REFERENCES
1. The Child Day Care Infectious Disease Study Group: Public health considerations of infectious diseases in child day care centers. J Pediatr 1984;105:683-701
2. Goodman RA, Osterholm MT, Granoff DM, et al: Infectious diseases and child day care. Pediatrics 1984;74:134-139
3. Hadler SC, Webster HM, Erben JJ, et al: Hepatitis A in day-care centers-A community-wide assessment. N EngI J
Med 1980;302:1222-1227
4. Farer LS, Flynn JP, Reza RJ, et al: Control of tuberculosis.
Am Rev Respir Dis 1983;128:335-342
5. Enarson D, Ashley MJ, Grzybowski 5: Tuberculosis in im-migrants to Canada. Am Rev Respir Dis 1970;119:11-18
Von Hippel-Lindau
Disease
in
an Adolescent
Von Hippel-Lindau (VHL) disease is an
autoso-mal dominant disorder characterized by
heman-gioblastomas of the CNS and retina and a variety
of other cystic and neoplastic lesions throughout
the body. It is a rare condition often unfamiliar to
pediatricians because it generally does not present
as a fully developed condition in childhood. Visceral
lesions, although often present at an early age, are
usually asymptomatic. Symptoms, when they arise
in adolescense, are most commonly caused by
reti-nal disease, but these may also be silent. Diagnosis
is, therefore, delayed until most of the
manifesta-tions of the disease become evident, as exemplified
by the following case of a 16-year-old girl who
presented in uncommon fashion with abdominal
pain and visceral and cerebellar components
with-out eye disease.
CASE REPORT
A 16-year-old white girl was seen in the emergency
room because of an approximately 10-month history of
midepigastric abdominal pain, nausea, vomiting, and
hic-cups. Periods of well-being had been interspersed with
days to weeks of abdominal distress resulting in a 7-kg weight loss. Protracted vomiting 2 weeks earlier had
necessitated a two-day admission to another hospital for
IV hydration. Diagnostic evaluation done at that time
included an upper gastrointestinal series and oral
chole-cystogram, the results of which were normal. Antacid
therapy had been instituted in the interim with little
symptomatic relief.
The patient had previously been in good health and
was a bright, well-adjusted high school junior. Past
med-ical history included only a tonsillectomy at 5 years of
age and all other systems seemed normal. The patient
specifically denied any headache, fever, rashes,
arthral-gias, diarrhea, hemetemesis, or melena. She was not
Reprint requests to (I.R.S.) Division of Adolescent Medicine, Schneider Children’s Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042.
PEDIATRICS (ISSN 0031 4005). Copyright © 1987 by the
American Academy of Pediatrics.
sexually active and denied drug use. There was no family
history of any gastrointestinal disturbance.
When first seen, the patient was pale and cachectic;
she vomited repeatedly and was in obvious pain. Her vital
signs were as follows: temperature 37.GC, pulse rate 80
beats per minute, respiratory rate 18 breaths per minute,
and BP 94/62 mm Hg. She was 161.3 cm tall (40th
percentile) and weighed of 38.1 kg (<5th percentile).
Physical examination findings were normal with the
ex-ception of her abdomen. She had epigastric tenderness
without peritoneal signs, abdominal masses, or
organ-omegaly. Initial laboratory tests included CBC count,
serum electrolytes, amylase, urinalysis, and ESR, the
results of which were all normal.
She was admitted to the adolescent unit and hydrated
with IV fluids. ECG, chest roentgenogram, and liver and
thyroid function test results were all normal. There was
no occult blood in her stool. Because of the cyclic nature
of her symptoms, the diagnosis of abdominal migraine
was entertained, and an EEG was performed and the
results were normal. Vomiting decreased during the
fol-lowing week, although the patient continued to complain
of nausea and epigastric discomfort. A noncontrast CT
examination of the head did not show any cortical
atro-phy consistent with anorexia nervosa. An endoscopy with
directed duodenal biopsies showed changes consistent
with mild esophagitis and chronic duodenitis; therefore,
antacid and cimetidine treatment was instituted. Her
symptoms did improve; however, despite careful
moni-toring of intake and accurate caloric counts, she did not
gain weight. Continuous nasogastric feedings were tried,
and evaluation for an underlying collagen vascular
dis-order was undertaken. This yielded an ANA positive at
1:640 in homogeneous pattern. The remainder of results
from an extensive list of immunologic tests including
rheumatoid factor, complements, and other specific
an-tibody tests were all negative, with the sole exception of
a positive antihistone antibody.
By the fifth week of hospitalization, her abdominal
pain had worsened, and severe vomiting recurred. Results
of an upper gastrointestinal series, an esophageal pH
probe, and a serum gastrin test were all found to be
normal. Metoclopramide was added to her drug regimen,
and symptoms again seemed to respond to the therapeutic
maneuver, but only temporarily and without weight gain.
Peripheral and later central hyperalimentation became
necessary. A repeat endoscopy performed during the
eighth week of hospitalization showed no remaining
evi-dence of inflammation, and acid perfusion of the
esoph-agus (Bernstein test) failed to reproduce the patient’s
DISCUSSION
Fig I. Transverse sonogram through upper abdomen at level of pancreas (P). A cyst is noted in tail of pancreas (arrow) to left of aorta. R, right; 5, spine; a, aorta.
throughout the pancreas (Fig 1). This finding was
con-firmed on abdominal CT scan (Fig 2). Her amylase level
had remained normal and an endoscopic retrograde
cho-langiopancreatogram showed no pathologic ductal
con-dition, however, the ANA had increased to 1:1280 and
the ESR had increased to 41. On the basis of these
findings, a presumptive diagnosis of lupus vasculitis was
made, and a trial of steroids was begun. Dramatic clinical
improvement occurred. Nausea, vomiting, and pain
corn-pletely resolved, and the patient was discharged from the hospital exactly 3 months after admission feeling well
and weighing 50 kg, on tapering doses of steroids. Kidney
and skin biopsy findings done to confirm the diagnosis
of systemic lupus erythematosus were normal.
Seventy-two hours after discharge, a global headache
developed with increased in severity and prompted
read-mission. Results of a neurologic examination, including
fundoscopy were normal. On lumbar puncture a protein
content of 420 mg/i#{174} mL was found. A CT scan of the
head with contrast now showed obstructive
hydrocepha-lus and a contrast-enhancing posterior fossa mass (Fig
3). Cerebral angiography delineated what appeared to be
a hemangioblastoma (Fig 4), and a tentative diagnosis of
Von Hippel-Lindau disease was made. The aorta, spine,
and kidneys were additionally visualized and were free of
tumor.
The patient underwent placement of a frontal Burr
hole for decompression, followed a day later by
suboccip-ital craniectomy with total excision of the tumor. Her
postoperative course was complicated by ventriculitis and
a urinary tract infection, but, overall, she did well. A
residual bilateral sixth cranial nerve palsy slowly re-solved. ANA decreased and ESR normalized. The
path-ologic specimen revealed a benign hemangioblastoma.
Her ophthalmologic examination for retinal angiomas
had repeatedly negative results, but specific postoperative
questions elucidated additional family history. Of
partic-ular note is that many female relatives, including the
patient’s 6-year-old sister, have had breast cysts. Her
mother was treated for a cystic spinal tumor as a child,
and a maternal uncle had had successful removal of a
cerebellar hemangioblastoma. This history combined
with the presence of pancreatic cysts and a cerebellar
hemangioblastoma establishes the diagnosis of Von Hip-pel-Lindau disease.
The constellation of lesions that has come to be
known as Von Hippel-Lindau disease was described
by Arvid Lindau’ in a classic 1926 monograph. Von
Hippel, in 1904, had described patients with retinal
hemangioblastomas (angiomatosis retinae). The
eponym became established after Lindau noted the
occurrence of cerebellar hemangioblastomas (the
so-called Lindau tumor) in 20% of these patients.
He also reported the occurrence of
hemangioblas-tomas in the medulla and spinal cord and visceral
lesions which included cysts of the pancreas and
kidney; adenomas of the liver, epidydimis, and
ad-renals; and renal cell carcinomas
(hypernephro-mas). With time came reports of additional lesions,
including cerebral and meningeal
hemangioblasto-mas, lung and ovarian cysts, and angiomas of bone,
liver, epididymis, and skin.24 Pheochromocytomas
were added to the syndrome list in the 1950s,57 and
in 1978, familial islet tumors were reported.8 In
spite of this expansive list of recognized lesions,
however, it should be emphasized that most of the
morbidity and mortality of the disease stems from
only six of the manifestations which include the
hemangioblastomas of the cerebellum, medulla,
spinal cord, and retina, the hypernephromas, and
the pheochromocytomas.
Knowledge of the genetics of Von Hippel-Lindau
evolved during the past half century. Lindau’
pointed out that 20% of his cases had a familial
incidence, and men and women were found to be
equally affected. In a pedigree spanning several
generations, M#{246}ller9found that the disease
ap-peared in approximately 50% of the descendents of
affected individuals. These observations led to its
classification as an autosomal dominant. It was
noted, however, that the disease occurred in certain
people with apparently unaffected parents, and it
was assumed, therefore, that the gene penetrance
was incomplete.’0 Estimates of gene penetrance in
different studies have ranged from 24%” to 5%,1O
but comparison is difficult because of varying
di-agnostic criteria, existence ofasymptomatic lesions,
and inclusion of patients of all ages. Many authors
have emphasized that continued follow-up of
sur-viving family members into late adult life is
asso-ciated with increasing incidence of the disease to
almost 100%.12.13
The most important lesion of Von Hippel-Lindau
is the cerebellar hemangioblastoma which is the
most frequent source of initial symptoms and, until
recently, the most common cause of death.
Symp-toms seldom occur before the age of 20 years,
a!-though it has been observed that clinical disease
seems to occur at earlier ages in successive
Fig 2. Top, Contrast-enhanced CT scan at about same level as sonogram of Fig 1. Note
previously shown large cyst in tail of the pancreas, with another small cyst anterior to it
(large arrow). Small cyst is also seen in head ofpancreas (small arrow). Bottom, At slightly
lower level, two cysts are seen in head of pancreas (arrow).
multiple in 10% of patients’ and cystic in 80%, and
manifests with increased intracranial pressure.’5”6
In contrast to our patient, more than 90% of
af-fected persons report headache.’7 There are
numer-ous reports, however, of similar paroxysms of
vom-iting with or without nausea, between which the
patient is perfectly well.’8”9 Some of these episodes
have preceded the acute symptoms by years, with
the average duration of complaints reported as 10
months.2#{176} One noteworthy patient of Kinney and
Fitzgerald2’ suffered from hiccups, nausea, and
vomiting for 3/2 years prior to her hospital
admis-sion. It is thought that the intermittency and
brev-ity of these patients’ hydrocephalic attacks are
re-sponsible for their relatively late papilledema
de-velopment.’8’22 Cerebellar symptoms and signs,
in-cluding ataxia, dysmetria, and nystagmus, may be
similarly delayed.
The occurrence of retinal hemangioblastomas in
Fig 3. Contrast-enhanced CT scan of head shows large enhancing tumor in inferior cerebellar vermis.
Fig 4. Vertebral arteriogram showing vascular lesion in anterior cerebellar vermis.
64%,23 and in those patients who have eye
involve-ment, visual problems are often the earliest
mani-festation. The typical ophthalmoscopic picture of
an involved retina shows a greatly dilated tortuous
artery/vein pair ending in a peripheral reddish
nod-ule (the hemangioblastoma).24 Tumors may occur
in other parts of the same retina, and with time,
both eyes may become involved. Untreated, the
lesion can undergo proliferative changes and cyst
formation with retinal detachment, glaucoma, and
blindness.25 In Lindau’s series,’ the appearance of
cerebellar symptoms trailed those of retinal disease
by 14 years. In several large pedigrees3’263#{176} in which
ocular disease was studied, it was present in most
of the patients in whom the diagnosis was made
before 20 years of age. The absence of eye findings
in our young patient with CNS disease, although
reported, is uncommon.3’
Abdominal pain, the patient’s primary complaint,
is unusual in Lindau’s disease. The etiology of the
symptom in this case remains unclear. Although
duodenal ulcer has been reported with CNS disease
and Von Hippel-Lindau disease,2 it was clearly not
present in this patient. Severe cystic involvement
of the pancreas, causing epigastric discomfort and
nausea, have been reported,3234 but in all of these
cases, an enlarged palpable pancreas was present
on abdominal examination. Hypernephromas,
which have been reported in 12% to 83% of cases
of Lindau’s disease’2 and have caused many deaths
from metastatic or uremic disease,23 tend to occur
later in life and are well demonstrated by CT scan.
In our patient, the pancreas was of normal size and
the kidneys were free of tumor by CT. The
resolu-tion of her abdominal pain with the removal of her
cerebellar tumor would indicate some central
mech-anism as a probable cause of her symptoms.
A most unusual aspect of this case previously
unreported in Von Hippel-Lindau disease is the
persistence of a positive ANA of homogeneous
pat-tern in high titers. Positive ANA findings are
known to occur in low titers in various neoplastic
diseases, including leukemias, lymphomas, and
cer-tam carcinomas.35 When positive ANAs develop in
patients with cancer, antibodies to histones are
found to be the main causative type,36 just as was
demonstrated in this patient. Because the ANA
titer did return to normal after removal of the
tumor, it is possible to speculate on the possible
usefulness of ANA as a tumor marker in this
dis-ease. Further study seems warranted.
This case also demonstrates the irreplaceable
importance of a detailed family history. Originally,
the diagnosis of Lindau’s disease required that both
retinal and cerebellar hemangioblastomas occur in
the same patient or that one of these two tumors
be present along with an inherited history of the
disease.22 In their extensive review of Von
Hippel-Lindau disease, Melmon and Rosen2 expanded the
criteria for diagnosis to include patients with any
single lesion of the Lindau complex, provided a
CNS hemangioblastoma has been proven in the
family. When pancreatic cysts were discovered on
CT examination of our patient, it would have been
possible to make the diagnosis of Von
Hippel-Lin-dau disease had we been aware of the history of
cerebellar hemangioblastoma in her uncle. An
ear-her suspicion of Von Hippel-Lindau disease in our
patient would certainly have prompted a more
When one of the lesions of Von Hippel-Lindau
disease is discovered in any individual, a complete
investigation of the family must be undertaken to
identify others who might be affected. In some
pedigrees, only one or two lesions can be
demon-strated.37’38 but all siblings have a one out of two
chance of being involved. In the family of our case
subject, it is obvious that genetic transmission
oc-curred from the maternal grandfather to the
mother. No evidence of this disorder could be found
in our patient’s sister, with the possible exception
of a history of breast cysts at the age of 6 years.
Although breast cysts are fairly common in adult
women and have not previously been reported in
Von Hippel-Lindau disease, their appearance in a
child this young is rare and may represent another
manifestation of Von Hippel-Lindau disease.
Medical evaluation of patients with verified or
suspected Von Hippel-Lindau disease should be
directed at three main areas. The first of these is
the retina where ophthalmoscopic examination
aided by fluorescein angiography can delineate
he-mangioblastomas.39 When found, these are readily
amenable to treatment by coagulation.25 Second,
contrast-enhanced CT should be used to identify
possible spinal or cerebellar tumors before they
cause increased pressure or hemorrhage.14’40’41 It
should be used in all who have reached pubertal
age. The third area requiring special attention is
the intraabdominal region. If renal involvement is
to be found early enough for successful excision,
routine screening must be carried out.425’ Because
life-threatening visceral lesions have rarely been
reported to occur before 20 years of age,52 18 years
has been used as an age at which to initiate
screen-ing.’2”3 Most investigators are in agreement that
abdominal CT has replaced IVPs and
nephroto-mograms as the method of choice for an initial
evaluation.’2”423’50’51 If any renal abnormality is
noted on CT, arteriography is then indicated.’4’53
Both of these techniques have the added value of
being able to delineate pheochromocytomas. For
annual follow-up, ultrasound may provide useful
information, but CT scanning is, again, the
rec-ommended modality for use in those with known
renal’4’5’ or pancreatic disease.5’
SUMMARY
Von Hippel-Lindau disease is a hereditary
neo-plastic syndrome that generally manifests in early
adulthood but does present occasionally in
adoles-cence. In the past, diagnosis and management of
this disorder fell within the domain of internists
and surgeons. Because pediatricians are now seeing
older patients, they must learn to recognize the
various components of this disorder. Additionally,
they must be able to assure appropriate medical
evaluation and follow-up and also arrange for
ge-netic counseling. Pediatricians already attuned to
the benefits of preventive medicine should find
themselves uniquely qualified to provide the level
of care that this disease requires.
REFERENCES
MICHELE L. SEITZ, MD I. RONALD SHENKER, MD
JOHN C. LEONIDAS, MD
MICHAEL P. NUSSBAUM, MD
EDWARD S.WIND, MD
Department of Pediatrics, Division
of Adolescent Medicine, and the
Department of Pediatric Radiology
Schneider Children’s Hospital of
Long Island Jewish Medical Center
New Hyde Park, NY
1. Lindau A: Studien uber Kleinhirneysten. Bau Pathogenese and Beziehungen zur Angiomatosis retinae. Acta Pat/wi Microbiol Scand 1926;1(suppl):1-128
2. Melmon KL, Rosen SW: Lindau’s disease: Review of the literature and study of a large kindred. Am J Med 1964; 36:595-617
3. Horton WA, Wong V, Eldridge R: Von Hippel-Lindau dis-ease: Clinical and pathological manifestations in nine fam-lies with 50 affected members. Arch Intern Med 1976;
136:769-777
4. Lee KR, Wulfsberg E, Kepes JJ: Some important radiolog-ical aspects of the kidney in Hippel-Lindau syndrome: The value of prospective study in an affected family. Radiology
1977;122:649-653
5. Glushien AS, Mansuy MM, Littman DS: Pheochromocy-toma: Its relationship to the neurocutaneous syndromes. Am
J Med 1953;14:318-327
6. Chapman RC, Kemp E, Taliaferro I: Pheochromocytoma associated with multiple neorofibromatosis and intracranial hemangioma. Am J Med 1959;26:883-890
7. Illingworth RD: Phaeochromocytoma and cerebellar hae-mangioblastoma. J Neurol Neurosurg Psychiatry 1967;30: 443-435
8. Hull MT, Warfel KA, Muller J, et al: Familial islet cell tumors in Von Hippel-Lindau’s disease. Cancer 1979;
44:1523-1526
9. M#{246}llerHU: Familial angiomatosis retinae et cerebelli-Lin-dau’s disease. Acta Ophthalmoi 1929;7:244-260
10. Silver ML: Hereditary vascular tumors of the nervous sys-tern. JAMA 1954;156:1053-1056
11. Christoferson LA, Gustafson MB, Petersen AG, et al: Von Hippel-Lindau’s disease. JAMA 1961;178:280-282
12. Fill WL, Larniell JM, Polk NO: The radiographic manifes-tations of von Hippel-Lindau disease. Radiology 1979; 133:289-295
13. Pyhtinen J, Suramo I, Lohela P, et al: Abdominal ultraso-nography and computed tomography in von Hippel-Lindau disease.Ann Ciin Res 1982;14:172-176
14. Wesolowski DP, Ellwood RA, Schwab RE, et al: Case re-ports: Hippel-Lindau syndrome in identical twins. Br J Radioi 1981;54:982-986
15. Olivecrona H: The cerebellar angioreticulomas. J Neurosurg
1952;9:317-330
16. Meredith JM, Hennigar, GR: Cerebellar hemangiornas: A dinicopathologic study of fourteen cases. Am Surg 1954; 20:410-423
cere-bellar cysts and the association of polycythemia. Arch Neu-rol Psychiatry 1952;67:237-252
18. Riddoch G: Discussion on vascular tumours of the brain and spinal cord. Proc R Soc Med 1931;24:382-383
19. Rowe PH: von Hippel-Lindau disease: Hereditary tendency in two families. Bull Northwestern Ciin 1955;6:117-125 20. Perlmutter I, Horrax G, Poppen JL: Cystic
henangioblas-tomas of the cerebellum: End results in 25 verified cases.
Surg Gynecoi Obstet 1950;91:89-99
21. Kinney TD, Fitzgerald PJ: Lindau-von Hippel disease with hemangioblastoma of the spinal cord and syringomyelia.
Arch Pat/wi 1947;43:439-455
22. Njcol AA McI: Lindau’s disease in five generations. Ann Hum Genet 1957;22:7-11
23. Hardwig P, Robertson DM: A familial, often lethal, multi-system phakomatosis. Ophthalmology 1984;91:263-270
24. Cushing F!, Bailey P: Hemangiornas ofcerebellum and retina (Lindau’s disease). Arch Ophthalmoi 1928;57:447-462 25. Wing GL, Weiter JJ, Kelly PR, et al: von Hippel-Lindau
disease: Angiomatosis of the retina and the central nervous system. Ophthalmology 1981;88:1311-1314
26. M#{246}llerHU: Ophthalmic symptoms and heredity in cerebel-lar angioreticuloma. Acta Psychiatry Neurol 1944;19:275-292
27. Bird AV, Krynauw RA: Lindau’s disease in a South African family. Br J Surg 1953;40:433-437
28. Otenasek FJ, Silver ML Spinal hemangioma (hemangio-blastoma) in Lindau’s disease. J Neurosurg 1961;18:295-300
29. Goodman J, Kleinholz E, Peck FC: Lindau’s disease-In the Hudson Valley. J Neurosurg 1964;21:97-103
30. Rho Y: Von Hippel-Lindau’s disease: A report of five cases.
Can Med Assoc J 1969;101:135-142
31. Schecterman L: Lindau’s disease: Report of an unusual case and two additional cases in a Negro family. Med Ann Dis Coi 1961;30:64-76
32. Nyggaard KK, Walters W: Polycystic disease ofthe pancreas (dysontogenetic cysts): Report of a case with partial pan-createctomy. Ann Surg 1937;106:49-53
33. Fishman RS, Bartholomew LG: Severe pancreatic involve-ment in three generations in von Hippel-Lindau disease.
Mayo Ciin Proc 1979;54:329-331
34. Jackaman FR: Polycystic pancreas: Lindau’s disease. J Coil Surg Edinb 1984;29:121-122
35. Steiner M, Klein E, Klein G: Antinuclear reactivity of sera in patients with leukemia and other neoplastic diseases. Ciin Immunoi Immunopathol 1975;4:374-381
36. Klajman A, Kafri B, Shohat T, et al: The prevalence of antibodies to histones induced by procainarnide, in old peo-pie, in cancer patients, and in rheumatoid-like disease. Gun
Immunol Immunopathol 1983;27:1-8
37. Pearson JC, Weiss ,J, Tanagho EA: A plea for conservation in renal adenocarcinorna associated with von Hippel-Lindau disease. J Urol 1980;124:910-912
38. Adams JE: Familial hemangioblastoma of the cerebellum: Pedigree of two families. J Neurosurg 1953;10:421-423 39. Welch RB: Fluorescein angiography in side-cell retinopathy
and Hippel-Lindau disease.
mt
Ophthalmol Gun 1977;17:137-154
40. Baleriaux-Waha D, Retif J, Noterman J, et al: CT scanning for the diagnosis of the cerebellar and spinal lesions of von Hippel-Lindau’s disease. Neuroradiology 1978;14:241-244 41. Seeger JF, Burke DP, Knake JE, et al: Computed
tomo-graphic and angiographic evaluation of hemangioblastomas.
Radiology 1981;138:633-634
42. Greene LF, Rosenthal MH: Multiple hypernephrornas of the kidney in association with Lindau’s disease. N EngI J Med
1951;244:633-634
43. Isaac F, Schoen I, Walker P: An unusual case of Lindau’s disease: Cystic disease of the kidneys and pancreas with renal and cerebellar tumors. AJR 1956;75:912-920
44. Malek RS, Greene LF: Urologic aspects of Hippel-Lindau syndrome. J Urol 1971;104:800-801
45. Richards RD, Mebust WK, Schirnke RN: A prospective study on Von Hippel-Lindau disease. J Urol 1973;110:27-30 46. Goodbody RA, Gamlen TR: Cerebellar hearnangioblastoma
and genitourinary turnours. J Neurol Neurosurg Psychiatry
1974;37:606-609
47. Coulam CM, Brown LR, Reese DF: Hippel-Lindau syn-drome. Semin Roentgenol 1976;11:61-66
48. Hubschmann OR, Vijayanathan T, Countee RW: Von Hip-pel-Lindau disease with multiple manifestations: Diagnosis
and management. Neurosurgery 1981;8:92-95
49. Bickler 5, Wile AG, Melicharek M, et al: Pancreatic involve-ment in Hippel-Lindau disease. West J Med
1983;140:280-282
50. Levine E. Collins DL, Horton WA, et al: CT screening of
the abdomen in von Hippel-Lindau disease. AJR
1982;139:505-510
51. Pockros PJ, Thurston D, Michelson JB, et al: Retinal an-giomatosis and pancreatic cysts in Von Hippel-Lindau dis-ease: Use of computed tomography scanning for diagnosis and surveillance. J Gomput Tomogr 1985;9:293-297
52. Atuk NO, McDonald T, Wood T, et al: Familial pheochro-mocytoma, hypercalcemia, and von Hippel-Lindau disease.
Medicine 1979;58:209-218
53. Levine E, Lee KR, Weigei JW, et al: Computed tomography in the diagnosis of renal carcinoma complicating Hippel-Lindau syndrome. Radiology 1979;130:703-706
Vulnerable
Sibling:
Hyponatremia
From
Caries
Prevention
Parental actions and beliefs shape every aspect
of a child’s health and development. Green and
Solnit’ described a vulnerable child syndrome in
which the child’s development was impaired when
the parents treated him or her in an inappropriate
fashion because they had an unreasonable
expec-tation that he or she would die. We recently cared
for a child who suffered a life-threatening event
(status epilepticus due to hyponatremia) because
the parents were trying to avoid reproducing a
serious, but not life-threatening, problem (nursing
bottle caries) that had occurred in the older sibling.
This distortion of professional advice created
vu!-nerability to significant neurologic problems.
Reprint requests to (D.L.C.) Department of Pediatrics, Medical College of Pennsylvania, 3300 Henry Ave., Philadelphia, PA 19129.
PEDIATRICS (ISSN 0031 4005). Copyright © 1987 by the American Academy of Pediatrics.
CASE REPORT
A 4#{189}-month-old male infant was brought to the
emer-gency room after the parents found him lying in bed
