Neonatal Surgical IssuesNeonatal Surgical Issues(Part 2)(Part 2)

Sue Ann Smith, MDSue Ann Smith, MD

NeonatologistNeonatologist

Anatomic survey (cont)Anatomic survey (cont)

Intestinal obstructionsIntestinal obstructionsGenitourinaryGenitourinaryAbdominal massesAbdominal masses Inguinal herniaInguinal herniaTesticular torsionTesticular torsionNeurosurgicalNeurosurgical

Intestinal obstructionsIntestinal obstructions

Atresias – duodenal, jejunal, colonicAtresias – duodenal, jejunal, colonicMeconium ileusMeconium ileusMeconium plugMeconium plugHirschsprung’s diseaseHirschsprung’s disease Imperforate anusImperforate anusMalrotation with volvulusMalrotation with volvulusAdhesions and stricturesAdhesions and strictures

AtresiasAtresias

Double bubble of duodenal atresiaHigh obstruction of jejunal atresia

Atresias (cont)Atresias (cont)

Duodenal atresia associated with trisomy Duodenal atresia associated with trisomy 21, or other anomalies21, or other anomaliesHx of polyhydramniosHx of polyhydramniosDelee more than 30-50ml from stomachDelee more than 30-50ml from stomach

Jejunal atresia is rarely associated with Jejunal atresia is rarely associated with other anomalies. other anomalies.

Decompress proximal bowel with repogle Decompress proximal bowel with repogle tube to low continuous suctiontube to low continuous suction

Meconium IleusMeconium Ileus

Obstruction of bowel by thick tenacious Obstruction of bowel by thick tenacious meconiummeconium

30% of intestinal obstruction in neonates30% of intestinal obstruction in neonatesFrequent cause of meconium peritonitisFrequent cause of meconium peritonitisMost are associated with cystic fibrosis Most are associated with cystic fibrosis

(but only 15% of infants with CF will have (but only 15% of infants with CF will have meconium ileus)meconium ileus)

Abdominal distention is typically present at Abdominal distention is typically present at birthbirth

Meconium Ileus (cont)Meconium Ileus (cont)

Diagnosis made with contrast enemaDiagnosis made with contrast enemaGastrograffin enema with aggressive Gastrograffin enema with aggressive

hydration can be used to treat somehydration can be used to treat someOperative evacuation of meconiumOperative evacuation of meconiumMay require ostomyMay require ostomyProximal bowel dilated and distal bowel Proximal bowel dilated and distal bowel

may be very small (microcolon) and may be very small (microcolon) and require time to dilate with userequire time to dilate with use

Meconium plugMeconium plug

Difference between meconium ileus and Difference between meconium ileus and meconium plug is site and severity of obstructionmeconium plug is site and severity of obstruction

Preterm infants, infants of diabetic mothers, Preterm infants, infants of diabetic mothers, IUGR babies, otherwise ill babiesIUGR babies, otherwise ill babies

Treatment with glycerin suppositories and warm Treatment with glycerin suppositories and warm saline enemassaline enemas

May require contrast enema to make diagnosisMay require contrast enema to make diagnosis Normal stooling pattern should follow evacuation Normal stooling pattern should follow evacuation

of plugof plug

Meconium plugs

Hirschsprung’s DiseaseHirschsprung’s Disease

Colonic agangliosisColonic agangliosisExtent can vary from very short segment Extent can vary from very short segment

of rectal tissue to entire colonof rectal tissue to entire colonShould be considered in any baby who Should be considered in any baby who

does not pass stool spontaneously by 24 does not pass stool spontaneously by 24 hours of age. hours of age.

Diagnosis by rectal biopsy to look for Diagnosis by rectal biopsy to look for ganglion cells ganglion cells

Hirschsprung’s Disease (cont)Hirschsprung’s Disease (cont)

Barium enema can show transition zoneBarium enema can show transition zoneShort segment disease can be treated with Short segment disease can be treated with

rectal irrigations followed by primary pull rectal irrigations followed by primary pull through procedurethrough procedure

Longer segment disease requires ostomy Longer segment disease requires ostomy followed by pull through when older followed by pull through when older (months usually). (months usually).

Imperforate AnusImperforate Anus

May pass meconium if a rectovaginal or May pass meconium if a rectovaginal or rectourinary fistula exists.rectourinary fistula exists.

Low imperforate anus: the rectum has Low imperforate anus: the rectum has descended through the puborectalis sling descended through the puborectalis sling and exists as a fistula on the perineum.and exists as a fistula on the perineum.May see mec on the perineum, may be seen May see mec on the perineum, may be seen

in the rugal folds or scrotum of males and in the rugal folds or scrotum of males and vagina of females. vagina of females.

These fistula may be dilated to temporarily These fistula may be dilated to temporarily relieve obstructionrelieve obstruction

Imperforate Anus (cont)Imperforate Anus (cont)

High imperforate anus: rectum ends above High imperforate anus: rectum ends above the puborectalis sling. the puborectalis sling.

No perineal fistula, but may have urinary No perineal fistula, but may have urinary fistulafistula

Temporary colostomy is necessary in all Temporary colostomy is necessary in all babies with high imperforate anus. babies with high imperforate anus.

Malrotation with volvulusMalrotation with volvulus

Can occur in the fetus – large calcified Can occur in the fetus – large calcified shadow in midabdomen on x-rayshadow in midabdomen on x-ray

Sudden onset of bilious emesis in infant – Sudden onset of bilious emesis in infant – requires rule outrequires rule out

Signs of shock and sepsis can be presentSigns of shock and sepsis can be presentSurgical emergency since intestinal Surgical emergency since intestinal

viability is at stake. viability is at stake. UGI to evaluate for position of ligament of UGI to evaluate for position of ligament of

TreitzTreitz

Malrotation Volvulus

Adhesions and stricturesAdhesions and strictures

Can occur following any abdominal Can occur following any abdominal surgical manipulation.surgical manipulation.

Can occur following NEC even if initial Can occur following NEC even if initial disease process required only medical disease process required only medical treatment.treatment.

Necrotizing Enterocolitis Necrotizing Enterocolitis

This is a whole lecture unto itselfThis is a whole lecture unto itself Incidence varies from center to center and Incidence varies from center to center and

from year to year within centersfrom year to year within centers2-5% of all NICU admissions and 5-10% of 2-5% of all NICU admissions and 5-10% of

VLBW infantsVLBW infantsPrematurity is greatest risk factorPrematurity is greatest risk factorMortality is 9-28% regardless of medical or Mortality is 9-28% regardless of medical or

surgical interventionsurgical intervention

NEC (cont)NEC (cont)

To be continued in future lecture……..?To be continued in future lecture……..?

GenitourinaryGenitourinary

Posterior urethral valvesPosterior urethral valvesExtrophy of the bladderExtrophy of the bladderCloacal extrophyCloacal extrophy

Posterior Urethral ValvesPosterior Urethral Valves

Obstruction of urinary flow at level of Obstruction of urinary flow at level of bladder outletbladder outlet

Exclusively in malesExclusively in malesMay lead to oligohydramnios and May lead to oligohydramnios and

pulmonary hypoplasiapulmonary hypoplasiaMay lead to destruction of renal May lead to destruction of renal

parenchymaparenchymaSymptomatology can sometimes be mildSymptomatology can sometimes be mild

VCUG in pt with Posterior UrethralValves

Posterior Urethral ValvesPosterior Urethral ValvesPlace urinary catheter to drain bladderPlace urinary catheter to drain bladderObtain VCUG to confirm diagnosisObtain VCUG to confirm diagnosisUrologist to ablate valves – usually with a Urologist to ablate valves – usually with a

transurethral approachtransurethral approachAssess renal function – urine output, Assess renal function – urine output,

electrolytes, BUN, creatinineelectrolytes, BUN, creatinineBladder may be dysfunctional Bladder may be dysfunctional Some will be OK in neonatal period and go Some will be OK in neonatal period and go

on to “outgrow” their renal function on to “outgrow” their renal function

Extrophy of the BladderExtrophy of the Bladder

May range from epispadias to complete May range from epispadias to complete extrusion of bladder on to abdominal wallextrusion of bladder on to abdominal wall

Initial surgical repair is most critical for Initial surgical repair is most critical for best functional outcomebest functional outcome

Apply sterile saline and lay saran wrap on Apply sterile saline and lay saran wrap on top of exposed bladder, but keep any top of exposed bladder, but keep any irritants away (no gauze, etc)irritants away (no gauze, etc)

Extrophy of bladderExtrophy of bladder

2:1 male to female ratio2:1 male to female ratioSymphysis pubis is widely separatedSymphysis pubis is widely separated Is a very complicated and prolonged repair Is a very complicated and prolonged repair

requiring urologist and orthopedist to work requiring urologist and orthopedist to work togethertogether

Patient immobilized for a week or more Patient immobilized for a week or more post-oppost-op

Phallic reconstruction may be done in later Phallic reconstruction may be done in later operation operation

Cloacal ExtrophyCloacal Extrophy

More complex than simple bladder More complex than simple bladder extrophyextrophy

May include: vesico-intestinal fissure, May include: vesico-intestinal fissure, omphalocele, extrophied bladder, omphalocele, extrophied bladder, hypoplastic colon, imperforate anus, hypoplastic colon, imperforate anus, absence of vagina in female, and absence of vagina in female, and microphallus in males. microphallus in males.

Series of complex operations neededSeries of complex operations needed

Abdominal massesAbdominal masses

Renal massesRenal massesOvarian cystOvarian cystTumorsTumorsAdrenal hemorrhageAdrenal hemorrhageHydrometrocolposHydrometrocolpos

Renal MassesRenal Masses

Polycystic kidneysPolycystic kidneysMulticystic dysplastic kidneysMulticystic dysplastic kidneysRenal duplicationsRenal duplicationsHydronephrosisHydronephrosis

Ovarian CystOvarian Cyst

One of the most common abdominal One of the most common abdominal masses in female fetus and newbornmasses in female fetus and newborn

May cause torsion and necrosis of ovaryMay cause torsion and necrosis of ovarySurgical resection if >4cm diameter or Surgical resection if >4cm diameter or

persistentpersistent

TumorsTumors

Teratomas are most common.Teratomas are most common.SacrococcygealSacrococcygealAbout 10% contain a malignant elementAbout 10% contain a malignant element

Neuroblastoma – 50% of malignant tumors Neuroblastoma – 50% of malignant tumors in neonates. Can resolve spontaneously.in neonates. Can resolve spontaneously.

Wilm’s tumorWilm’s tumorSarcoma botryoides – grapelike tumor Sarcoma botryoides – grapelike tumor

arises from edge of vulva or vagina. arises from edge of vulva or vagina.

Sacrococcygeal teratoma

Sarcoma Botryoides

HydrometrocolposHydrometrocolpos

Imperforate hymen (or other vaginal Imperforate hymen (or other vaginal obstruction) with back up of secretions in obstruction) with back up of secretions in the uterus, which can cause intestinal the uterus, which can cause intestinal obstructionobstruction

Hymen will bulgeHymen will bulgeMay have edema and cyanosis of the legsMay have edema and cyanosis of the legsMay cause hydronephrosisMay cause hydronephrosis

Inguinal HerniaInguinal Hernia

Frequent in preterm babies, male>femaleFrequent in preterm babies, male>femaleUsually repair prior to discharge if possibleUsually repair prior to discharge if possibleMonitor for incarcerationMonitor for incarceration

Incarcerated hernia can usually be reduced Incarcerated hernia can usually be reduced with sedation and steady firm pressurewith sedation and steady firm pressure

Even if reduced, should be repaired as soon Even if reduced, should be repaired as soon as edema is resolvedas edema is resolved

Testicular TorsionTesticular Torsion

70% occur in fetal life70% occur in fetal lifeTestes is tender, firm, swollen with a Testes is tender, firm, swollen with a

slightly bluish cast to the scrotum on slightly bluish cast to the scrotum on affected sideaffected side

Differential dx includes scrotal hematomaDifferential dx includes scrotal hematomaUS with doppler flow can be usefulUS with doppler flow can be usefulAny possibility of recent torsion – surgery Any possibility of recent torsion – surgery

within 4-6 hrs within 4-6 hrs Orchiopexy of contralateral sideOrchiopexy of contralateral side

Neurosurgical Neurosurgical

MyelomeningoceleMyelomeningoceleHydrocephalusHydrocephalus Intracranial hematomaIntracranial hematoma

MyelomeningoceleMyelomeningocele

Saccular outpouching of neural elements Saccular outpouching of neural elements through defect in bone and soft tissues in through defect in bone and soft tissues in posterior thoracic, sacral and/or lumbar posterior thoracic, sacral and/or lumbar regions (lumbar 80%)regions (lumbar 80%)

Most common primary neural tube defectMost common primary neural tube defectHydrocephalus in 84%Hydrocephalus in 84%Arnold-Chiari II malformation in 90%Arnold-Chiari II malformation in 90%

The Chiari II malformation is a complex The Chiari II malformation is a complex congenital malformation of the brain, congenital malformation of the brain, nearly always associated with nearly always associated with myelomeningocele. This condition myelomeningocele. This condition includes downward displacement of the includes downward displacement of the medulla, fourth ventricle, and cerebellum medulla, fourth ventricle, and cerebellum into the cervical spinal canal, as well as into the cervical spinal canal, as well as elongation of the pons and fourth ventricle, elongation of the pons and fourth ventricle, probably due to a relatively small posterior probably due to a relatively small posterior fossa.fossa.

Chiari II malformation. Sagittal T1-weighted MRI of posterior fossa abnormalities in Chiari II malformation: (1) colpocephaly; (2) beaked tectum; (3) cascade of an inferiorly displaced vermis behind the medulla; (4) elongated tubelike fourth ventricle; (5) low-lying torcular herophili; (6) cerebellar hemispheres wrapping around the brainstem anteriorly; (7) concave clivus; (8) medullary spur; and (9) medullary kink.

HydrocephalusHydrocephalus

Associated with MMAssociated with MMAqueductal Stenosis – male with X-linked Aqueductal Stenosis – male with X-linked Post-hemmorhagic – most frequent in Post-hemmorhagic – most frequent in

premature populationpremature population