Sanjay Chawla, MD

 Full-term, 5100 g girl delivered by spontaneous vaginal delivery after an uncomplicated pregnancy to a 31-year-old gravida 6, para 2 mother. Prenatal ultrasonograms showed normal findings at 17 weeks and 31 weeks, although view of neck was suboptimal at 31 weeks. Neonate cried immediately after birth. Apgar scores, 9 at 1 minute and 9 at 5 minutes. 

 After delivery, infant noted to have a soft, fluctuant, ill-defined, nontender, nonpulsatile mass measuring 5  13 cm on left posterolateral side of the neck 

. Overlying skin not warm or discolored; mass not attached to it. Mass transilluminated well 

Figure 1 - This ill-defined mass was present in the left posterolateral part of the neck. The overlying skin was normal.

Figure 2 - Transillumination through the entire mass suggests that the mass is filled with fluid.

Figure 3 - Ultrasonogram of the mass shows that it is composed of multiple, large, cystic spaces separated by thin septae (arrow). Some debris is visible within the fluid. No solid mass was found.

Figure 4 - Color Doppler ultrasonogram of the septated cystic mass in the posterior left neck shows no flow within the cysts, confirming that these are not vessels. 

Ultrasonography showed a large multiseptated cystic mass in the posterior part of the left side of the neck. No obvious vascular flow evident within the mass 

No other unusual physical findings. Normal vital signs; no signs of respiratory distress or dysphagia. No other masses clinically evident on physical examination.

 In children, the differential diagnosis of a neck mass is broad. When a mass is present at birth, however, the differential is more limited. In newborns, diagnostic considerations include branchial anomalies, thyroglossal duct cyst, thymic cyst, teratoma, dermoid cyst, lymphangioma, and hemangioma. A soft, fluctuant, ill-defined, transilluminating mass in the posteriolateral part of the neck at birth suggests a lymphangioma or cystic hygroma. 

Cystic hygromas (a type of lymphangioma) are the result of maldevelopment and obstruction of the lymphatic system.

 This leads to sequestrations of lymphatic tissue that do not communicate with the normal lymphatic system. About 50% to 65% of cystic hygromas are present at birth; 90% appear by the second year of life. They are located in the posterior triangle of the neck in about 75% of cases and in the axilla in about 20% of cases. The remainder occur in the mediastinum, retroperitoneum, pelvis, and groin.

 The cyst may be unilocular, but more often there are numerous cysts of various sizes that permeate surrounding structures.

Symptoms are related to the lesion's size and location. Most patients are asymptomatic, but massive congenital lymphatic malformations may cause dysphagia, feeding problems, and airway compromise. Cysts can become enlarged with viral infection or hemorrhage within the lesion because of the presence of nests of poorly supported vascular channels. When lymphangiomas are detected before 30 weeks of gestation, there is a high incidence of associated anomalies-including an abnormal karyotype and hydrops fetalis.

Surgical resection is the recommended standard of management.4 Elective surgical excision between 4 and 12 months is indicated for asymptomatic patients.

 Complete surgical resection is often difficult, and clinical recurrence is common. Aspiration may temporarily reduce the lesion's size.

Sclerotherapy with OK-432, a lyophilized mixture of 

 and benzylpenicillin, has been shown to be an excellent treatment for patients with macrocystic lymphatic malformations.

Leung AKC, Robson WLM. Cystic hygroma. Consultant For Pediatricians. 2006;12:787-789. 

Albanese CT, Sylvester KG. Pediatric Surgery. In: Doherty GM, Way LW, eds. Current Surgical Diagnosis and Treatment. 12th ed. New York: McGraw-Hill; 2006:1279-1280. 

Hartman GE, Boyajian MJ, Choi SS, et al. Surgical care of conditions presenting in the newborn. In: MacDonald MG, Mullett MD, Seshia MM, eds. Avery's Neonatology: Pathophysiology and Management of the Newborn. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2005:1099-1100. 

Fageeh N, Manoukian J, Tewfik T, et al. Management of head and neck lymphatic malformations in children. J Otolaryngol. 1997;26:253-258. 

Peters DA, Courtemanche DJ, Heran MK, et al. Treatment of cystic lymphatic vascular malformations with OK-432 sclerotherapy. Plast Reconstr Surg. 2006;118:1441-1446. 

Articles

Ultrasonography showed a large multiseptated cystic mass in the posterior part of the left side of the neck. No obvious vascular flow evident within the mass (Figures 3 and 4).

Cystic Hygroma in an Infant Girl

A 15-year-old Hispanic boy with refractory T-cell acute lymphoid leukemia was hospitalized because of fever and pain and swelling of the right knee of 3 days' duration. The patient was taking nelarabine for a recurrence of his leukemia, which was diagnosed a year earlier. He appeared nontoxic. His temperature was 39.2

F). Other vital signs were within normal limits. The right knee was warm and tender, with mild restriction of movement.

The white blood cell count was 9100/µL, with an absolute neutrophil count of 5733/µL, hemoglobin level of 9.2 g/dL, and platelet count of 36,000/µL. A radiograph and ultrasonogram of the right knee revealed moderate joint effusion. Intravenous cefepime and vancomycin were started pending the results of blood and urine cultures.

Arthrotomy with debridement of the right knee was performed; 150 mL of cloudy straw-colored fluid was removed. Fluid analysis revealed 7000 nucleated cells/mL, with 92% neutro- phils. Gram stain showed no organisms. Synovial fluid and blood cultures grew 

 After sensitivity testing, vancomycin was discontinued and cefepime was replaced with ceftriaxone.

MRI of the right knee showed an area of enhancement in the anterior distal femoral metaphysis that represented early focal osteomyelitis (

The patient underwent a second arthrotomy with irrigation and debridement of the right knee. Culture of the drained fluid again grew 

 which was sensitive to ceftriaxone, ciprofloxacin, and cefepime. The swelling and pain of the right knee decreased after 10 days. Subsequent blood culture results were negative.

On further questioning, it was learned that the patient had been eating fresh rattlesnake meat (that was seasoned and grilled) for a month after the diagnosis of leukemia. The boy's family believed that the snake meat would cleanse his blood. Consumption of rattlesnake meat is associated with 

 infection, which is a cause of osteomyelitis.

RATTLESNAKE MEAT "CURE"--A CAUSE OF SALMONELLOSIS

 However, it has occurred in many Hispanic persons who consumed rattlesnake meat, capsules, or powders as a form of alternative medicinal therapy.

 These preparations are widely believed to be beneficial for many illnesses, including arthritis, cancer, and HIV infection.

 A survey of the literature shows that many cases of 

infection had an underlying disease, such as AIDS, systemic lupus erythematosus, cancer, or leukemia.

is a facultative anaerobic Gram-negative bacillus of the family Enterobacteriaceae. It is found in many animals, including reptiles, fowl, ducks, rats, dogs, and cats.

 Reptiles, particularly snakes, are the natural reservoir of 

 They are asymptom- atic carriers of this pathogen and are probably infected through contaminated water, food, or soil. The bacteria usually exist as commensal flora in the animal gut.

 They are thought to enter the human host through the GI tract after consumption of contaminated food products.

Gastroenteritis is the most common clinical presentation of 

 infection; gastroenteritis associated with 

 is similar to that of salmonellosis. The incubation period is usually 2 to 48 hours and is characterized by fever, abdominal pain, vomiting, and diarrhea. The risk of dehydration is substantial because of the severity of diarrheal illness. In addition to gastroenteritis, bacteremia, sepsis, meningitis, osteomyelitis (as in this case), pneumonia, otitis media, peritonitis, and wound infection have been reported.

 A carrier state is not uncommon and recurrence of 

 sepsis in an immunocompromised patient after 1 year has been reported.

 has been isolated from blood, urine, stool, pleural fluid, and lymph nodes. The infection can be confirmed with culture of the affected area, serological testing, or polymerase chain reaction assay.

 appears to be susceptible to commonly prescribed antibiotics (

 Drugs for salmonellosis include ampicillin, trimethoprim/ sulfamethoxazole, and chloramphenicol, although fluoroquinolones, aminoglycosides, and vancomycin also have been used in various combinations with apparent effectiveness.

 Immunocompromised patients require prolonged therapy.

 Infection has recurred after apparent clearance of the organism demonstrated by negative cultures.

It is essential to ask all patients whether they use of alternative medicinal therapy--especially those in whom conventional medical treatment fails. Advise those who do about the potentially harmful outcomes associated with such therapies and with certain folk remedies.	*

Hoag JB, Sessler CN. A comprehensive review of disseminated 

 infection with an illustrative case presentation. 

Riley KB, Antoniskis D, Maris R, Leedom JM. Rattlesnake capsule-associated 

Cone LA, Boughton WH, Lehv LH. Rattlesnake capsule-induced 

Bhatt BD, Zuckerman MJ, Foland JA, et al. Disseminated 

 infection associated with rattlesnake meat ingestion. 

Babu K, Sonnenberg M, Kathpalia S, et al. Isolation of salmonellae from dried rattlesnake preparations. 

Waterman SH, Juarez G, Carr SJ, Kilman L. 

 infection in Latinos associated with rattlesnake folk medicine. 

Kelly J, Hopkin R, Rimsza ME. Rattlesnake meat ingestion and 

 infection in children: case report and review of the literature. 

Fainstein V, Yancey R, Trier P, Bodey GP. Overwhelming infection in a cancer patient caused by 

Ripley GD. Mexican-American folk remedies: their place in health care. 

Mermin J, Hoar B, Angulo FJ. Iguanas and 

 infection in children: a reflection of the increasing incidence of reptile-associated salmonellosis in the United States. 

Mahajan RK, Khan SA, Chandel DS, et al. Fatal case of 

 gastroenteritis in an infant with microcephaly. 

Libby SJ, Lesnick M, Hasegawa P, et al. Characterization of the spv locus in 

Hentges K, Shields CE, Cantu C. Folk medicine and medical practice. 

A 15-year-old Hispanic boy with refractory T-cell acute lymphoid leukemia was hospitalized because of fever and pain and swelling of the right knee of 3 days' duration. The patient was taking nelarabine for a recurrence of his leukemia, which was diagnosed a year earlier. He appeared nontoxic. His temperature was 39.28C (102.68F). Other vital signs were within normal limits. The right knee was warm and tender, with mild restriction of movement.

Author | Sanjay Chawla, MD

Articles

Cystic Hygroma in an Infant Girl

Folk Remedy as a Cause of Septicemia in a Child With Leukemia