Brighita Weinberg, MD

A female infant born at 29 weeks' gestation after premature membrane rupture was admitted to the neonatal ICU in respiratory distress. Delivery was complicated by maternal fever and meconium-stained amniotic fluid. The mother had not been screened for group B streptococci (GBS) infection and had received 1 dose of penicillin less than 4 hours before delivery. Results of a complete blood cell (CBC) count, cerebrospinal fluid (CSF) analysis, and urinalysis were unremarkable; cultures showed no bacterial growth. The infant received a 10-day course of antibiotics. Findings on cranial ultrasonograms at 2 and 10 days of life were normal. She was discharged on day 37 of life in stable condition.

Figure 1 – Coronal cranial ultrasonogram shows hydrocephalus. Echogenic ependymal lining of the lateral ventricles represents ventriculitis. Linear echogenic strands within the ventricles are consistent with synechiae. 

Sixteen days later, the infant was brought to the hospital because of poor feeding, diarrhea, and lethargy of 24 hours' duration. On admission, she appeared lethargic and was afebrile, tachypneic, and tachycardiac. Her anterior fontanelle was flat, with widened sagittal sutures. Occipital frontal circumference (OFC) was 35 cm (90th percentile for adjusted gestational age); the OFC at birth was 27 cm (50th percentile). Weight was 2.7 kg (25th to 50th percentile); birth weight was 1.43 kg (50th to 90th percentile). Remaining examination findings were normal. Serum white blood cell count was 2700/μL. Results of other parameters in the CBC count, urinalysis, and basic metabolic panel were normal. CSF protein and glucose levels were low, 2 mg/dL and 15 mg/dL, respectively. CSF, blood, and urine cultures were positive for GBS.

Figure 2 – An ultrasonogram obtained 7 days later demonstrates resolution of the hydrocephalus and reabsorption of the linear intraventricular strands. 

On day 2 of hospitalization, a cranial ultrasonogram revealed hydrocephalus, with echogenic ependyma of the lateral ventricles, related to ventriculitis (

). Linear synechiae noted within the ventricles were characteristic of inflammation. The 3rd and 4th ventricles were normal. The findings represented a noncommunicating hydrocephalus at the level of the Monro foramen. A week later, a follow-up ultrasonogram showed resolution of the hydrocephalus and reabsorption of the intraventricular echogenic strands (

); there was no evidence of a focal brain lesion. A contrast-enhanced CT scan on day 17 revealed enhancement of the ependymal lining of the lateral ventricles with no hydrocephalus (

). Slight cisternal enlargement at the left lateral cerebral convexity and right frontal region, consistent with sympathetic effusion (exudative effusion caused by disease in nearby structure), was also present. The parenchyma was normal.

Figure 3 – Contrastenhanced CT scan shows enhancement of the ependyma of the lateral ventricles, consistent with ventriculitis. Slight prominence of the cisterns at the left cerebral convexity and right frontal region is also present. 

After 2 weeks of treatment with benzylpenicillin and gentamicin, repeated cultures were negative for GBS. However, GBS antigen remained in the CSF. The antibiotic therapy was continued for 1 more week. Four weeks after admission, cranial ultrasonographic findings were unremarkable.

GBS infection is a common cause of neonatal sepsis and meningitis; its reported incidence is 0.5 to 3.7 per 1000 live births.1 Neonatal sepsis may be categorized as early or late onset. The incidence of early-onset disease is 2 to 4 per 1000 live births.1 Early-onset sepsis occurs in neonates aged 5 days or younger and usually presents as pneumonia and septicemia. Acquisition of GBS infection in this age-group is from the maternal genital tract in utero or during passage through the birth canal.

 The incidence of late-onset sepsis is 1 to 2 per 1000 live births.

 It typically presents as meningitis after the first week of life and is not often associated with maternal obstetrical complications.

 GBS infection usually occurs as an isolated illness characterized by the absence of antecedent obstetric or perinatal complications and the presence of more specific signs of meningitis (eg, fever, lethargy, seizures).

 Acute hydrocephalus as a sequela of GBS infection has been described in 6 of 74 patients with meningitis.

 Postinfectious hydrocephalus is secondary to purulent exudates from the meninges and ependymal surfaces of the ventricles, perivascular inflammation, and brain edema. Pus may accumulate in significant quantities in the sulci, subarachnoid space, and basal cisterns. These exudates and adhesions can impede the flow of CSF through the narrowest points of the ventricular system-such as the Monro foramen, aqueduct, and foramina of the 4th ventricle-or impair the absorption of the CSF by inflamed arachnoid villi. When the fontanelles are open, exudates can accumulate without increasing intracranial pressure. GBS infection initiates a cascade of molecular and biological events that lead to impaired cerebral perfusion, disruption of the blood-brain barrier, edema, intracranial hypertension, and cell death.

 In one study, 90% of neonates with GBS meningitis and neurological sequelae presented with lethargy and fever.

The patient presented here was afebrile with increased OFC and ultrasonographic evidence of hydrocephalus. We assumed that the presence of ventriculitis and synechiae induced by the GBS meningitis resulted in an obstructive hydrocephalus at the level of the Monro foramen. Previous reports of GBS ventriculitis describe initial presentations with obstructive hydrocephalus or septation formation,

 suggesting that ventriculitis may occur as a primary process.

 Cranial ultrasonography is an excellent imaging modality in the initial evaluation of infants with suspected meningitis and the associated complications. Serial ultrasonograms are helpful in monitoring the patient’s response to treatment. Obtaining MRI and contrast-enhanced CT studies during management is also helpful, because they provide better soft tissue resolution and visualization of the posterior fossa and can detect small extra-axial fluid collections, which may be missed on cranial ultrasonography.

 The recommended treatment of GBS infection is benzylpenicillin, either alone or in combination with an aminoglycoside

-which is how this patient was treated. In most cases reported to date, patients who received intravenous antibiotics at presentation ultimately required placement of a ventriculoperitoneal shunt because of progression of obstructive hydrocephalus; in some patients, antibiotic treatment failed because of the development of intraventricular loculations.

 Spontaneous resolution of acute hydrocephalus secondary to intraventricular hemorrhage without aspiration of CSF has been reported.

The atypical clinical presentation and spontaneous resolution of hydrocephalus in this premature infant was unique in that she was treated at birth with a 10-day course of antibiotics and presented with GBS meningitis at an adjusted gestational age of 36 weeks. The infant's head circumference remained stable throughout her hospitalization. Thus, ventriculoperitoneal shunt placement was unnecessary. Since discharge, she has been well and developing normally. At 9-month follow-up, she was developmentally appropriate for an infant aged 6.5 months (adjusted gestational age), with a weight of 3.8 kg (50th percentile) and OFC of 36.2 cm (50th to 75th percentile).

1. Srinivasan L, Ramanan R. Hydrocephalus secondary to group B streptococcal meningitis. 

2. Mulder CJ, Zanen HC. Neonatal group B streptococcal meningitis. 

3. Neonatal Meningitis: Infections in Neonates: 

http://www.merck.com/mmpe/sec19/ch279/chch279k.html

4. Miyairi I, Causey KT, DeVincenzo JP, Buckingham SC. Group B streptococcal ventriculitis: a report of three cases and literature review. 

5. Yikilmaz A, Taylor GA. Sonographic findings in bacterial meningitis in neonates and young infants. 

6. Abubacker M, Bosma JJ, Mallucci CL, May PL. Spontaneous resolution of acute obstructive hydrocephalus in the neonate. 

Articles

A female infant born at 29 weeks' gestation after premature membrane rupture was admitted to the neonatal ICU in respiratory distress.

Hydrocephalus Secondary to GBS Meningitis

For 3 months, a 9-year-old boy   had swelling of the left upper   arm. An MRI scan obtained at   another facility 1 week after   onset showed extensive edema   of the soft tissue at the midhumeral   level. Laboratory results,   including complete blood cell (CBC)   count and Lyme titer, were normal.



   Two weeks before presentation, the   swelling had worsened and associated   pallor, fatigue, and lower extremity   pains had developed. A repeated   CBC count was consistent with   leukemia: white blood cell (WBC)   count, 336,000/μL (with 96% blasts);   hemoglobin level, 5.2 g/dL; platelet   count, 36,000/μL. The patient was   referred for further evaluation.

   On examination, the child was   pale, with splenomegaly but no   hepatomegaly or lymphadenopathy.   The left arm mass was firm and nontender,   without inflammation. Bone   marrow aspiration revealed hypercellular   marrow, with more than 95%   blasts. Flow cytometry of the bone   marrow aspirate was consistent with   precursor B-cell acute lymphoblastic   leukemia (ALL). Fluorescence in   situ hybridization analysis revealed   clonal rearrangement of the MLL   gene at 11q23 (present in 87% of   cells). Spinal fluid revealed no cells.

   Before treatment, a repeated   MRI scan showed a large (15 X 4.5-   cm) mass medial to the humerus infiltrating   the biceps brachii, brachioradialis,   and triceps muscles with   extension into the subcutaneous fat.   The ulna neurovascular bundle, brachial   artery, and vein were all encircled   by the mass. On T-1 pulse sequence,   the mass was isointense to   the muscle with a normal bone cortex.   The bone marrow showed low   signal intensity, which signified an   infiltrative process. A T-1 fat-saturated   sequence revealed high signal intensity   of the bone marrow, related   to an absence of normal fatty bone   marrow. T-1 fat-saturated pulse sequence   postgadolinium injection   showed marked enhancement of the   soft tissue mass and bone marrow.



   Virtually the entire humerus was involved,   with no cortical erosion (A).   ALL is one of the most common   malignancies in children, accounting   for 30% of all childhood   cancers.

 This primary malignancy   arises from bone marrow. It can occur in any age-group and is slightly   more common in girls in the first   decade of life, with a peak incidence   between 2 and 5 years. Leukemic   skin infiltrations can involve the epidermis,   dermis, and subcutaneous   fat. These lesions are seen in 1% of   patients.



   Children with ALL usually present   with cytopenia, fever, and bone   pains.

 Although asymptomatic skeletal   involvement may be seen in 40%   to 60% of patients at presentation, extramedullary   leukemic masses outside   the CNS and testes are exceedingly   rare.

 The few that are seen   typically involve the eyes, ovaries,   kidneys, tonsils, mediastinum, masseter   muscle, scrotum, bone, or paraspinal   areas.

 Such extramedullary   leukemic masses are almost always   a manifestation of relapsed ALL and   not the primary presenting feature.   However, it is important to consider   ALL in the differential diagnosis   when evaluating patients with a soft   tissue mass.

   This patient received standard   chemotherapy consisting of prednisolone,   vincristine, daunorubicin,   PEG asparaginase, and intrathecal   methotrexate. Within a week, his   WBC count decreased to less than   1000/μL, with no blasts on the blood   smear, and flow cytometry showed   no abnormal immature B-cell populations.   These findings were consistent   with remission.

   An MRI scan obtained 6 days   after therapy showed marked decreased   enhancement of the soft tissue   (B). On all sequences (T-1, T-1   fat-saturated, and fat-saturated with   contrast), the size of the mass had   decreased and normal muscle tissue   was visible. However, the bone marrow   signal was still abnormal, signifying   persistence of the infiltrative   process replacing the normal bone   marrow. This patient will continue to   receive close monitoring by the   hematology/oncology department.

 Ahn JY, Choi EW, Kang SH, Kim YR. Isolatedmeningeal chloroma (granulocytic sarcoma) in achild with acute lymphoblastic leukemia mimickinga falx meningioma. 

 Franck P, Duffner U, Schulze-Seemann W, et al.Testicular relapse after 13 years of complete remissionof acute lymphoblastic leukemia. 

 Taljanovic MS, Hulett RL, Graham AR, et al.Acute lymphoblastic leukemia of the skin and subcutaneoustissues; the first manifestation of diseasein a 6-month-old infant: a case report with literaturereview. Emerg Radiol. 2004;11:60-64.  
  

 Jonsson OG, Sartain P, Ducore JM, BuchananGR. Bone pain as an initial symptom of childhoodacute lymphoblastic leukemia: association with nearlynormal hematologic indexes. J Pediatr. 1990;117(2, pt 1):233-237.  
  

 Sadawaite S, Jijina F, Nair CK, et al. An unusualpresentation of pediatric acute lymphoblastic leukemia.Indian J Hematol Blood Transfus. 2008;24:59-62.  
  

 Wimperis JZ, Brandt LJ, O’Connor S, et al. Unusualpresentation of common acute lymphoblasticleukemia antigen-positive extramedullary disease inchildhood. Two patients with isolated masseter muscleinvolvement. Cancer. 1992;70:897-901.

For 3 months, a 9-year-old boy had swelling of the left upper arm. An MRI scan obtained at another facility 1 week after onset showed extensive edema of the soft tissue at the midhumeral level. Laboratory results, including complete blood cell (CBC) count and Lyme titer, were normal.

Brighita Weinberg, MD

Articles

Hydrocephalus Secondary to GBS Meningitis

Acute Lymphoblastic Leukemia Presenting as Soft Tissue Mass