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Case 1: Acute, Intermittent, and Colicky AbdominalPain in a 12-year-old Boy

Case 2: Emesis Since Birth in a 2½-month-old InfantCase 3: Worsening Cough Since Birth in a 16-month-

old Toddler

Case 1 PresentationA 12-year-old boy presents to theemergency department with acute-onset, intermittent, colicky right lo-wer quadrant abdominal pain for 3to 4 hours. The pain is associatedwith nausea and one episode of non-bilious, nonbloody vomiting. He hasnot had diarrhea, constipation, fever,urinary symptoms, trauma, or pastsimilar episodes.

On examination, the boy’s tem-perature is 38.8°C, his heart rate is146 beats per minute, his respiratoryrate is 26 breaths per minute, and hisblood pressure is 110/65 mm Hg.He looks tired. His weight is 38.8kg (35th percentile). Abdominal ex-amination reveals a soft and nondis-tended abdomen, but he is tender inthe left and right lower quadrants.He has no rebound tenderness, guard-ing, palpable masses, or organomegaly.The findings on the rest of the physicalexamination are unremarkable.

Initial laboratory evaluations showhemoglobin of 13.6 g/dL, a whiteblood cell count of 18 � 103/mL(82% neutrophils, 12% lymphocytes,6% monocytes, and 1% eosinophils),and a platelet count of 277 � 103/mL. His blood levels are as follows:total serum bilirubin, 0.6 mg/dL; di-rect bilirubin, <0.1 mg/dL; alanineaminotransferase, 18 IU/L; aspartateaminotransferase, 28 IU/L; alkalinephosphatase, 175 IU/L; amylase, 34IU/L; and lipase, 42 IU/L. Serumelectrolyte levels are normal. The onlyabnormality on urinalysis is 1þ blood.Abdominal radiographs show a nonspe-cific bowel gas pattern. Ultrasonogra-phy of the abdomen reveals elongated

tubular structures with hypoechoic cen-ters in the small intestine, which suggesta diagnosis (Fig 1). An additional studyconfirms the diagnosis.

Case 2 PresentationA 2½-month-old boy is referred toour hospitalist service from an out-side hospital. His parents report daily,nonbloody, nonbilious vomiting sincebirth. The vomiting often is worse af-ter feeding and has been so frequentthat the patient has not gained weightappropriately. His stools have beennormal. In addition, he has had fre-quent episodes of screaming and arch-ing of his back each day that lastseveral hours and occasionally are as-sociated with feeding. His prenatalhistory was unremarkable, and hisbirthweight was at the 50th percentile.A gastroenterologist has diagnosed theinfant as having milk allergy and gas-troesophageal reflux, but trials of hy-drolyzed formulas and lansoprazolehave not improved his symptoms.

On the day of transfer, the patientis smiling and interactive. His weightis at the 5th percentile, his head cir-cumference is at the 5th percentile,and his vital signs are normal. Find-ings on examination are completelynormal, including his abdominal andneurologic evaluations. A basic meta-bolic panel on the day of transfer revealsthe following levels: sodium, 136mEq/L; potassium, 3.3 mEq/L; chlo-ride, 102 mEq/L; bicarbonate, 24mEq/L; BUN, 12 mg/dL; creatinine,0.4 mg/dL; and glucose, 93 mg/dL.

The evaluation performed at theoutside hospital included MRI of the

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brain, upper gastrointestinal (GI) series,abdominal radiograph, pyloric ultraso-nography,esophagogastroduodenoscopywith biopsy, and routine stool stud-ies; the results of all these tests werenegative. An additional diagnostic testreveals the probable cause of his painfulepisodes and vomiting.

Case 3 PresentationA 16-month-old boy presents to thepediatric clinic because of aworseningcough. His parents report that he hashad a runny nose and cough for 2 to3 days, but today his symptoms areworse. His mother had regular prena-tal care, and he has been seen by hispediatrician regularly since birth.

On physical examination, the boy’sweight and length are at the 40th and55th percentiles, respectively. Histemperature is 37.8°C, his heart rateis 116 beats per minute, his respiratoryrate is 48 breaths per minute, and ox-ygen saturation is 94% on room air. Hehas nasal congestion, mild pharyngealhyperemia, and wheezy lung sounds.Other findings, including abdominalevaluation, are normal.

A complete blood cell count withdifferential and chest radiograph areordered, and therapy is started with sal-butamol inhalation. His white bloodcell count is 9.3 � 103/mL (68% lym-phocytes, 23% segmented neutro-phils, and 1% bands), his Hgb levelis 11.2 g/dL, and platelet count is278 � 103/mL. A presumptive diag-nosis of viral upper respiratory tractinfection and acute bronchiolitis ismade. However, results of the chestradiograph aid in the diagnosis, whichis confirmed with additional imagingstudies.

Case 1 DiscussionVarious medical and surgical condi-tions can present with acute abdominal

pain. Although most of these areself-limited, benign conditions, it isextremely important to rule outmore emergent disorders in a timelymanner to prevent morbidity andmortality. A thorough history and ex-amination with focused laboratoryevaluation usually establish diagno-sis. In this patient, initial laboratoryevaluation ruled out some seriousconditions, such as intestinal obstruc-tion, pancreatitis, hepatitis, and acutecholangitis. Ultrasonography of theabdomen was performed to rule outacute appendicitis, and the resultssuggested the presence of round-worms. Stool microscopy showedthe fertilized eggs of roundworms,thus confirming the diagnosis ofascariasis.

The ConditionAscariasis is the most common hel-minthic infestation, with more than1 billion people infested worldwide.However, it is seen less frequentlyin the United States, and most USpatients diagnosed with the conditionare immigrants from endemic coun-tries. The infection is caused by in-gestion of food contaminated withsingle-cell stage fertilized eggs. Thefemale worm produces 200,000 ormore eggs in a day, and these eggscan survive in extreme environmen-tal conditions. The eggs must gothrough more than one molting inthe soil; hence, person-to-personcontact does not occur. The adultworm survives for 10 to 12 monthsin the small intestine, which makesthe infestation as a cause of abdom-inal pain unlikely more than a yearafter immigration.

The clinical presentation dependson the load of infestation and the or-gans involved. Most children with alow to moderate load remain asymp-tomatic. Some develop vague abdom-inal symptoms and may show signsof malnutrition. The most common

acute presentations are due to ob-structionof the intestine or biliary tractand pulmonary symptoms. Loefflerpneumonia occurs during the pas-sage of larvae through the lungsand is characterized by coughing,wheezing, and difficulty of breathing,associated with blood eosinophiliawith fleeting pulmonary infiltrates.More serious conditions, includingacute appendicitis, toxic megacolon,liver abscess, ascending cholangitis,and pancreatitis, can occur rarely.

DiagnosisTraditionally, the demonstration offertilized or unfertilized eggs in stoolhas been diagnostic; however, high-density, higher frequency ultrasonog-raphy of the abdomen sometimescan be useful in establishing the diag-nosis. The live worm on longitudinalsection appears as a writhing tubularshadow having brighter margins, de-scribed by some as the “strip sign.”The hypoechoic core produces the“inner tube sign.” The cross-sectionalpicture is also characteristic of a tubu-lar body, described as the “ring sign”or “bull’s-eye sign” (Fig 2).

Treatment and PreventionTreatment consists of antihelminthicmedications, including albendazolein a single dose, mebendazole for 3

Figure 1. Abdominal ultrasonographyshowing a hyperechoic tubular structurewith a hypoechoic center.

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days or as a single dose, and ivermec-tin in a single dose. A follow-up stoolspecimen at 3 weeks after treatmentto assess elimination of eggs can bedone but is not essential. Intestinalobstruction as a complication of asca-riasis primarily requires conservativemanagement. Piperazine may have arole in the treatment of intestinal ob-struction but is not available in theUnited States. However, surgical in-terventions may be required in certainsituations, such as intestinal obstruc-tion with failed conservative manage-ment, complete intestinal obstruction,biliary obstruction, volvulus, or perfo-ration peritonitis.

Loeffler pneumonia primarily re-quires symptomatic treatment, in-cluding bronchodilators, oxygen, andcorticosteroids. Some clinicians rec-ommend postponement of antihel-minthic treatment until the patient isclinically stable because migration ofworms after the antihelminthic treat-ment may worsen the symptoms. Bet-ter sanitation and personal hygiene areimportant in prevention.

This patient was treated with a sin-gle 500-mg dose of mebendazole,was observed overnight, and was dis-charged after the pain resolved.

Lessons for the Clinician

• Helminthic infestation should beconsidered as a cause of abdomi-nal pain, especially in immigrantpopulations.

• Ascariasis usually presents withmild abdominal symptoms. How-ever, occasionally, the infestationmay cause serious complications.

• Abdominal ultrasonography maybe helpful in the diagnosis ofascariasis.

(ArunSaini,MD,SwatiA.Karmarkar,MD, Chokechai Rongkavilit, MD,Children’s Hospital of Michigan, De-troit, MI)

Case 2 DiscussionComplete abdominal ultrasonogra-phy performed to re-evaluate the py-lorus and search for other potentialcauses for the boy’s symptoms dem-onstrated grade 3 hydronephrosison the left with no evidence of hydro-ureter. A voiding cystourethrogramrevealed a normal bladder withoutvesicoureteral reflux. A 99mTc-mercaptoacetyltriglycine (MAG3) re-nal scan demonstrated equal renalfunction, with prolonged uptake oftracer and delayed washout on the left.

These findings were suggestive ofleft ureteropelvic junction (UPJ) ob-struction. The patient underwent aleft pyeloplasty, with excision of thenarrowed UPJ segment and place-ment of an internal ureteral stent.The next day, he tolerated feeds with-out difficulty. The parents reported thathe was finally a “happy baby.” Follow-up visits have shown that his vomitinghas resolved, and he has been gainingweight well. On repeat ultrasonogra-phy, he has mild residual hydronephro-sis on the left. RepeatMAG3 renal scandemonstrates normalization of renaldrainage with preservation of function.

Differential DiagnosisThe differential diagnosis of an infantwho presents with nonbilious vomit-ing depends largely on the durationof the vomiting. When vomitinghas been present since birth, over-feeding, gastroesophageal reflux,proximal intestinal obstruction, met-abolic disorders, and increased intra-cranial pressure should be consideredstrongly. Some disorders causingchronic vomiting in infancy are relatedto allergy, such as protein-induced eo-sinophilic gastropathies. Therefore, itis critical to take a thorough historyregarding diet, relationship of thevomiting to eating, and presence ofdiarrhea or hematochezia.

The infant’s growth and develop-ment also can provide clues to diag-nosis. Developmental delay mightsuggest a metabolic disorder or in-creased intracranial pressure. Failureto thrive could be due to the degreeof vomiting and calorie loss or couldbe secondary to an underlying condi-tion. The physical examination shouldinclude the infant’s state of hydration,as well as thorough neurologic, ab-dominal, and genital assessments.

Further diagnostic evaluation ofan infant who experiences chronicvomiting should be based on the di-agnoses considered most likely aftera careful history and physical exami-nation are performed. A completemetabolic profile can offer clues tothe diagnosis or can indicate the se-verity or chronicity of the vomiting.Hypochloremic metabolic alkalosissuggests that the vomiting has beensevere, and hypoglycemia, metabolicacidosis, or elevated liver enzyme levelsmight suggest a metabolic disorder.

If vomiting is related to feedings,gastroesophageal reflux and proxi-mal intestinal obstruction should beconsidered, and an upper GI series,pH probe testing, or abdominal ul-trasonography should be performed.

Figure 2. Abdominal ultrasonographyshowing a cross-section of a tubularstructure, which is a roundworm.

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If developmental delay or abnormalresults on neurologic examinationare noted, imaging of the brain ora metabolic evaluation might be in-dicated. If an allergic condition issuspected, elimination of intact milkprotein from the diet and the use of el-emental formula should be undertaken,and esophagogastroduodenoscopy canbe performed to look for evidence ofinflammation or tissue eosinophilia.

In this patient, an elimination dietdid not improve the symptoms, andthe results of biopsies showed noevidence of allergy. These findingssuggest that milk allergy was the in-correct diagnosis. The discovery ofthe unilateral hydronephrosis led tothe evaluation for UPJ obstruction.In hindsight, the mother remem-bered that she had been told that pre-natal ultrasonography had indicatedthat one kidney was “slightly larger”than the other, but that the differ-ence was not significant.

The ConditionUPJ obstruction is the most commoncause of fetal hydronephrosis, ac-counting for approximately 50% ofsuch cases, and the finding is morecommon in males. Causes for UPJ ob-struction include fibrosis, an aperistalticsegment, or compression by a renalvessel. Two thirds of all cases occuron the left side, and 10% to 40% ofcases are bilateral. The incidenceranges from 1 in 500 to 1 in 1,500.Some affected children present laterin childhood.

Most neonates and young chil-dren afflicted with the disorder areasymptomatic, whereas older patientsfrequently have symptoms. Acute ob-struction and subsequent distentionof the renal pelvis activate parasympa-thetic afferent fibers, causing nausea,vomiting, or pain. Some patients, in-cluding those with intermittent ob-struction, have impaired drainageof urine and therefore develop

symptoms only when urine produc-tion exceeds the drainage of urinefrom the renal pelvis. These patientsoften will note flank pain and nauseaafter periods of increased fluid con-sumption, a phenomenon known asDietl’s crisis.

The strictest definition of UPJ ob-struction includes delayed drainage incombination with either symptomsor impaired renal function. The mostcommon test used in the diagnosis isa nuclear renal scan such as MAG3.This type of scan can assess the drain-age of each kidney and can provideinformation on the contribution ofeach kidney to overall renal function.

Affected children who show noevidence of impaired renal functionmay be observed over time with rou-tine ultrasonographic follow-up, be-cause in 50% to 75% of cases, UPJobstruction will resolve spontane-ously without the patient developingany symptoms. However, if renalfunction is impaired or if symptomsdevelop, corrective surgery shouldbe considered. The most commonsurgical technique is an open or lap-aroscopic pyeloplasty, which involvesexcising the obstructing segment andre-anastomosing the ureter to the re-nal pelvis. Complications of pyelo-plasty include extravasation of urineand recurrence of obstruction dueto scar formation. A few childrenwith impaired renal function beforesurgery may experience declining re-nal function despite correction of theobstruction.

Lessons for the Clinician

• Ureteropelvic junction (UPJ) ob-struction is a rare cause of vomit-ing and pain in infants.

• Gastrointestinal (GI) pathology isa much more common cause ofvomiting in infants and should beevaluated fully and treated beforeconsidering other causes.

• Failure to identify GI causesshould prompt an evaluation forother disorders.

(JoNell Bolton, MD, Christopher Roth,MD, Robin English, MD, LouisianaState University Health Sciences CenterNew Orleans, LA)

Case 3 DiscussionThe chest radiograph revealed hyper-lucent, gas-filled areas in the rightlower chest, suggesting a diaphrag-matic hernia. A lateral chest radio-graph confirmed the diagnosis ofdiaphragmatic hernia (Figs 3 and 4).Computed tomography scan of thechest confirmed an anterior dia-phragmatic defect, also known as aMorgagni hernia (Fig 5). The dia-phragmatic hernia was repairedelectively.

The ConditionIn the developing fetus, the septumtransversum, bilateral pleurope-ritoneal membranes, chest wall mus-cles, and dorsal mesentery of theesophagus contribute to the forma-tion of the diaphragm. During week8 of gestation, these structures fuse,and thus the pleural and peritonealcavities are separated. The fusion ofthe septum transversum and pleuro-peritoneal membranes results in thepleuroperitoneal canal. The failureof closure of the pleuroperitoneal ca-nal during gestational weeks 8 to 10results in congenital diaphragmatichernia (CDH).

The incidence of CDH is 1 in2,000 to 3,000 live births. CDHsare classified traditionally as Bochdalek(posterolateral), Morgagni (anterior),and hiatal hernias. Approximately 85%are left-sided Bochdalek hernias.

In classic posterolateral CDH,herniation of intraabdominal organsinto the thoracic cavity causes both

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ipsilateral and contralateral pulmonaryhypoplasia and associated pulmonaryhypertension. The compromised de-veloping lung in a fetus with CDHis surfactant deficient and associatedwith anatomic changes that predis-pose to pulmonary hypertension. In

contrast, most anterior Morgagnihernias are not associated with thesepulmonary findings, and incidentaldiscovery is more common.

Approximately one half of thecases are diagnosed in the prenatalperiod by using ultrasonography.However, prenatal CDH diagnosis

rates are variable, depending on theincidence of prenatal screening withultrasonography.

Infants presenting with symptom-atic CDH typically have acute respi-ratory compromise that may requireprompt airway control and assistedmechanical ventilation and oxygena-tion. An orogastric tube should beplaced early to prevent gastric disten-tion and further pulmonary compro-mise. The diagnosis is confirmed withthe use of chest radiographs. Echo-cardiography may be useful to de-termine whether congenital cardiaclesions exist and to quantify the de-gree of pulmonary hypertension.

Some infants with CDH will pres-ent with subtle nonspecific signs andsymptoms such as recurrent coughand repeated respiratory infections,bouts of variable respiratory distress,and failure to thrive. Morgagni her-nias tend to be less symptomatic thanposterolateral hernias, and a high de-gree of suspicion, along with a carefulhistory, examination, and diagnostic

Figure 3. Radiograph of the chest showsintestines in the lower lung areas onboth sides and also overriding thecardiac image.

Figure 4. Lateral radiograph of chestshowing intestines in the retrosternalarea.

Figure 5. Computed tomography scan of the chest showing intestines within the thorax.

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imaging, is required. Clinicians shouldrealize that a previously normal chestradiograph does not completely ex-clude a diagnosis of CDH.

ManagementThe treatment of CDH is indivi-dualized, based on anatomic loca-tion, physiologic status, age, andassociated congenital anomalies. Pos-terolateral CDH in a newborn maybe acutely symptomatic, requiring es-calation of ventilatory managementto provide adequate gas exchange.This therapy includes the use ofhigh-frequency oscillatory ventila-tion, inhaled nitric oxide, and ex-tracorporeal membrane oxygenation

support. Therefore, newborns bornwith posterolateral CDH experienceconsiderable morbidity and mortalityreflective of the anatomic and physi-ologic derangement.

In contrast, most anteriorMorgagnihernias do not present with pulmonaryhypoplasia and pulmonary hyperten-sion. Therefore, the diagnosis typicallyis delayed for months to years, and thecondition may be found incidentally.After accurate diagnosis of a Morgagnihernia, prompt elective operative repairis indicated to prevent respiratory dis-tress and potential GI obstruction fromincarceration. The expected outcomeshould be excellent, with low morbid-ity and near-zero mortality.

Lessons for the Clinician

• Congenital diaphragmatic hernia(CDH) presents with a spectrumof signs and symptoms based onanatomic location and physiology.

• Unlike posterolateral CDH, pre-senting symptoms may be subtleand diagnosis delayed in those bornwith anterior Morgagni hernias.

(AyhanCeri,MD,MemorialDiyarbakirHospital, Diclekent, Kayapinar,Diyarbakir, Turkey)

To view Suggested Reading lists forthese cases, visit http://pedsinreview.aappublications.org and click onthe “Index of Suspicion” link.

ClarificationIn the “Care of the Well Newborn” article in the January 2012 issue (Warren JB, Phillipi CA. Pediatr Rev. 2012;33(1):4–18),erythromycin eye ointment is cited as the most commonly used medication to prevent ophthalmia neonatorum. Although it

is effective in the prevention of gonococcal ophthalmia, erythromycin ointment is not effective in preventing conjunctivitis

caused by Chlamydia trachomatis. Optimal prevention of ophthalmia involves screening and treating pregnant women for

both infections. Ointment administration should not promote complacency in clinicians, who should thoroughly evaluate

conjunctivitis appearing in the first month after birth, especially when the mother has not been screened for Chlamydiainfection.

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DOI: 10.1542/pir.34-1-412013;34;41Pediatrics in Review 

Christopher Roth and Robin EnglishArun Saini, JoNell Bolton, Ayhan Ceri, Swati A. Karmarkar, Chokechai Rongkavilit,

Index of Suspicion

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DOI: 10.1542/pir.34-1-412013;34;41Pediatrics in Review 

Christopher Roth and Robin EnglishArun Saini, JoNell Bolton, Ayhan Ceri, Swati A. Karmarkar, Chokechai Rongkavilit,

Index of Suspicion

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