Harlequin syndrome in a pediatric population: a case series

Author(s):  
N. Beullens ◽  
G. Tourlamain ◽  
L. Vallaeys ◽  
F. Roelens ◽  
M. D’Hooghe
2020 ◽  
pp. 000313482096006
Author(s):  
Joseph G. Brungardt ◽  
Quinn A. Nix ◽  
Kurt P. Schropp

Background Congenital diaphragmatic hernia (CDH) is a pathology most often affecting the pediatric population, although adults can also be affected. Few studies exist of adults undergoing repair of this defect. Using a national database, we sought to determine demographics and outcomes of this population. Methods An analysis of the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) database (2015-2018) was performed, capturing patients with postoperative diagnosis of CDH, distinct from ventral hernia. Two groups were created based upon surgical approach of open or minimally invasive (MIS) repair. Baseline demographics and outcomes were compared. Results 110 patients undergoing surgical correction of CDH were captured in the database. We found rates of return to the operating room (4.55%), postoperative respiratory failure (5.45%), and reintubation (3.64%) with no difference between groups. There was no mortality and no difference between groups in length of operation, discharge to home, or postoperative complications. Patients undergoing open repair had a longer length of stay than patients in the MIS group (6.47 ± 10.76 days vs. 3.68 ± 3.74 days, P = .0471). Mesh was used in MIS more often than the open group (47.30% vs. 5.56%, P < .0001). Discussion This study describes rates of postoperative complications in patients undergoing repair of CDH, and suggests outcomes those are equivalent between patients receiving open or MIS approaches. Further case series or retrospective studies are needed to further describe this population of patients.


2021 ◽  
Vol 9 (7_suppl3) ◽  
pp. 2325967121S0002
Author(s):  
Indranil Kushare ◽  
Ramesh Babu Ghanta ◽  
Nicole A. Wunderlich

Background: Traumatic internal degloving injury i.e. Morel-Lavallée lesion (MLL) develops as a result of blunt trauma with tangential shear forces. In the pediatric population, these have been described only as small case reports till date. Purpose: To describe the largest case series of lower extremity Morel-Lavallée lesion (MLL) in the pediatric population, to determine its etiology (especially its relationship to sports), treatment and outcomes. Methods: Retrospective study of patients <18 years presenting to a tertiary children’s hospital with MLL between 2013-2019. Demographics, clinical data, imaging features, treatment and outcomes data was collected. Descriptive statistical analysis was conducted. Results: 38 patients (21 males, 17 females) having MLL with mean age 14.6 years were classified into 2 groups- hip/thigh MLL (9 patients) and lower leg MLL (29) (Table 1.1). Most common cause of lower leg MLL was sports injury (79%). Most commonly implicated sports in MLL were football and baseball(24% each). Associated injuries were seen in 9 (23%) patients. Radiographs done in 58% of patients showed no bony abnormalities. Imaging modality of choice was MRI for lower leg MLLs (72%) and US for hip/thigh MLL (67%)(Fig.1.2). MLL size was larger for hip/thigh MLL (168 cm3) as compared to leg MLL (38.6 cm3). 25 (65.8%) of MLLs were treated with conservative management,12 (31.5%) with minimally invasive methods and 1(2.6%) needed surgical management. 2/9 (22.2%) of the associated injuries required operative management. 2/38 (5%) patients were given prophylactic antibiotics. 72.4% Lower leg MLLs were treated with conservative management while intervention was needed more in hip/thigh MLLs (55.6%). Patients returned to activities at 14.3 weeks for hip/thigh MLL;9.1 weeks for lower leg MLL. 24 patients with adequate documentation demonstrated lower extremity functional score (LEFS) and pain level of 74/80 (92.5%) and 0.7/10 respectively at mean 12.5 months follow-up. Leg cellulitis was seen as a complication in one patient. Conclusion: The largest case series on MLL exclusively in the pediatric population suggests that they are more common in knee/leg region region and usually caused by sports injuries which is notably different as compared to adult population. Most pediatric injuries are treated conservatively, especially sports related MLLs. Although return to activities takes longer for hip/thigh injuries, majority of patients regain satisfactory functionality post-injury. [Table: see text][Figure: see text]


2013 ◽  
Vol 61 (5) ◽  
pp. 488 ◽  
Author(s):  
ArunGrace Roy ◽  
KollencheriPuthenveettil Vinayan ◽  
Anand Kumar

2019 ◽  
Vol 24 (3) ◽  
pp. 246-257 ◽  
Author(s):  
Jennifer L. Quon ◽  
Lily H. Kim ◽  
Peter H. Hwang ◽  
Zara M. Patel ◽  
Gerald A. Grant ◽  
...  

OBJECTIVETransnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors.METHODSThe authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon.RESULTSThe series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1–120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8–120 months). Most patients (76%) were offered adjuvant therapy.CONCLUSIONSIn this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.


Author(s):  
Vadlakonda Sruthi ◽  
Annaladasu Narendra

Background: Tramadol use has been increasing in the adult and pediatric population. Practitioners must be alert because Tramadol misuse can lead to severe intoxication in which respiratory failure and seizures are frequent. Overdoses can lead to death. We report 47 pediatric cases with history of accidental tramadol exposure in children.Methods: An observational, retrospective, single center case -series of children with a history of accidental tramadol exposure in children admitted in pediatric intensive care unit of tertiary care center, Niloufer Hospital (Osmania Medical College) Hyderabad, Telangana India.Results: Of 47 children, 22 (47%) are male and 25 (53%) were female. At presentation 11 (23%) had loss of consciousness, 14 (29%) seizures, 17 (36%) hypotonia was noted. Pupils were miotic in 22 (47%) mydriatic in 2 (4.2%) normal in rest of children. Hemodynamic instability noted in 13 (27.6%). Serotonin syndrome (tachycardia, hyperthermia, hypertension, hyper reflex, clonus) was noted on 5 (10.6%) children. Respiratory depression was seen in 4 (8%) children who needed ventilatory support. Antidote Naloxone was given in 7 children. No adverse reaction was noted with Naloxone. All 47 children were successfully discharged.Conclusions: Overdoses can lead to death and practitioners must be alert because of the increasing use of tramadol in the adult and pediatric population. The handling of the tramadol should be explained to parents and general population and naloxone could be efficient when opioid toxicity signs are present.


2021 ◽  
Vol 100 (3) ◽  
pp. 193-199
Author(s):  
D.G. Akhaladze ◽  
◽  
G.S. Rabaev ◽  
N.V. Zhukov ◽  
M.A. Pyatnitskiy ◽  
...  

The level of tumor mutation burden (TMB) is a predictive factor of immune checkpoints that determines the potential effectiveness of immune checkpoint inhibitors and indications for their prescription regardless of the type of tumor in adults and children. However, the prevalence of tumors with high TMB in the pediatric population has not been well studied. Objective of the research: to assess the detection frequency of high TMB (>10 mutations per megabase) in pediatric hepatocellular carcinoma (HCC). Materials and methods of research: Next Generation Sequencing, Ion AmpliSeq™ Comprehensive Cancer Panel (409 genes) of tumor DNA samples from 4 children with HCC was performed. The calculation of the mutational load was carried out according to the work of Z.R. Chalmers et al. Results: out of 4 analyzed samples, TMB levels have lower cut-off value than needed for the immunity checkpoint inhibitors for 2 patients to administer: 5,9 mut/MB (primary tumor), 9,2 mut/MB (metastasis), while in 2 other patients the TMB has level above the threshold – 10,9 mut/MB (primary tumor) and 48,5 mut/MB (relapse metastasis), respectively. Conclusion: this paper presents the initial experience of estimation of the TMB level in children with HCC. In our case series report, the level allowing the prescription of immunotherapy (>10 mut/MB) was observed in 2 patients. Further research on a larger cohort of patients is useful to assess the role of mutational burden in disease prediction and effectiveness of immunotherapy.


2008 ◽  
Vol 132 (7) ◽  
pp. 1079-1103 ◽  
Author(s):  
Megan K. Dishop ◽  
Supriya Kuruvilla

Abstract Context.—Primary lung neoplasms are rare in children, but they comprise a broad and interesting spectrum of lesions, some of which are familiar from other tissue sites, and some of which are unique to the pediatric lung. Objective.—To determine the relative incidence of primary and metastatic lung tumors in children and adolescents through a single-institution case series, to compare these data to reports in the medical literature, to discuss the clinical and pathologic features of primary tumors of the tracheobronchial tree and lung parenchyma in children, and to provide recommendations for handling pediatric lung cysts and tumors. Data Sources.—A 25-year single institutional experience with pediatric lung tumors, based on surgical biopsies and resections at Texas Children's Hospital from June 1982 to May 2007, an additional 40 lung tumors referred in consultation, and a review of the medical literature. Conclusions.—A total of 204 pediatric lung tumors were diagnosed at our institution, including 20 primary benign lesions (9.8%), 14 primary malignant lesions (6.9%), and 170 secondary lung lesions (83.3%). The ratio of primary benign to primary malignant to secondary malignant neoplasms is 1.4:1:11.6. The common types of lung cancer in adults are exceptional occurrences in the pediatric population. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumor. Other primary pediatric lung tumors include congenital peribronchial myofibroblastic tumor and other myofibroblastic lesions, sarcomas, carcinoma, and mesothelioma. Children with primary or acquired immunodeficiency are at risk for Epstein-Barr virus–related smooth muscle tumors, lymphoma, and lymphoproliferative disorders. Metastatic lung tumors are relatively common in children and also comprise a spectrum of neoplasia distinct from the adult population.


1996 ◽  
Vol 11 (S2) ◽  
pp. S35-S35
Author(s):  
Anthony Ciccone ◽  
Carol Irving ◽  
Robert F. Lavery ◽  
Bartholomew J. Tortella

Purpose: The prevalence of asthma in the pediatric population is approximately 9% and the incidence of acute exacerbation in this population has been increasing. The purpose of this study was to determine if there has been a change in the pre-hospital presentation and treatment of pediatric asthma.Methods: This observational study was a retrospective consecutive case series comparing pediatric (#18 yrs) asthmatics treated and transported by an inner city EMS system over two years. (1987, 1992). Data collected included patient demographics, prehospital treatment, and evaluated diagnostic criteria used by paramedics to initiate treatment. Students t test was performed for continuous data and contingency analysis (chisquare) for non-continuous data. A Wilks stepwise discriminant analysis was performed on 1987 and 1992 data to evaluate diagnostic factors.Results: There were 407 transports in 1987 and 652 in 1992, representing a 60% increase. 237(56%) of patients received advanced life support (ALS) in 1987 and 344(53%) in 1992. Mean age of ALS patients in 1987 was 10.25 (±5.3) and was 8.03 (±2.8) in 1992 (p <0.001). Albuterol inhalation supplanted epinephrine (1987-205, 1992-1) and aminophylline (1987-15, 1992-1) as the treatment for asthma in our service system. The discriminant analysis revealed that in 1987, paramedics treatment decision making was influenced by age, cough, cold, and/or fever, home medications, accessory muscles, absence of wheezing, and normal respiratory rate. This changed in 1992 where only the presence or absence of wheezing and accessory muscle-use to be highly correlated with treatment decisions.


Vaccines ◽  
2019 ◽  
Vol 7 (3) ◽  
pp. 65
Author(s):  
Chisholm ◽  
Howe ◽  
Best ◽  
Petousis-Harris

Pertussis vaccines have been effective at reducing pertussis-associated morbidity and mortality. However, they have a complex array of limitations, particularly associated with the duration of protection against clinical disease and imperfect immunity (carriage and transmission). Little is known about risk factors for pertussis vaccination failure. Understanding pertussis vaccination failure risk is most important in the paediatric population. This study aims to investigate risk factors for pertussis vaccination failure in (1) infants between birth and six weeks of age born to mothers who received pertussis booster vaccinations during pregnancy and (2) infants after the completion of the primary series (approximately five months old) to four years old. This will be achieved in a two-step process for each study group. Pertussis vaccination failure cases will first be described using a case series study design, relevant case characteristics will be sourced from six national administrative datasets. The case series study results will help select candidate risk factors (hypothesis generating step) to be tested in the retrospective cohort study (hypothesis testing step. Pattern analysis will be used to investigate risk factor patterns in the cohort study. The identification of higher risk groups enables targeting strategies, such as additional doses, to better prevent pertussis disease.


Sign in / Sign up

Export Citation Format

Share Document