Pediatric Pediatric Umbilical Umbilical

AbnormalitiesAbnormalitiesScott Nguyen MDScott Nguyen MD

Mount Sinai School of MedicineMount Sinai School of Medicine

Dept of SurgeryDept of Surgery

Abnormalities of Abnormalities of Umbilical CordUmbilical Cord

Umbilical abnormalities result from Umbilical abnormalities result from failure of umbilical ring to close or failure of umbilical ring to close or persistence of umbilical structurespersistence of umbilical structures

Understanding embryology of cord is Understanding embryology of cord is essential in understanding the essential in understanding the pathophysiology of umbilical pathophysiology of umbilical abnormalitiesabnormalities

Embryology - 3Embryology - 3rdrd week week

EmbryologyEmbryology

EmbrologyEmbrology

EmbryologyEmbryology

66thth wk – midgut loop elongates and wk – midgut loop elongates and herniates out through umbilical cordherniates out through umbilical cord

Midgut rotates 270 degreesMidgut rotates 270 degrees Returns to abdomen by 10Returns to abdomen by 10thth wk wk Anterior abdominal wall Anterior abdominal wall

progressively closes leaving only progressively closes leaving only umbilical ringumbilical ring

Umbilical AbnormalitiesUmbilical Abnormalities

Urachal AbnormalitiesUrachal Abnormalities Vitelline Duct AbnormalitiesVitelline Duct Abnormalities Umbilical Hernia Umbilical Hernia OmphalitisOmphalitis Delayed Cord SeparationDelayed Cord Separation

Umbilical granulomaUmbilical granuloma

Urachal formationUrachal formation Bladder forms from Bladder forms from

ventral portion of ventral portion of cloacacloaca

Bladder descends Bladder descends into pelvis w/ into pelvis w/ urachus connecting urachus connecting apex to umbilicusapex to umbilicus

Usually urachus Usually urachus involutes to a involutes to a fibrous cord – fibrous cord – median umbilical median umbilical ligamentligament

Urachal abnormalitiesUrachal abnormalities

• failure of obliteration of urachus failure of obliteration of urachus resulting complete or partial patency resulting complete or partial patency of urachus of urachus

• < 1/1000 live births< 1/1000 live births• inflammation or drainage from inflammation or drainage from

umbilicusumbilicus• US, CT, contrast studies, or injection US, CT, contrast studies, or injection

of dye into tract can confirm of dye into tract can confirm diagnosis diagnosis

• Patent Urachus (50%)Patent Urachus (50%)• Urachal cyst (30%)Urachal cyst (30%)• Urachal sinus (15%)Urachal sinus (15%)• Vesicourachal diverticulum (5%) Vesicourachal diverticulum (5%)

Patent Urachus Patent Urachus

StudiesStudies

Catherization of tract Catherization of tract and injection of dyeand injection of dye

Voiding Voiding cystourethrogramcystourethrogram

USUS

UltrasoundUltrasound

CTCT

VCUGVCUG

Treatment Patent Treatment Patent UrachusUrachus

Patent UrachusPatent Urachus

Urachal CystUrachal Cyst

Usually assx until Usually assx until infectedinfected

Rarely become Rarely become infected in infected in newborn period, newborn period, usu manifests as usu manifests as young adultyoung adult

Infected Urachal cystInfected Urachal cyst

Fever, voiding symptoms, midline Fever, voiding symptoms, midline hypogastric tenderness, mass, UTI hypogastric tenderness, mass, UTI

May drain into bladder or umbilicusMay drain into bladder or umbilicus Rarely can rupture into Rarely can rupture into

preperitoneal tissues or peritoneal preperitoneal tissues or peritoneal cavitycavity

Cultures - Staph AureusCultures - Staph Aureus

USUS

CTCT

Infected Urachal cyst - Infected Urachal cyst - treatmenttreatment

Incision and drainage Incision and drainage Percutaneous drainagePercutaneous drainage Complete surgical excision of Complete surgical excision of

all urachal tissueall urachal tissue 30% recurrence if only drainage30% recurrence if only drainage Staged approach limits amount Staged approach limits amount

of bladder resected of bladder resected

Urachal SinusUrachal Sinus

Becomes Becomes symptomatic symptomatic when infectedwhen infected

Tx – drainage Tx – drainage and resection and resection of urachal of urachal tissuetissue

SinogramSinogram

Urachal DiverticulumUrachal Diverticulum

Blind sac Blind sac at bladder at bladder apexapex

Mostly Mostly assx assx

Urachal DiverticulumUrachal Diverticulum

Vitelline Duct Vitelline Duct AbnormalitiesAbnormalities

Vitelline DuctVitelline Duct

Vitelline Duct is connection Vitelline Duct is connection between midgut and yolk sac between midgut and yolk sac

Usually involutes in 7Usually involutes in 7thth – 9 – 9thth weeks weeks

Vitelline duct Vitelline duct abnormalitiesabnormalities

Meckel’s DiverticulumMeckel’s Diverticulum

Meckel’s DiverticulumMeckel’s Diverticulum

contains ectopic gastric or contains ectopic gastric or pancreatic mucosapancreatic mucosa

In 2% of populationIn 2% of population 2 feet from ileocecal valve, 2 feet from ileocecal valve,

antimesenteric borderantimesenteric border Majority of symptomatic < Majority of symptomatic <

2yrs old2yrs old

PresentationPresentation

Painless GI Bleeding (50%) Painless GI Bleeding (50%) Bowel Obstruction (30%)Bowel Obstruction (30%) Inflammation – Inflammation –

diverticulitis (20%)diverticulitis (20%)

GI BleedingGI Bleeding

Most common cause of Most common cause of bleeding in childrenbleeding in children

Painless, massive, usually Painless, massive, usually self resolvingself resolving

Due to mucosal ulceration Due to mucosal ulceration from acid secretionfrom acid secretion

Meckel’s Scan – GI Meckel’s Scan – GI bleedingbleeding

Bowel ObstructionBowel Obstruction

Due to intussusception, Due to intussusception, diverticulum is the lead pointdiverticulum is the lead point

Sudden severe pain out of Sudden severe pain out of proportion to physical examproportion to physical exam

Hydrostatic Barium enema Hydrostatic Barium enema diagnostic, rarely therapeuticdiagnostic, rarely therapeutic

IntussusceptionIntussusception

IntussusseptionIntussusseption

Meckel’s DiverticulitisMeckel’s Diverticulitis

Sx like appendicitis Sx like appendicitis Result of lumenal obstruction, Result of lumenal obstruction,

bacterial invasion, progressive bacterial invasion, progressive inflammationinflammation

Ectopic gastric mucosa Ectopic gastric mucosa predisposespredisposes

30% incidence of perforations30% incidence of perforations Higher risk of peritonitis Higher risk of peritonitis

TreatmentTreatment

Surgical Resection without Surgical Resection without removal of ileum removal of ileum

V shaped incision at baseV shaped incision at baseresection of involved resection of involved

segment of ileum w/ segment of ileum w/ primary anastamosisprimary anastamosis

Fibrous Vitelline Fibrous Vitelline RemnantRemnant

Fibrous Vitelline Fibrous Vitelline RemnantRemnant

Barium EnemaBarium Enema

Vitelline Umbilical Vitelline Umbilical FistulaFistula

Vitelline Umbilical fistulaVitelline Umbilical fistula

Umbilical polyp Umbilical polyp May drain May drain

enteric enteric contentscontents

Fistulogram Fistulogram shows shows communication communication w/ bowelw/ bowel

HerniationHerniation

Umbilical HerniaUmbilical Hernia

Umbilical herniaUmbilical hernia

ProtrudesProtrudes Rarely incarceratesRarely incarcerates Incidence 10-25% infantsIncidence 10-25% infants 6-10x higher incidence in Black infants6-10x higher incidence in Black infants More in girls, prematureMore in girls, premature Assoc w/ Down’s Synd, Beckwith-Assoc w/ Down’s Synd, Beckwith-

Wiedemann synd, hypothyroidism, Wiedemann synd, hypothyroidism, mucopolysaccharidosismucopolysaccharidosis

TreatmentTreatment

Most close by 3-4 years age Most close by 3-4 years age (>90%)(>90%)

Defect greater than 1.5 – 2 Defect greater than 1.5 – 2 cm less likely to closecm less likely to close

Surgical closure indicated in Surgical closure indicated in kids >5 yearskids >5 years ageage

Proboscoid Umbilical Proboscoid Umbilical HerniasHernias

Proboscoid umbilical Proboscoid umbilical herniashernias

15-20% of umbilical hernias15-20% of umbilical hernias Same sized fascial defectSame sized fascial defect Same likelihood of closing Same likelihood of closing

spontaneouslyspontaneously Excessive redundant umbilical Excessive redundant umbilical

skinskin Surgical repair for social and Surgical repair for social and

cosmetic reasonscosmetic reasons

OmphalitisOmphalitis

OmphalitisOmphalitis

erythema and edema of umbilical erythema and edema of umbilical area area

excellent medium for bacterial excellent medium for bacterial colonizationcolonization

poor hygiene or hospital-acquired poor hygiene or hospital-acquired infectioninfection

Staphylococcus, Streptococcus, Staphylococcus, Streptococcus, Gram (-) rodsGram (-) rods

TreatmentTreatment

IV AntibioticsIV Antibiotics Local cleaning w/ EtohLocal cleaning w/ Etoh Can rapidly progress to Can rapidly progress to

Necrotizing fasciitis (16%) Necrotizing fasciitis (16%) Usually polymicrobialUsually polymicrobial Rapidly fatal (50%)Rapidly fatal (50%) Surgical debridement necessarySurgical debridement necessary

Delayed Cord SeparationDelayed Cord Separation

Separation > 3 wks may be associated Separation > 3 wks may be associated w/ an immune deficiencyw/ an immune deficiency

Normal separation via leukocyte Normal separation via leukocyte infiltration, subsequent necrosisinfiltration, subsequent necrosis

Inherited malfunction of neutrophil, Inherited malfunction of neutrophil, monocyte, or natural killer cellsmonocyte, or natural killer cells

Susceptible to severe bacterial Susceptible to severe bacterial infectionsinfections

Immunologic workupImmunologic workup

Leukocyte Adhesion Leukocyte Adhesion DeficiencyDeficiency

Deficiency of phagocyte surface Ag – Deficiency of phagocyte surface Ag – CR3CR3

Cell surface proteins responsible for Cell surface proteins responsible for phagocyte adhesion to endothelium phagocyte adhesion to endothelium

Inability to egress from circulation to Inability to egress from circulation to areas of inflammation areas of inflammation

Phagocytic activity, degranulaton, and Phagocytic activity, degranulaton, and oxidative metabolism also affectedoxidative metabolism also affected

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