Undescended Testis

Also known as: Cryptorchidism

Undescended testis, or cryptorchidism, occurs when one or both testes fail to reach the dependent scrotum.

The testis may lie:

Intra abdominal
Within the inguinal canal
At the external ring
Prescrotal

An ectopic testis has left the normal path of descent and may lie in the superficial inguinal pouch, perineum, femoral region or another abnormal position.

A retractile testis can be manipulated into the scrotum without tension and remains there temporarily. It is not a true undescended testis but requires surveillance because secondary ascent can occur.

Longstanding cryptorchidism is associated with:

Progressive germ cell loss
Reduced fertility potential
Testicular malignancy
Torsion
Associated inguinal hernia

Spontaneous descent after approximately 6 months corrected age is uncommon.

Examine in a warm room with the child relaxed.

Palpate from the anterior superior iliac spine toward the scrotum.

Classify as:

Palpable
Nonpalpable

A nonpalpable testis can represent:

Intra abdominal testis
Canalicular testis not appreciated clinically
Atrophic testicular remnant
Absent testis

Bilateral nonpalpable testes

This requires urgent assessment for a disorder of sex development, particularly when associated with:

Hypospadias
Micropenis
Ambiguous genitalia

Do not assume the child is simply a routine bilateral cryptorchid case.

Timing

If the testis remains undescended at 6 months corrected age, plan surgery.

Perform orchiopexy ideally between 6 and 12 months, and by 18 months at the latest. Earlier orchiopexy preserves germ cell potential better than delayed treatment.

Hormonal therapy should not be used routinely simply to induce descent of a unilateral undescended testis.

Palpable inguinal testis

Perform inguinal orchiopexy.

Key steps:

Mobilise the testis and spermatic cord
Divide restrictive cremasteric fibres
Identify and ligate a patent processus vaginalis
Mobilise cord sufficiently to obtain tension free scrotal placement
Create a subdartos pouch
Place the testis in the dependent scrotum without torsion

Do not stretch the testicular vessels excessively merely to reach the scrotum.

A low palpable testis can sometimes be treated through a single scrotal approach.

Nonpalpable testis

Perform examination under anaesthesia followed by diagnostic laparoscopy if still nonpalpable.

Possible findings include:

Blind ending vessels

Suggest vanishing testis.

Vas and vessels entering the internal ring

Explore the inguinal canal for a testis or remnant.

Intra abdominal testis

Management depends on distance from the internal ring and vascular length.

Low intra abdominal testis

When adequate vessel length exists, perform primary laparoscopic orchiopexy while preserving the native spermatic vessels.

High intra abdominal testis

When the testis cannot reach the scrotum on intact vessels, options include a Fowler Stephens strategy.

Two stage Fowler Stephens

Stage 1:

Divide the testicular vessels while preserving collateral flow from:

Deferential artery
Cremasteric vessels

Allow collateral circulation to develop.

Stage 2:

Approximately 6 months later, mobilise the testis into the scrotum.

Two stage treatment has a lower reported atrophy rate than one stage vessel division.

Preserving the gubernacular blood supply where feasible can further protect perfusion.

Testicular remnant

A clearly atrophic nonfunctional nubbin can be excised, particularly if intra abdominal.

Postpubertal undescended testis

Management changes after puberty.

A postpubertal intra abdominal testis with a normal opposite testis frequently undergoes orchiectomy because fertility benefit from orchiopexy is minimal while cancer risk remains significant.

Long term follow up

Orchiopexy reduces but does not eliminate future malignancy risk.

Teach testicular self examination after puberty.

Monitor for:

Reascent
Testicular atrophy
Hernia
Fertility problems, particularly bilateral disease

Clinical toolsCalculators

BMI GCS EDD Parkland Formula

Corrected Sodium Corrected Calcium Anion Gap Creatinine Clearance Insulin Calculations qSOFA Wells PE Wells DVT CHA₂DS₂-VASc HAS-BLED CURB-65 Centor/McIsaac

Hirschsprung Disease

Also known as: Hirschsprung's disease, Congenital aganglionic megacolon

Hirschsprung disease is a congenital enteric neuropathy caused by failure of neural crest derived ganglion cells to populate a variable length of distal intestine.

The aganglionic segment lacks ganglion cells in both:

Submucosal plexus
Myenteric plexus

This produces persistent tonic contraction of the affected bowel and functional distal obstruction.

The bowel proximal to the aganglionic segment becomes progressively dilated.

Anatomical extent

Short segment disease

Aganglionosis limited to rectum and sigmoid colon.

This is the commonest form.

Long segment disease

Aganglionosis extends proximal to the sigmoid.

Total colonic aganglionosis

The entire colon is affected, occasionally with distal small bowel involvement.

Total intestinal aganglionosis

Rare and severe.

The transition zone is the segment between normal ganglionated bowel and completely aganglionic bowel.

A pull through must not end within the histological transition zone.

Hirschsprung disease is associated with Down syndrome and several genetic conditions.

Typical neonatal presentation includes:

Delayed passage of meconium beyond 24 to 48 hours
Progressive abdominal distension
Bilious vomiting
Feeding intolerance

Digital rectal examination can produce explosive passage of stool and gas after withdrawal.

Some infants present with Hirschsprung associated enterocolitis, characterised by:

Abdominal distension
Fever
Lethargy
Explosive foul or watery stool
Sepsis
Shock

Older children can present with:

Severe lifelong constipation
Abdominal distension
Failure to thrive
Dependence on enemas
Faecal impaction

A history of constipation beginning after normal stooling for several years is less typical of Hirschsprung disease than constipation present from infancy.

Rectal biopsy

Diagnosis requires histological confirmation.

A representative rectal suction biopsy should be taken at least approximately 2 cm above the dentate line, and usually multiple suction samples are obtained to ensure adequate submucosa. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC13342790/))

Histology demonstrates:

Absence of ganglion cells
Hypertrophic nerve trunks

Ancillary staining can include:

Increased acetylcholinesterase activity
Absent or markedly reduced calretinin staining in aganglionic mucosa

An inadequate superficial biopsy should not be interpreted as negative disease.

Contrast enema

Useful for defining the probable level of transition.

Perform without prior aggressive rectal washout when possible because decompression can obscure the transition.

Contrast enema supports operative planning but does not replace rectal biopsy. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC13342790/))

Anorectal manometry

Absence of the rectoanal inhibitory reflex supports Hirschsprung disease.

It is most useful in older infants or children and does not replace histology when definitive surgery is planned.

Preoperative decompression

Use saline rectal irrigations approximately 1 to 3 times daily to decompress the colon while preparing for definitive pull through. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC13342790/))

Teach caregivers an effective irrigation technique before discharge when outpatient decompression is planned.

If adequate decompression cannot be achieved, reconsider:

Long segment disease
Incorrect irrigation technique
Severe enterocolitis
Stricture or another obstruction

Hirschsprung associated enterocolitis

This can become rapidly fatal.

Management includes:

Nil orally in significant disease
IV fluid resuscitation
Correction of electrolytes
Broad IV antibiotics
Repeated rectal irrigations

A practical severe infection regimen is:

Piperacillin tazobactam 80 to 100 mg/kg/dose of the piperacillin component IV every 6 to 8 hours, adjusted for age, renal function and local paediatric dosing standards.

An alternative is a third generation cephalosporin plus metronidazole.

Do not rely on antibiotics alone while the colon remains obstructed.

A stoma is indicated when:

Rectal irrigation fails to decompress the bowel
Severe enterocolitis fails to respond
Perforation occurs. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC13342790/))

Definitive pull through

The principles are:

Identify normally ganglionated bowel
Resect aganglionic bowel and transition zone
Bring well vascularised ganglionated bowel to the anus
Preserve the anal canal and sphincter complex

Common operations include:

Swenson type pull through

Full thickness rectal dissection with coloanal anastomosis.

Soave endorectal pull through

Mucosal dissection through a rectal muscular cuff.

Avoid an excessively long muscular cuff because it can cause postoperative obstruction.

Duhamel operation

Ganglionated colon is brought behind the aganglionic rectum and a common channel is created.

No technique has demonstrated universal superiority. Outcomes depend heavily on accurate pathology, meticulous technique and postoperative management. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC13342790/))

For uncomplicated rectosigmoid disease, a transanal pull through with or without laparoscopic assistance is common.

Elective pull through is usually performed when the child is stable, growing and well decompressed, often within approximately 2 to 3 months after diagnosis rather than requiring neonatal surgery in every case. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC13342790/))

Intraoperative pathology

Confirm ganglionated proximal bowel before completing the anastomosis.

A circumferential proximal specimen is more reliable than assuming that gross bowel calibre alone identifies normal bowel.

Postoperative obstruction

Persistent obstructive symptoms after pull through require structured evaluation for:

Anastomotic stricture
Twisted pull through
Long Soave muscular cuff
Duhamel spur or pouch
Transition zone pull through
Residual aganglionosis
Functional internal sphincter hypertonicity

Evaluation can include:

Rectal examination
Contrast enema
Review of original histology
Repeat biopsy

When anatomy and histology are normal, botulinum toxin injection into the internal sphincter can improve functional outlet obstruction in selected children. ([pmc.ncbi.nlm.nih.gov](https://pmc.ncbi.nlm.nih.gov/articles/PMC13342790/))

Long term bowel function

Follow for:

Enterocolitis
Constipation
Faecal soiling
True incontinence
Growth and nutrition
Psychosocial impact

Children who fail to achieve expected continence require formal colorectal reassessment rather than being labelled as having an unavoidable poor outcome.

Undescended Testis

Overview

Clinical Features

Diagnosis

Management

Overview

Clinical Features

Diagnosis

Management