David Effron, MD

A 14-year-old girl was referred for evaluation of a several-year history of growth failure, chronic abdominal pain, and intermittent emesis. The parents described the child as a "picky eater," and various foods (eg, meat products and beans) frequently caused abdominal distention. The patient had gastroesophageal reflux symptoms during the first 2 months of life; results of an upper GI series performed at that time were normal. Family history was noncontributory. The patient had a twin brother who had normal growth parameters.

The patient's current height and weight were less than 2 standard deviations below the 5th percentile for age. Her abdomen was soft and nontender. However, the epigastric region appeared to be moderately distended and was tympanic to percussion. Bowel sounds were normal; no palpable masses or hepatosplenomegaly was noted. Results of blood tests, including a complete blood cell count, erythrocyte sedimentation rate, thyroid function tests, serum electrolytes, serum transaminases, amylase, and lipase, were within normal limits.

An abdominal radiograph revealed pneumoperitoneum, massive gastric distention, and 2 radiopaque foreign bodies (

). A CT scan confirmed these findings and demonstrated diffuse pneumatosis cystoides intestinalis (PCI) (

During upper endoscopy, a penny and a pebble were removed from the duodenum with a rat tooth forceps and retrieval basket. A duodenal web with a 5-mm orifice to the intestine was visualized after the foreign bodies were removed (

). A subsequent upper GI barium series revealed a malrotation.

The patient underwent excision of the duodenal web, duodenoduodenostomy, and division of the Ladd bands. She is currently eating well with no abdominal symptoms. Her growth parameters have normalized to some degree.

PNEUMATOSIS CYSTOIDES INTESTINALIS CAUSED BY OBSTRUCTION

Duodenal webs are congenital and are thought to result from failure of duodenal recanalization during embryogenesis. Symptoms may be insidious; duodenal webs can present at any age with symptoms of gastric outlet obstruction.

PCI is a rare entity characterized by emphysematous bowel wall changes consistent with gas-filled cysts.

 In asymptomatic patients, PCI is often found incidentally as a primary condition; however, secondary causes of pneumatosis, such as a gastric bezoar, have been described.

 Although PCI is most typically associated with necrotizing enterocolitis in premature infants, it also may be found in otherwise healthy pediatric patients and in children with underlying pathology (from, for instance, bone marrow transplantation, congenital heart disease, intestinal motility disorders, short-bowel syndrome, and gastroschisis).

Treatment may be unnecessary in asymptomatic patients with PCI, although hyperbaric oxygen therapy and antibiotics, such as metronidazole, have been used with variable success.

 Patients with an obstructive cause of PCI--duodenal stenosis or a duodenal web--require immediate surgical correction.

The constellation of findings of PCI, duodenal web, and malrotation in this patient is unusual. Unlike a previous case report of an infant with Down syndrome and associated gastric pneumatosis, duodenal stenosis, and malrotation, this teenager had no underlying chromosomal disorder and presented at a substantially older age.

The duodenal web may have contributed to this patient's presentation with PCI. A barium upper GI series is the typical method of determining its presence, although expertise is often necessary to visualize an obstructing web using fluoroscopic findings, such as the "duo- denal dimple."

Older children and adults with duodenal webs can present with abdominal pain and distention as well as electrolyte disturbances, including hyponatremia.

 Because abdominal pain is also associated with PCI, it is difficult to determine whether the pain experienced by this patient was secondary to the PCI, the duodenal web, or a combination of both.

In addition, the foreign bodies removed during endoscopy were probably present for a long period. Their inability to clear the duodenum may have led to a ball-valve effect, which accentuated gastric distention and may have precipitated gas dissection into the stomach and intestinal wall.

 Duodenal webs can present at any age with symptoms consistent with gastric outlet obstruction.

 PCI is generally a benign finding. However, it can occur with serious underlying medical conditions.

 Although PCI may respond to medical therapy, surgical intervention is often required when an obstruction is present.

 Wyllie R. Pyloric stenosis and other congenital anomalies of the stomach. In: Behrman RE, Kliegman R, Jenson HB, eds. 

 16th ed. Philadelphia: WB Saunders Co; 2000:1130-1132.


 Karl T. Pneumatosis cystoides coli--a rare differential diagnosis in colonoscopy [in German]. 

 Kawano S,Tanaka H, Daimon Y, et al. Gastric pneumatosis associated with duodenal stenosis and malrotation. 

 Stollman NH, Lee KF. Primary pneumatosis cystoides intestinalis. 

 Chintapalli KN. Gastric bezoar causing intramural pneumatosis. 

 Kurbegov AC, Sondheimer JM. Pneumatosis intestinalis in non-neonatal pediatric patients. 

 Dwek JR, Teich S. The duodenal dimple: a specific fluoroscopic correlate to the duodenal web. 

 Milla P. Motor disorders including pyloric stenosis. In: Walker W, Durie P, Hamilton J, et al, eds. 

Pediatric Gastrointestinal Disease Pathophysiology, Diagnosis and Management.

 2nd ed. St Louis: Mosby-Year Book; 1996:543-553.


 Tzeng G, Lin C, Chen H, et al. Congenital duo- denal web in late infancy and childhood: a report of two cases. 

 Adebamowo C, Oduntan O. Duodenal web causing gastric outlet obstruction in an adult. 

Articles

A 14-year-old girl was referred for evaluation of a several-year history of growth failure, chronic abdominal pain, and intermittent emesis. The parents described the child as a "picky eater," and various foods (eg, meat products and beans) frequently caused abdominal distention.

Case In Point: Pneumatosis Cystoides Intestinalis: An Unusual Radiographic Finding

A 4-year-old girl presents with a highly pruritic rash. The day before, she had been playing outdoors at her grandmother's house. No pets were present, and the patient does not recall being stung or bitten by insects. There are bushes on the grandmother's property.

The next morning, a vesicular rash had erupted on the girl's wrists, posterior knees, and the corners of her mouth. Some of the vesicles ruptured after the patient scratched them. None of the other children with whom the patient was playing have similar symptoms. She has received the varicella vaccine and has a history of mild eczema. 

The patient's vital signs are stable, and she is breathing and swallowing without difficulty. Oral examination reveals a small group of vesicles (0.1 to 0.2 cm) on the tip of her tongue (see 

on page 738) and an erythematous dry, crusty rash in the corners of her mouth. There is no swelling in the mouth or throat. Vesicles on the wrists range from 0.25 cm to 0.5 cm and contain clear fluid. The 0.25-cm fluid-filled vesicles on the backs of the knees form a linear pattern. The patient has no stridor and the rest of the examination is unremarkable.

What is the most likely cause of this patient's symptoms?

(Answer and discussion on the next page.)

 after playing in a bush that was infested with poison ivy. Rhus dermatitismay be caused by poison ivy, poison sumac, or poison oak. The oil on the leaves, roots, and stems contain urushiols, highly allergenic compounds that cause a type IV hypersensitivity reaction. Allergic persons are sensitized to the compound before reacting to the oil. Our patient had had a previous case of poison ivy. Because this is a delayed hypersensitivity reaction, the vesicles appear 8 to 72 hours after contact. 

Vesicle location depends on the site of exposure and whether the oil was transmitted along the skin by a vector, including scratching. Scratching creates a linear pattern. Lesions persist for 10 to 21 days. Vesicle fluid does not contain urushiol and does not cause further spread of the rash if the vesicle is ruptured. Clothing, tools, and other objects that are exposed to the oil must be washed because of the continued possibility of transmission.

Because the patient had no secondary infection, her treatment was symptomatic. Management included daily colloidal oatmeal baths, topical over-the-counter lotions, and cold, wet compresses followed by topical hydrocortisone 2.5% to relieve pruritus and swelling. The tongue vesicles were treated with topical anesthetics. In more severe cases, oral corticosteroids are taken daily and tapered over a 3-week period. 

 (pompholyx) is a chronic relapsing and recurring eruption of vesicles on the palms, lateral fingers, and soles. The rash is highly pruritic and features symmetric vesicles that range from 0.1 to 0.5 cm. Although several causes have been proposed, the pathophysiology of dyshidrotic eczema is not yet understood. Sweat gland dysfunction was originally thought to be the principal cause, but this has been disputed.

Dyshidrotic eczema may be triggered by exogenous factors (such as infection or contact dermatitis), emotional stress, or environmental factors (for example, seasonal changes or humidity). Many patients have associated atopic dermatitis. The vesicles generally resolve in about 3 weeks. Treatment includes high- potency topical corticosteroids, oral corticosteroids, and antihistamines.

 lesions at different stages--vesicles, pustules, and crusts--are present simultaneously. The 0.2- to 0.4-cm vesicles are described as "dew on a rose petal" because of the clear fluid and erythematous base. The disease is contagious as long as the vesicles are not all in the crust stage. Vesicles can form all over the body, including the oral cavity and vagina. Although our patient was vaccinated against varicella, the seroconversion rate after 1 dose is 95%. This leaves open the possibility of a less severe form called "breakthrough varicella." Treatment of varicella consists of antihistamines and mild antipruritic lotions.

 has 2 clinical presentations--bullous and nonbullous. It typically appears on the face but can also involve the scalp, arms, and legs. Nonbullous impetigo usually has a honey-yellow crust that forms after the vesicles or pustules rupture. The erythematous base does not typically extend beyond the lesions. 

The treatment for localized infections consists of topical antibiotic agents, such as mupirocin 2%. An oral antibiotic, such as cephalexin or dicloxacillin, is recommended for systemic disease.

m Brodell RT, Williams L. Taking the itch out of poison ivy. Are you prescribing the right medication? 

 1999;106:69-70.m Crosti C, Lodi A. Pompholyx: a still unresolved kind of eczema. 

1993;186:241-242.m Hirschmann JV. Impetigo: etiology and therapy. 

2002;22:42-51.m Kunkel DB, Spoerke DG. Evaluating exposures to plants. 

1984;2:133-144.m Wilkinson JD. Vesicular palmoplantar eczema. In: Freedburg IM, Eisen AZ, Wolff K, eds. 

Fitzpatrick's Dermatology in General Medicine.

 5th ed. New York: McGraw-Hill; 1999:1489-1494.

Photo Finish: Acute Dx: What Cause of Sudden Illness? rhus dermatitis

 an infection with sometimes serious sequelae that involves the soft tissue of the orbit posterior to the orbital septum. Children are more likely than adults to contract orbital cellulitis; the median age of those affected is 7 years. Preseptal cellulitis--the other major infection of the ocular and adnexal orbital tissue--involves the soft tissue of the eyelids and periocular region anterior to the orbital septum and is considered less severe.

 Orbital cellulitis has a variety of causes:

•Direct extension of infection from the periorbital structures--usually the paranasal sinuses, but also the face, globe, eyelids, ocular adnexum or other periocular tissue, or lacrimal sac.

•Direct inoculation of the orbit from trauma or surgery.

Venous drainage from the middle third of the face, including the paranasal sinuses, occurs mainly via the orbital veins. Because these veins do not have valves, they allow antegrade and retrograde passage of infection. Osteomyelitis of the orbital bones, phlebitis of the facial veins, and dental infection may also result in orbital cellulitis.

Ethmoidal sinusitis accounts for more than 90% of all cases of orbital cellulitis. Edema of the sinus mucosa with subsequent blockage of normal sinus drainage allows indigenous microflora to proliferate and invade the edematous mucosa, which results in suppuration. The organisms, usually aerobic non-spore-forming bacteria, gain access to the orbit through the thin bones of the orbital walls, venous channels, foramina, and dehiscences. Subperiorbital and intraorbital abscesses may occur.

Any injury involving perforation of the orbital septum may secondarily result in orbital cellulitis. Orbital inflammation may occur as soon as 48 to 72 hours after an injury. In the case of a retained orbital foreign body, the onset of inflammation may be delayed for months.

Orbital cellulitis occurs more frequently during the winter because of the increased incidence of sinusitis. Before the advent of antibiotics, the mortality associated with orbital cellulitis was 17%; 20% of survivors became blind in the affected eye. Prompt diagnosis and appropriate antimicrobial therapy have significantly reduced these rates.

Cardinal signs and symptoms include proptosis, ophthalmoplegia, and chemosis, which result from elevated intraorbital pressure and which may be associated with orbital pain and tenderness, purulent nasal discharge, conjunctival hyperemia, and resistance to retropulsion of the globe. Other findings include limited ocular motility, pain with eye movement, reduced visual acuity, orbital congestion, headache, fever, lid edema, rhinorrhea, and malaise.

Suspect orbital cellulitis in any child who presents with facial or dental infection in association with any of the above findings.

Leukocytosis with a left shift is common. It is best to obtain blood cultures before administering antibiotics, although cultures infrequently reveal the responsible organism. Send samples of purulent nasal material for cultures and Gram staining.

High-resolution CT scans, including axial and coronal views (

), are imperative. MRI may help de-fine orbital abscesses and evaluate the possibility of cavernous disease. Consultation with specialists in infectious disease, ophthalmology, or otolaryngology is recommended. 

 Prompt hospitalization and administration of intravenous antibiotic therapy are imperative for successful treatment. Therapy for orbital cellulitis associated with ethmoidal sinusitis should include coverage for 

Staphylococcus aureus, Haemophilus influenzae,

Dental infections that result in maxillary and orbital cellulitis are typically attributable to microorganisms indigenous to the mouth, including anaerobes. The usual culprits are 

 species. Soft tissue infections of the eyelids and face are most commonly caused by staphylococci and 

 species are found to be the culprit organisms. Tetanus prophylaxis should be updated as needed.

The infection in the present case was thought to have resulted from maxillary sinusitis. The patient was admitted to the hospital, where she was treated with intravenous ampicillin and sulbactam sodium. The erythema decreased significantly after 1 day and the swelling after 3 days. On discharge, the patient underwent an 18-day course of treatment with amoxicillin and potassium clavulanate and recovered completely.

Case and images courtesy of David Effron, MD, Case Western Reserve University.

 Steinkuller PG, Jones DB. Microbial preseptal and orbital cellulitis. In: Tasman W, ed. 

The child has orbital cellulitis, an infection with sometimes serious sequelae that involves the soft tissue of the orbit posterior to the orbital septum. Children are more likely than adults to contract orbital cellulitis; the median age of those affected is 7 years. Preseptal cellulitis--the other major infection of the ocular and adnexal orbital tissue--involves the soft tissue of the eyelids and periocular region anterior to the orbital septum and is considered less severe.

e-Photo Quiz: Answer to Last Month’s Online Challenge - Orbital cellulitis

 The parents of a 4-year-old girl are concerned because she has experienced hair loss for several weeks. The child is otherwise healthy and active, has no known disorders, and takes no medications.

 an entity first described in 1907 in girls and women in Greenland who styled their hair in a ponytail. It is found primarily in African American women and girls and is also seen in Japan. In India, the condition is observed in Sikh men who pull their scalp hair into a bun and tightly roll their beard hair.

Hairstyling practices such as braiding, cornrows, and the use of weaves and rollers may also cause traction alopecia. Sustained tension on the scalp results in breakage of the outermost hairs. Hair loss is often symmetric; it frequently occurs along the frontotemporal hairline. The initial loss is reversible; however, prolonged traction may result in permanent hair loss.

In the first stages of traction alopecia, pruritus and perifollicular erythema are often present. They may be accompanied by secondary hyperkeratosis that resembles seborrheic dermatitis. Over time, scales and pustules may develop. Eventually, follicular atrophy results in the replacement of normal long, coarse hair by thinner, finer short hair.

Traction alopecia may be marginal or nonmarginal. Marginal alopecia (alopecia linearis frontalis) is a pattern of hair loss associated with the use of rollers, tight curlers, or straighteners in childhood. It occurs in the temporal region of the scalp and extends in a triangle from the preauricular area. Chignon alopecia is a form of nonmarginal alopecia that is characterized by hair loss in the occipital region where a bun is located. This condition is typically seen in middle-aged women who have pulled their hair into a bun for many years.

Early diagnosis is important to help prevent irreversible hair loss. Once the offending practices have been discontinued, it may take several months before regrowth occurs. Antibiotics may be required for secondary inflammation and infection. No medical treatment is available once hair loss has become irreversible; hair transplantation procedures may be considered.

 a traumatic alopecia, is a psychiatric disorder characterized by compulsive hair pulling that results in patchy hair loss. Hair loss may cover from several centimeters to large portions of the scalp. In addition to the scalp, the eyebrows, eyelashes, and pubic area may be affected. Treatment is directed toward the underlying disorder. Most adult and teenage patients are women; in younger patients, boys are affected slightly more often than girls. In children, nail biting may be seen concurrently with trichotillomania.

 is the most common dermatophytosis of childhood (it accounts for up to 90% of dermatophytoses in children younger than 10 years) and is characterized by partial alopecia and scaly patches. This superficial fungal infection affects the skin of the scalp, eyelashes, and eyebrows. The affected areas have hairs broken 1 to 3 mm above the skin. Patches of hair loss occur singly or in groups and are usually round or oval. 

 are the usual culprits. The diagnosis is confirmed by Wood lamp examination, potassium hydroxide wet-mount preparations, or fungal cultures.

is a chronic dermatosis that primarily affects women aged 40 to 60 years. The lesions consist of erythematous papules or plaques with slight to moderate scaling and are characterized by central hypopigmentation with peripheral areas of hyperpigmentation. The lesions are most commonly seen on the head and neck but may extend to the oral cavity, arms, palms, and soles. Atrophy and scarring alopecia of the scalp are typical findings.	

 Hantash BM, Schwartz RA. Traction alopecia in children. 

 2003;71:18-20.m Muller SA. Trichotillomania and related disorders. In: Orfanos CE, Happle R, eds. 

 Berlin: Springer-Verlag; 1990.m Springer K, Brown M, Stulberg DL. Common hair loss disorders. 

The parents of a 4-year-old girl are concerned because she has experienced hair loss for several weeks. The child is otherwise healthy and active, has no known disorders, and takes no medications.

Author | David Effron, MD

Articles

Case In Point: Pneumatosis Cystoides Intestinalis: An Unusual Radiographic Finding

Photo Finish: Acute Dx: What Cause of Sudden Illness? rhus dermatitis

e-Photo Quiz: Answer to Last Month’s Online Challenge - Orbital cellulitis

Traction Alopecia