This information has beenwritten for the parents ofTOF children by TOFS(Tracheo-OesophagealFistula Support) – helpingchildren born unable toswallow.

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VACTERL - renal anomaliesText created in association with Dr David Milford,consultant nephrologist at Birmingham Children’s Hospital.

Renal problems in VACTERL varygreatly – from those which will have noeffects on health to abnormalities whichare incompatible with life. It is thereforenot possible to make blanket statementsabout the importance of renal anomaliesto the child, and this leaflet thereforedescribes the various possibilities in turn.

All of the problems described also occurin children who do not have VACTERLas they are abnormalities of very earlykidney development that may occur inchildren without any other problems.

Some kidney defects may be detectedduring antenatal scans – however this isusually only the case if detailed scanningis performed (e.g. if there is somesuspicion that the unborn baby is likely tohave problems and additional scans arecarried out). Routine scans are relativelyunlikely to pick up kidney anomalies.

The normal kidneysA normal, healthy individual has twokidneys; one on each side of the body.These organs continuously filter the blood,removing some substances and balancingthe concentration of others. The kidneysalso have key roles related to control ofwater balance and blood pressure.

The fluid produced by the kidneys is knownas urine. Urine travels from the kidneys tothe bladder through a muscular tube called aureter and is stored until the bladder isemptied through the single urethra.

Bilateral renal agenesisThis condition describes the situationwhere neither kidney is present (‘bilateral’relates to ‘both sides’ and ‘agenesis’ means‘failure to form’). Because urine producedby the baby in the womb contributes tothe amniotic fluid (‘waters’) whichsurround it, and adequate quantities ofamniotic fluid are essential for lung andlimb development, a lack of kidneys canresult in a marked underdevelopment ofthe lungs and abnormalities of limbposition. These babies usually die soonafter birth because of breathingdifficulties or severe kidney failure.

Unilateral renal agenesis‘Unilateral’ means ‘one sided’ – so in thiscondition one kidney has failed to form.So long as the remaining kidney functionsand remains healthy, this will cause noproblems – we don’t really need two at all.

If a newborn baby is found to have onlyone kidney, the ultrasound scans of theremaining kidney are examined closely. Ifproblems are found, then further tests maybe required. Even if the kidney appearshealthy there may be an abnormality of thejunction between the bladder and theureter allowing urine to flow back towardsthe kidney (‘vesicoureteric reflux’).Because this can lead to kidney damage ifthere is a urine infection it is important toexclude this problem at an early stage toprevent infections damaging the oneremaining kidney (see separate entry).

It is useful to know if a person only hasone kidney, and on which side of the bodyit is ... if an injury is sustained, thisinformation may be useful to doctors. Amedical identity bracelet/necklace shouldbe worn, such as those from ‘Medicalert.’

Hypoplastic ordysplastic kidneys‘Hypoplasia’ refers to inadequate develop-ment i.e. ‘small in size’. Kidneys which aresmall in size at birth are frequentlyreferred to as ‘hypoplastic’ but the term‘dysplastic’ (related to disordered or faultydevelopment) is frequently more accurate.

If only one kidney is affected, there is noproblem – the remaining one can cope. Ifboth are affected, the consequences willdepend on the amount of functioningtissue remaining. This may be determinedby an isotope scan (DMSA scan) whichindicates the distribution and amount offunctioning kidney tissue. If kidneyfunction is inadequate, the child may besuitable for dialysis and a kidney transplant.

Horseshoe kidneyAn individual with a horseshoe kidneyhas two kidneys which have fused theirlower poles while the upper part remainsseparate. This does not usually cause any

I f a w h y n o t j o i n u s ?Information availablefrom either TOFS officeor the TOFS web site. T O F S

Related leaflets fromTOFS which you mightlike to read:1. Conditions occurring

with TOF/OA2. VACTERL -

an overview3. VACTERL -

vertebral anomalies4. VACTERL -

anal anomalies5. VACTERL -

cardiac anomalies6. VACTERL -

limb anomalies

These are all availablefrom the TOFS web site(www.tofs.org.uk)or from TOFS office.

TOFS also publishes abook, ‘The TOF Child,’which is suitable for bothparents and medicalprofessionals. Details areavailable from TOFS.

problems although it canoccasionally be associatedwith vesicoureteric reflux(see separate entry) orobstruction to urinary flow.Sometimes there may bedilatation of the uretersuggesting obstruction toflow; this is usually causedby the abnormal route theureter has to take with aconsequent delay inemptying the ureter.

Crossed renalectopiaIn this condition bothkidneys lie on the same sideof the body, one kidneylocated beneath the other ...the ureter belonging to theabnormally located kidneycrosses back to join thebladder in its normal location. Sometimesthere is fusion between the two kidneys.

Crossed renal ectopia is not usuallyassociated with any clinical problemsalthough the abnormally placed kidneymay be felt as a lump in the abdomen.

Duplex kidneyA duplex kidney is one which is split intotwo separate units, each with its own ureter(ureteric duplication). Duplex kidneys canbe present on one or both sides.

Sometimes only one unit is functional; theureter from the non-functioning unit mayshow gross vesicoureteric reflux (seeseparate entry). Another possibility is that aureterocoele forms. This is a thin layer oftissue over the opening of the ureter thatcan obstruct it completely or, morecommonly, allow urine out through a smallhole in the tissue layer causing a balloon-like structure to appear in the bladder.Surgery is often required to allow freedrainage of urine and to reduceobstruction to urine flow out of the ureter.

Cystic dysplastic kidneyThis is sometime wrongly called apolycystic kidney (a term which shouldbe reserved only for a specific, geneticcondition which develops with age). Itrefers to a kidney which has developedwrongly (is ‘dysplastic’) and containsmultiple cystic structures and abnormaltissue types e.g. cartilage, reflecting theunderlying developmental abnormality.

The consequences dependon how much functioningrenal tissue remains.Sometimes a badly cystickidney will shrivel and evendisappear; if this happensbefore birth it may appear tobe a case of unilateral renalagenesis. Some surgeonsremove ‘shrivelled’ kidneyremnants, however othersbelieve this unnecessary.

VesicouretericrefluxThis is a condition whereurine flows back up theureters, from the bladdertowards the kidneys. It isassociated with a highincidence of urine infections– which is its importance, forin a young baby this can lead

to kidney scarring. Scarring has twoconsequences: firstly, it represents a loss offunctional kidney tissue; secondly, the remainingkidney tissue is required to undertake extra workand this may cause damage through over-work,resulting in a further loss of kidney tissue. Thelatter can lead to kidney failure. High bloodpressure commonly accompanies kidney scarringbecause the damaged kidney tissue produces ahormone which increases blood pressure.

Children of up to three or four years who havereflux are given low dose antibiotics to reducethe incidence of urine infections as this is themost vulnerable age for developing kidney scars.

Reflux can be found in the presence or absence ofpre-existing kidney abnormalities.

Ureteric obstructionPelviureteric obstruction may be present as adevelopmental anomaly; this restricts the flowof urine from the kidney. If severe, thenarrowed section of the ureter that joins thepelvis must be surgically removed.

Any obstruction to urine flow may damage thekidneys and must be dealt with effectively.

Follow upLong term monitoring of children is onlyreally necessary in five circumstances:

• if impaired kidney function is present.• If kidney scarring is extensive.• if high blood pressure is present.• if there is vesicoureteric reflux.• if there is obstruction.