Thoracoscopic surgery for congenital lung malformations: Does previous infection really matter?

Background/ PurposeElective resection of congenital lung malformations (CLMs) is still debatable. The two main risks are malignant transformation and recurrent pulmonary infections. Our study aimed to assess the effect of previous pulmonary infection on the intraoperative and postoperative courses of thoracoscopic surgery for CLMs.MethodsThis is a retrospective study including all thoracoscopic lung resections for CLMs between 2010 and 2019. Ninety patients were included. There was a history of previous pulmonary infection in 28 patients (group A) and no such history in 62 patients (group B).ResultsThe median age at operation for group A was 20.4 months (IQR:14.9–41.4) versus 15.1 months (IQR:9.7–20.8) in group B (p = 0.006). There were 10 conversions (35.7%) in group A and 8 (12.9%) in group B (p = 0.02). The operative time was significantly shorter in group B (p<0.002). In group A, 32.1% of patients experienced postoperative fever versus 11.3% of group B (p = 0.03), with higher antibiotics requirement (28.6% versus 6.5% respectively, p = 0.007). However, no significant differences were found in terms of postoperative complications (p = 0.99).ConclusionEarlier intervention for CLMs before the development of pulmonary infection carries higher chances for the success of the thoracoscopic approach with shorter operative time and more uneventful postoperative courses.     

Musculoskeletal congenital malformations: do paternal occupational exposures play a role?

Background

Paternal occupational exposures to potential health hazards are likely to affect congenital malformations through the spermatogenesis cycle.

Purpose

The aim of this case–control study was to assess the relationship between the risk of musculoskeletal congenital malformations in offspring and paternal workplace exposure to potential health hazards during the preconception period.

Method

The study comprised 105 patients (cases) with a musculoskeletal congenital malformation(s) and 135 controls matched for age and demographic characteristics. Both parents of each case and control were interviewed in the hospital by a trained physician. They also completed a questionnaire focusing on the preconception period and on the 3-month period immediately before and after the pregnancy conception date, respectively, of the child under study.

Results

The odds of having a child with a congenital malformation was higher (P < 0.05) if the father was occupationally exposed to pesticides, solvents, or welding fumes during the preconception period.

Conclusion

Control of workplace exposures and adherence to threshold limit values of these potential health hazards should be adopted to minimize the risk of fathers having offspring with a congenital malformation.     

Pure alcohol injection of a congenital splenic cyst: a valid alternative?

Congenital splenic cysts are a rare entity. They are more frequent in children and young adults. They are true cysts, lined by epithelium with a typical trabeculation. Aspiration and injection with antibiotics (minocycline-tetracycline) or pure alcohol has been reported sparsely with variable results. The purpose of this case study is to report our experience with ultrasound (US)-guided aspiration and injection of a congenital splenic cyst, which, in our case, did not prove a viable alternative to surgical defenestration.     

Fistula-in-ano in infants: is nonoperative management effective?

PURPOSE: Recently, a number of studies have reported positive results from the nonoperative management of fistula-in-ano in infancy, although it has not been of use in all patients. The purpose of this study was to discern the effective treatment methods of fistula-in-ano in infants. METHODS: A retrospective review was done of 310 children who required operative management for fistula-in-ano or perianal abscess between January 1991 and July 2000. Eighteen patients displayed an onset of symptoms at less than 1 year of age and a duration of symptoms longer than 12 months. The authors analyzed these patients' medical records. RESULTS: All patients were boys. The mean duration of the symptoms was 26.6 +/- 27.5 months. Fourteen patients had shown an onset of symptoms at less than 6 months of age. The longest duration was 10 years. The patients showed conservative periods of over 12 months because their parents did not want them to undergo surgery. The disease in these patients followed 2 patterns. One (6 patients) was an onset of symptoms followed by a silent fistula-in-ano state. The other (12 patients) was an onset of symptoms followed by an intermittent relapse of inflammation. All patients underwent fistulotomy, and none of them had recurrent fistula during the follow-up period. CONCLUSIONS: Although the advantages of a nonoperative management of fistula-in-ano in infants include the avoidance of general anesthesia and surgical intervention, the lesions cannot be cured by a period of conservation. Surgical management is more effective in respect to the time factor.     

Is congenital lobar emphysema a surgical disease?

Background

The management of congenital lobar emphysema has traditionally been surgical. Because of increased use of imaging, this lesion is frequently found in asymptomatic and mildly symptomatic children, prompting us to adopt a more conservative approach to these children.

Methods

All children with congenital lobar emphysema presenting between 1995 and 2002 were included. Medical records, imaging files, and pathology reports were reviewed.

Results

Twenty children (0-17 years) were identified. Eight were diagnosed antenatally. Fourteen were managed without surgery. Of the 11 symptomatic children, 6 showed spontaneous improvement.

Conclusions

The favorable outcome of both asymptomatic and mildly symptomatic children suggests that a nonoperative approach should be considered in these patients.     

Endoscopic management of a low-grade thalamic glioma: a safe alternative to open microsurgery?

Background

Despite considerable advances in preoperative and intraoperative imaging and neuronavigation, resection of thalamic gliomas remains challenging. Although both endoscopic biopsy and third ventriculostomy (ETV) for the treatment of secondary hydrocephalus are commonly performed, endoscopic resection of thalamic gliomas has been very sparsely described.

Method

We report and illustrate the surgical procedure and patient’s outcome after full endoscopic resection of a thalamic glioma and to discuss this approach as an alternative to open microsurgery.

Results

In 2016, a 56-year-old woman presented with disorientation, dysphasia and right facial hypaesthesia in our department. Cranial magnetic resonance imaging revealed a left thalamic lesion and subsequent hydrocephalus. Initially, hydrocephalus was treated by ETV but forceps biopsy was not diagnostic. However, metabolism in ¹⁸F-fluoroethyl-L-tyrosine positron emission tomography indicated glioma. Subsequently, endoscopic and neuronavigation-guided tumour resection was performed using a <1 cm², trans-sulcal approach through the left posterior horn of the lateral ventricle. While visibility was poor using the intraoperative microscope, neuroendoscopy provided excellent visualisation and allowed safe tumour debulking. Neither haemorrhage from the tumour or collapse of the cavity compromised endoscopic resection.

Conclusions

In accordance with one previously published case of endoscopic resection of a thalamic glioma, no surgery-related complications were observed. Although this remains to be determined in larger series, endoscopic resection of these lesions might be a safe and feasible alternative to biopsy or open surgery. Future studies should also aim to identify patients specifically eligible for these approaches.

     

Is lung biopsy useful for surgical decision making in congenital heart disease?

Sixty patients with congenital heart disease (CHD), age range 3 months-45 years (median 2 years), underwent lung biopsy to exclude pulmonary vascular disease (PVD): 25 had an atrioventricular (AV) septal defect, 14 a ventricular septal defect (VSD), 7 a complete transposition of the great arteries (TGA) + VSD, 3 an atrial septal defect, and 2 a patent ductus arteriosus; 9 had other malformations. Scoring of histological section (0-4) according to a modified Heath-Edwards classification disclosed that 30 patients had severe "irreversible" PVD (greater than or equal to grade 3) (11 AV septal defect, 8 VSD, 6 TGA + VSD and 5 others); 8 patients were younger than 1 year. Pulmonary vascular resistance (PVR) was calculated in 51 patients and exceeded 7 U/m2 in 1 of 2 patients with grade 0, in 9 of 18 with grade 1, in 1 of 4 with grade 2, in 11 of 18 with grade 3, and in 8 of 9 with grade 4. PVR was also calculated after 100% oxygen administration in 19 patients, 14 of whom had a resting PVR greater than or equal to 7 U/m2. PVR persisted greater than or equal to 7 U/m2 in 5 patients: 4 had PVD greater than or equal to grade 3 and died after surgery. PVR fell to under 7 U/m2 in 14 patients, 3 of whom had PVD greater than or equal to grade 3:1 patient with grade 4 was not operated upon and 2 with grade 3 were operated upon: all are alive. Follow-up in discharged living patients was 100%, with a mean of 50 months. Fifty-five patients underwent surgery with 10 early and 2 late deaths. Among the 12 subjects with a fatal outcome, 10 had irreversible lesions. The 5 non-operated patients all had irreversible lesions at biopsy, and are still alive.(ABSTRACT TRUNCATED AT 250 WORDS)     

Results of biventricular repair of congenital cardiac malformations: definitive corrective surgery?

Objectives: Congenital cardiac malformations are usually corrected in the neonatal period or in early infancy. Corrective surgery may not always be definitive, especially in complex malformations. Long-term morbidity is influenced by reoperations and their risk. Methods: This study analyzes our single-center experience over more than 20 years in a selected group of patients. Data were gathered, with special focus on causes and incidence for reoperations, respectively. Results: Freedom from reoperation after 5, 10, and 15 years for each cardiac malformation was determined. The numbers describe in the following order patient years (y), number of patients (n), and freedom from reoperation at follow-up interval (%), respectively: atrial septal defect (15 864y, n=1198, 99±0/99±0/99±0), partial atrioventricular septal defect (2506y, n=234, 95±2/93±2/93±2), total anomalous pulmonary venous connection (742y, n=141, 93±1/91±0/91±0), complete atrioventricular septal defect (1715y, n=377, 81±3/76±3/72±4), tetralogy of Fallot <1 year (1503y, n=197, 94±4/85±1/74±3), transposition of great arteries (1459y, n=375, 88±2/83±4/73±7), interrupted aortic arch (IAA) (481y, n=98, 63±6/52±7/45±8), common arterial trunk (CAT) (599y, n=109, 64±6/24±6/11±5). Conclusions: In most congenital malformations surgical correction is definitive and the rate of reoperations is low. In complex anomalies, such as CAT and IAA, reoperations at long-term are more common. Analysis of such results and recognition of a sometimes inevitable operative morbidity helps to predict long-term outcome and influences the follow-up.     

Is repeated radiosurgery an alternative to staged radiosurgery for very large brain arteriovenous malformations?

OBJECT: The results of a novel radiosurgical approach to treat large arteriovenous malformations (AVMs) with repeated radiosurgery are presented and discussed. METHODS: The outcome was studied following repeated Gamma Knife surgery (GKS) for large AVMs, defined as a nidus volume of 9 ml or more. The philosophy was to treat the whole AVM with a low dose of radiation (> or = 10 Gy), and to repeat the treatment if the AVM shrank but was not obliterated. The study included 133 patients with AVMs treated at one of three different institutions. Clinical information was available for all patients, and complete radiological follow-up was available in 89 patients after the first treatment, and in 19 after the second treatment. RESULTS: The estimated obliteration rate following repeated GKS was 62%. Four patients (3%) developed neurological deficits caused by the radiation, whereas five others (4%) developed cystic changes. The annual incidence of hemorrhage was high (7%), of which 35% occurred within the 1st year after the first treatment. CONCLUSIONS: Repeated radiosurgery seems to be a viable option for some AVMs considered to be too large for conventional radiosurgical treatment. The incidence of posttreatment hemorrhages seems to be a larger clinical problem than radiation-induced complications.     

Asymptomatic primary hyper-N-acetyl-beta-D-glucosaminidaseuria: a new clinical entity?

Urinary N-acetyl-beta-D-glucosaminidase (NAG) is excreted from renal tubular cells and increased urinary excretion reflects renal tubular damage. There are many causes for the elevation of urinary NAG; however, primary elevation of urinary NAG (primary hyper-NAGuria) has never been reported. Recently, we encountered two siblings with continuously elevated urinary NAG levels that could not been explained by any known causes. Two brothers, aged 13 and 11 years, were found to have continuously elevated urinary NAG levels that were repeatedly confirmed by urinary NAG levels (both per liter and urinary NAG/creatinine ratios). However, simultaneously measured urinary beta(2)-microglobulin levels were always within normal ranges. The elder brother is a heterozygous carrier for familial hypercholesterolemia, although this was not attributable to the urinary NAG elevation. The two patients were receiving no drugs when they were first examined at our hospital, and no nephrotoxic substances were found to be the cause for the elevation. Renal biopsy revealed no abnormal findings attributable to the abnormally high excretion of urinary NAG. Our two patients are considered to have asymptomatic primary hyper-NAGuria, which has not been previously reported. Although more cases are needed, this asymptomatic primary hyper-NAGuria is probably a new clinical entity of renal tubular disorders.     

Medical management of stress urinary incontinence: Is there a future?

Stress urinary incontinence (SUI) is a common problem among women worldwide. Multiple treatment modalities exist, ranging from physiotherapy to surgery. Numerous reports demonstrate mixed results for efficacy and safety of several oral agents used to treat SUI. Although there are data suggesting reasonable efficacy for several medications, surgery still remains the mainstay of treatment for most women. This article reviews the available oral agents that have been studied and assesses the data supporting their use while highlighting the limitations of each.