytren contracture: case report and review of the literature.

Treatment of Dupuytren disease with collagenase clostridium histolyticum is increasingly used among hand surgeons. Although it is generally safe and efficacious, complications related to enzymatic fasciotomy occur. Postapproval surveillance and communication among hand surgeons continues to refine the indications, contraindications, and complications recognized in the treatment of Dupuytren disease with enzymatic therapy. Major treatment-related adverse events previously reported include flexor tendon rupture and complex regional pain syndrome. We report a patient who experienced total loss of a well-established volar ring finger skin graft following collagenase injection and propose a potential mechanism of vulnerability. This case may illustrate the susceptibility of type I collagen, which is uniformly present in a healed skin graft bed, to degradation with collagenase. We propose a cautious approach when considering treatment of a Dupuytren cord with collagenase in the presence of an overlying skin graft, regardless of the age of the graft.

Evaluation of Optical Coherence Tomography to Detect Elevated Intracranial Pressure in Children

Importance Detecting elevated intracranial pressure in children with subacute conditions, such as craniosynostosis or tumor, may enable timely intervention and prevent neurocognitive impairment, but conventional techniques are invasive and often equivocal. Elevated intracranial pressure leads to structural changes in the peripapillary retina. Spectral-domain (SD) optical coherence tomography (OCT) can noninvasively quantify retinal layers to a micron-level resolution. Objective To evaluate whether retinal measurements from OCT can serve as an effective surrogate for invasive intracranial pressure measurement. Design, Setting, and Participants This cross-sectional study included patients undergoing procedures at the Children’s Hospital of Philadelphia from September 2014 to June 2015. Three groups of patients (n = 79) were prospectively enrolled from the Craniofacial Surgery clinic including patients with craniosynostosis (n = 40). The positive control cohort consisted of patients with hydrocephalus and suspected intracranial hypertension (n = 5), and the negative control cohort consisted of otherwise healthy patients undergoing a minor procedure (n = 34). Main Outcomes and Measures Spectral-domain OCT was performed preoperatively in all cohorts. Children with cranial pathology, but not negative control patients, underwent direct intraoperative intracranial pressure measurement. The primary outcome was the association between peripapillary retinal OCT parameters and directly measured elevated intracranial pressure. Results The mean (SD) age was 34.6 (45.2) months in the craniosynostosis cohort (33% female), 48.9 (83.8) months in the hydrocephalus and suspected intracranial hypertension cohort (60% female), and 59.7 (64.4) months in the healthy cohort (47% female). Intracranial pressure correlated with maximal retinal nerve fiber layer thickness (r = 0.60, P ⩽ .001), maximal retinal thickness (r = 0.53, P ⩽ .001), and maximal anterior retinal projection (r = 0.53, P = .003). Using cut points derived from the negative control patients, OCT parameters yielded 89% sensitivity (95% CI, 69%-97%) and 62% specificity (95% CI, 41%-79%) for detecting elevated intracranial pressure. The SD-OCT measures had high intereye agreement (intraclass correlation, 0.83-0.93) and high intragrader and intergrader agreement (intraclass correlation ≥0.94). Conventional clinical signs had low sensitivity (11%-42%) for detecting intracranial hypertension. Conclusions and Relevance Noninvasive quantitative measures of the peripapillary retinal structure by SD-OCT were correlated with invasively measured intracranial pressure. Optical coherence tomographic parameters showed promise as surrogate, noninvasive measures of intracranial pressure, outperforming other conventional clinical measures. Spectral-domain OCT of the peripapillary region has the potential to advance current treatment paradigms for elevated intracranial pressure in children.

Distraction Osteogenesis Versus Conventional Fronto-Orbital Advancement for the Treatment of Unilateral Coronal Synostosis: A Comparison of Perioperative Morbidity and Short-Term Outcomes.

Abstract Fronto-orbital advancement and remodeling (FOAR) remains the most widely practiced treatment of unicoronal craniosynostosis (UCS) despite recent studies of ocular dysfunction and aesthetic shortcomings in the long-term. The aim of the study was to compare perioperative morbidity and short-term outcomes of a recently developed, nondevascularizing, distraction-based treatment of UCS with conventional FOAR. To do so, the authors compared the first 6 patients who were treated with a new osteotomy/distraction approach to the last 6 patients who underwent traditional FOAR for the treatment of UCS with regards to demographics, operative details, perioperative morbidity, and short-term outcomes. Between July 2012 and June 2014, 6 patients underwent each procedure. Duration of surgery and length of hospital stay in the distraction group were on average 2 hours 7 minutes and 3.4 days, respectively, significantly less than in the traditional FOAR group (P = 0.039, P = 0.032, respectively). Perioperative blood loss averaged 169 mL, which trended toward less than in the traditional group (mean of 400 mL, P = 0.065). Patients undergoing conventional compared with distraction osteogenesis-mediated FOAR were significantly more likely to develop new-onset strabismus postoperatively (odds ratio 15.4; P = 0.0384). All 12 patients completed therapy without complications and with Whitaker grade I results at latest follow-up. In the perioperative period, distraction-mediated cranial vault remodeling provides similar correction of the aesthetic deformity associated with UCS and an improved morbidity profile. Longer follow-up is needed to determine how distraction compares with FOAR with respect to neuropsychologic and long-term aesthetic outcomes.

Perioperative Complications in Posterior Pharyngeal Flap Surgery: Review of the National Surgical Quality Improvement Program Pediatric (NSQIP-PEDS) Database

Background Posterior pharyngeal flap (PPF) surgery is effective for treating velopharyngeal insufficiency but has historically been associated with risk of airway compromise. This study aims to identify risk factors for complications from and readmission after PPF using a national database. Methods Patients who underwent PPF surgery were selected from the 2012 American College of Surgeons National Surgical Quality Improvement Program Pediatric (NSQIP-PEDS) database. Patient characteristics, comorbidities, and complication/readmission data were analyzed. Results Among 225 study patients, 12 (5.3%) suffered perioperative complications. The most common complications were pulmonary in nature (5 patients, 2.2%), including prolonged postoperative mechanical ventilation (3 patients, 1.3%). Underlying asthma (P = .024) or any cardiac risk factor (P = .047) conveyed significant complication risk. Further, severe cardiac risk factors were associated with postoperative bleeding (P = .024). Readmission (4 patients, 1.7%) and reoperation (3 patients, 1.3%) occurred at mean intervals of 9 and 10 days after the original procedure. Seventy-nine patients (35%) were discharged postoperatively on an outpatient basis, and this subgroup included only one patient (1.2%) with a complication (P = .038). Conclusions The overall perioperative complication rate for PPF surgery is low at 5.3%. Patients with underlying cardiac risk factors, severe American Society of Anesthesiologists Physical Status class, and asthma should prompt greater attention given their heightened risk profiles.

Maternofetal trauma in craniosynostosis

Background: Premature cranial suture fusion may prevent neonatal skull malleability during birth, increasing the risk of unplanned cesarean delivery and neonatal birth trauma caused by cephalopelvic disproportion. We sought to determine the incidence of perinatal maternofetal complications in cases of craniosynostosis. Methods: Records of children presenting with nonsyndromic craniosynostosis to a tertiary pediatric hospital from 1996 to 2012 were reviewed retrospectively with focus on birth history and birth-related complications. Results: Six hundred eighteen births were reviewed. Rates of cesarean delivery among mothers of children with craniosynostosis [n = 201 (32.5 percent)] exceeded the overall regional rate of 24.5 percent (OR, 1.50; p < 0.0001). Unplanned cesarean delivery occurred in 19.7 percent of births, and were most associated with nulliparous mothers, breech fetal presentations, and lambdoid or multisuture synostosis patterns. Eleven neonates (1.8 percent) exhibited cranial birth trauma, including cephalohematoma and subgaleal hematoma. Neonates with sagittal or multisuture synostosis patterns were more likely to suffer birth trauma and had a higher mean head circumference than those who did not (81st versus 66th percentile, p < 0.05). Conclusions: In the setting of craniosynostosis, birth trauma is increased—for mothers in the form of increased cesarean delivery risk, and for fetuses in the form of subgaleal and subperiosteal perinatal bleeding. Difficult maternal labor may be mediated especially by multisuture or lambdoid synostosis, whereas fetal birth trauma may be mediated to a greater extent by large head size. Prenatal diagnosis of craniosynostosis could influence decision-making in the management of labor. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, IV.