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Paediatric Surgery
Prune Belly Syndrome
Built from Puri — Pediatric Surgery and Pediatric Urology
What’s inside
10 sections · 78 slides
Overview
- What this deck will teach you
The Big Picture
What PBS is, its names, and how common it is
- Start here: what is prune-belly syndrome?
- The names, and a little history
- How common is PBS?
- Who gets it — and a falling incidence
What Causes PBS?
Genetics and the competing developmental theories
- The genetics: mostly sporadic, occasionally inherited
- Theory 1: an early fetal urethral obstruction
- Theory 2: a primary mesodermal defect
The Urinary Tract
Kidneys, ureters and bladder
- The kidneys: dysplasia and dilation
- Renal dysplasia with a dilated, refluxing megaureter
- The ureters: wide, tortuous and poorly peristaltic
- The bladder: massively enlarged with a urachal remnant
- Urachal diverticulum and enlarged bladder in PBS
- How well does the PBS bladder work?
Prostate, Urethra and Testes
The rest of the genitourinary picture
- Prostate and accessory sex organs
- Anterior urethra and penis: watch for megalourethra
- Vesicostomy and scaphoid megalourethra
- The testes: high, intra-abdominal and undescended
- Intra-abdominal testes overlying dilated ureters
- Fertility and cancer risk in PBS testes
The Abdominal Wall and Beyond
The floppy belly and its knock-on effects
- Abdominal wall deficiency: the visible hallmark
- Why the floppy belly matters day to day
- Variable abdominal wall flaccidity
- Severe flaccidity with in-utero limb amputation
- Associated abnormalities: PBS is a whole-body disorder
- Non-urologic abnormalities associated with PBS
- Orthopedic problems
- Pulmonary problems
- Thoracic deformity after reconstruction
- Gastrointestinal and cardiac associations
- A key newborn priority
Diagnosis and Classification
From the prenatal scan to the three severity categories
- Prenatal diagnosis
- Should we intervene before birth?
- Neonatal presentation and the three categories
- The spectrum of PBS: three categories
- Category I: the severe, often lethal form
- Category II: the intermediate, most problematic form
- Category III and other presentations
Evaluation and Medical Management
Working the newborn up and steering by category
- The newborn work-up: breathing and heart first
- Laboratory assessment and a key prognostic number
- Imaging the urinary tract safely
- Management by category
- The case for surgery in Category II
Surgical Treatment
Reconstructing the tract, bringing down the testes, rebuilding the wall
- Three surgical objectives
- Temporary urinary diversion
- Ureteral reconstruction
- Reduction cystoplasty
- Improving bladder outflow
- Surgical repair of scaphoid megalourethra
- Orchidopexy: bringing the testes down
- One-stage Fowler-Stephens orchidopexy
- Abdominal wall reconstruction: the principle
- Abdominal wall reconstruction: the techniques
- Abdominoplasty: skin resection
- Abdominoplasty: fascial overlap closure
- Abdominoplasty: umbilical and skin closure
- Putting it together: comprehensive reconstruction
- Excretory urogram before and after reconstruction
Outlook and Key Messages
How these children do, and what to remember
- The long-term outlook
- Key outcome numbers
- Conclusions and future directions
- Three things to remember
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- References (5/5)
- Pediatric Surgery and Pediatric Urology