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Anorectal malformation

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The most important points

  • Anorectal malformation is congenital and describes a clinical picture with a malformed or absent anus.
  • Newborns are examined for possible accompanying malformations.
  • Depending on the type of anorectal malformation, corrective surgery is performed at around 3 months of age or a temporary artificial bowel outlet is created in the neonatal period with corrective surgery and repositioning of the artificial bowel outlet at around 3 months of age.

Clinical picture

What is an anorectal malformation?

Anorectal malformation describes a congenital condition in which the anus is not properly formed. The anal opening is either too small and in the wrong place (so-called fistula) or no anal opening is visible. The anorectal malformation can be present in isolation or in combination with diseases of the kidneys, heart, oesophagus or other organs (so-called VACTERL association).

Symptoms

What are the typical signs of an anorectal malformation?

Newborns either have difficulty defecating through an opening that is too small and incorrectly positioned, or they are unable to defecate at all due to a missing opening.

Diagnosis

How is an anorectal malformation diagnosed?

The diagnosis is made by the paediatric surgeon who will examine your child on the first day of life. If it is an anorectal malformation, examinations of the heart (echocardiography), an ultrasound of the abdomen and an examination of the urine and spine are carried out to rule out concomitant diseases.

Therapy

How can an anorectal malformation be treated?

If your child has an incorrectly positioned anal opening, it is often possible for your child to defecate regularly with the help of a special calibrating rod (so-called Hegar pen), which is inserted into the opening once a day. In this case, the actual operation is planned at around three months of age.

If your child does not have an anal opening, an artificial anus must be temporarily inserted in the first few days of life. During the first three months of life, your child will defecate via the artificial anus.

The actual corrective surgery is performed in both cases at around three months of age. The anus is sutured into the correct position in the centre of the sphincter complex. If an artificial anus is present, this is moved back during the same anaesthetic.

Your child will then be monitored and checked by our team at regular intervals over several years.

Hospitalisation

What happens during my child's hospitalisation?

Hospitalisation in the neonatal period lasts approximately one to two weeks. After the corrective surgery at around three months of age, your child will be hospitalised for another five to seven days.

Forecast

What is the prognosis for an anorectal malformation?

The prognosis for later faecal continence depends mainly on the type of anorectal malformation your child has. Depending on the severity, the continence rate with correctly performed corrective surgery varies between approx. 55 % and
100 %.

Literature

Further reading

  • Hirschsprung Disease and Anorectal Malformations - An Uncommon Association. Journal of Pediatric Surgery 2020 May 16 Vuille-dit-Bille RN, De la Torre L, Peña A, Bischoff A.
  • European consensus meeting of ARM-Net members concerning diagnosis and early management of newborns with anorectal malformations. Tech Coloproctol. 2015 Mar;19(3):181-5. van der Steeg HJ, Schmiedeke E, Bagolan P, Broens P, Demirogullari B, Garcia-Vazquez A, Grasshoff-Derr S, Lacher M, Leva E, Makedonsky I, Sloots CE, Schwarzer N, Aminoff D, Schipper M, Jenetzky E, van Rooij IA, Giuliani S, Crétolle C, Holland Cunz S, Midrio P, de Blaauw I.
  • Practice of dilatation after surgical correction in anorectal malformations. Surg Int. 2012 Nov;28(11):1095-9. Jenetzky E, Reckin S, Schmiedeke E, Schmidt D, Schwarzer N, Grasshoff-Derr S, Zwink N, Bartels E, Rissmann A, Leonhardt J, Weih S, Obermayr F, Rädecke J, Palta M, Kosch F, Götz G, Hofbauer A, Schäfer M, Reutter H, Holland-Cunz S, Märzheuser S.

Responsible department

Who treats this clinical picture?

Counselling hotline for child and youth emergencies

The Medgate Kids Line provides quick and uncomplicated medical advice if your child is unwell. The medical team of our partner Medgate is available to you by telephone around the clock.

058 387 78 82
(billing via health insurance)

For emergencies abroad: Call the emergency number of your health insurance company. You will find this number on your health insurance card.

More information: On the Page of the emergency ward you will find everything you need to know about behaviour in emergencies, typical childhood illnesses and waiting times.

Important emergency numbers

144 Outpatient clinic
145 Tox Info Suisse (Poisonings)
117 Police
118 Fire brigade

UKBB

University Children's Hospital Basel
Spitalstrasse 33
4056 Basel | CH

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