Complicated airway management in a child with prune‐belly syndrome

Barış, Sibel; Karakaya, Deniz; Üstün, E.; Tür, Ayla; Rizalar, Rıza

doi:10.1046/j.1460-9592.2001.00712.x

Cited by 10 publications

(7 citation statements)

References 15 publications

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“…In addition, respiratory infections are common due the inability to cough effectively and consequent retention of secretions [10]. Finally, PBS patients have been noted to have micrognathia, which may result in difficulties with intubation [12]. …”

Section: Discussionmentioning

confidence: 99%

Impact and frequency of extra-genitourinary manifestations of prune belly syndrome

Grimsby

Harrison

Granberg

et al. 2015

Journal of Pediatric Urology

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Summary Introduction Extra-genitourinary (extra-GU) manifestations are serious comorbidities beyond the genitourinary (GU) anomalies of prune belly syndrome (PBS). It was hypothesized that there is an underestimation of the reported frequency and understated impact on quality of life (QOL) of extra-GU comorbidities in people with PBS who survive the newborn period. To assess this, the extra-GU manifestations of PBS in a contemporary cohort of living patients were compared to those that have previously been described in the literature. Second, the impact of extra-GU manifestations of PBS on the QOL of the patients and their families were assessed via a non-validated open-ended survey. Methods From 2010-2013, living people with PBS were prospectively recruited at Children's Health Dallas or at three PBS Network National Conventions. The family/subject was asked to complete a detailed PBS questionnaire, non-validated QOL survey, and provide medical records for review. Clinical data were extracted from medical records for local patients. The frequency of extra-GU manifestations was compared between the contemporary, living cohort and a historical cohort derived from PubMed publications. Results Of the 706 identified publications on PBS, seven historical studies that were published a mean of 30 years ago met inclusion criteria and tabulated the frequency of extra-GU PBS manifestations. Clinical data from the present institution were available for 65 living patients: 99% were male and the mean age was 10 years (1 month-45 years). They had a statistically significantly higher incidence of gastrointestinal (63%), orthopedic (65%), and cardiopulmonary (49%) diagnoses when compared with the historical cohort of 204 PBS subjects from the literature (Fig. 1). A total of 43 people completed the QOL survey: 11 males with PBS and 32 family members. Of these, 47% listed at least one non-GU problem (i.e. lung disease, skeletal problems, constipation) as negatively affecting their QOL; 42% listed at least one GU problem (i.e. self-catheterization, recurrent UTIs) as negatively affecting their QOL; 56% reported musculoskeletal surgery and 21% reported gastrointestinal surgery/medication as positively impacting on their QOL. Discussion In this largest reported series of living people with PBS, the frequency of gastrointestinal, orthopedic, and cardiopulmonary diagnoses was found to be significantly higher than previously published. These findings are very important for the urologist, as non-GU manifestations of PBS, such as cardiopulmonary disease and constipation, may affect the medical and surgical treatment of the PBS patient. The present study also sought to identify what aspects of PBS matter the most to those affected by and living with PBS. As expected, the QOL survey of PBS revealed that the majority felt that GU problems negatively affected their QOL. However, nearly 50% of the respondents indicated that a non-GU aspect of the disease also had a negative effect on QOL and that treatment of these conditions improved ...

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Section: Discussionmentioning

confidence: 99%

Impact and frequency of extra-genitourinary manifestations of prune belly syndrome

Grimsby

Harrison

Granberg

et al. 2015

Journal of Pediatric Urology

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“…Prune-Belly sendromlu hastalarda larinksin pozisyonunun sefale yönlenmesi ve yüzdeki anatomik değişikliler nedeniyle zor hava yolu olguları bildirilmiştir. 9 Hastalarımızda hava yolu yönetimi açısından bir zorlukla karşılaşılmamıştır, fakat Prune-Belly sendromlu hastalarda zor hava yolu ile ilgili tüm hazırlıklar yapılarak anestezi indük-siyonuna başlanılmış, nöromusküler kullanılmadan hava yolu açıklığı kontrol edilmesinin önlem olabileceği düşünülmüştür. Bu nedenle birinci olguda nöromusküler bloker kullanılmamış, ikinci olguda ise kısa etkili ve sugammadex ile antagonize edilebilen roküronyum tercih edilmiştir.…”

Section: Discussionunclassified

“…Barış ve ark.nın olgu sunumlarında, üç saati geçmeyen olgularda laringeal maske kullanımının güvenli olduğu bildirilmiştir. 9 Prune-Belly sendromlu hastalarda, var olan anatomik güçlüklerin yanı sıra anestezi sırasında ve sonrasında da fonksiyonel rezidüel kapasite, total akciğer kapasitesinin azalmasının, ek olarak, pnömoperitonyum ve trendelenburg pozisyonu gibi etkenlerin atelektazi riskini arttırdığı bildirilmiştir. 10 Sunulan ilk olguda, laparoskopik cerrahi olması açısından postoperatif solunumsal komplikasyon görülme olasılığının artabileceği düşünülmüş, aynı zamanda bu dö-nemde rezidüel nöromusküler bloker etkisini ortadan kaldırmak, akciğer volümlerinin daha az etkilenmesini sağlamak amacıyla nöromusküler bloker kullanılmamıştır.…”

Section: Discussionunclassified

Anesthetic Management of Prune-Belly Syndrome: Case Report

Şeker¹,

Tütüncü²

2015

Turkiye Klinikleri J Case Rep

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“…Infants born to younger mothers are at greater risk, 12,13 and the incidence of PBS in twins is 4 times greater than in single births (12.2 per 100,000 live newborns). 14 Very limited data are available on the true incidence of HAAs.…”

Section: Discussionmentioning

confidence: 99%